Post on 02-Jan-2017
2012
Primary Renal Disease (PRD) codes
(version 1.0)
ERA‐EDTA Registry Academic Medical Center Department of Medical Informatics PO Box 22700 1100 DE Amsterdam The Netherlands Telephone +31 20 566 7637 Fax +31 20 691 9840 E‐mail eracoding@amc.uva.nl
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This material includes SNOMED Clinical Terms® (SNOMED CT®) which is used by permission of the International
Health Terminology Standards Development Organisation (IHTSDO). All rights reserved. SNOMED CT®, was
originally created by The College of American Pathologists. “SNOMED” and “SNOMED CT” are registered
trademarks of the IHTSDO.
Unless an IHTSDO Affiliate License to use SNOMED CT is held, the user of the SNOMED CT content included in this
Mapping Spreadsheet is required to accept the following restrictions:
a) The use is limited to SNOMED CT concept codes, descriptions and expressions contained in the Mapping
Spreadsheet;
b) The use is limited to purposes related to diagnosis, treatment and management of renal disorders;
c) The use does not involve or provide access to SNOMED CT relationships, except indirectly through inclusion of
expressions in the Mapping Spreadsheet;
d) The use does not permit download or use of portions of SNOMED CT that are not covered by this agreement;
e) The use does not involve more than a total of six‐hundred (600) SNOMED CT concepts.
Furthermore, the use has limitations as stated in the Affiliate license as they apply to sub‐licensees
(www.ihtsdo.org/license.pdf).
Disclaimer
This ERA‐EDTA Coding system for Primary Renal Disease and the related web‐based PRD search tool are tools
assisting in recording the diagnosis of a renal disease. They are offered for free to those who want to use it for
research, teaching and for improvement of the quality of care for patients after a diagnosis has been made by the
nephrologist caring for the patient. In publications users are kindly requested to cite the related paper by Venkat‐
Raman G. et al. in Nephrology Dialysis Transplantation 2013.
ERA‐EDTA, the members of its Coding and Definitions Working Group, and its Registry housed at AMC
(Amsterdam, The Netherlands) disclaim any express or implied warranty of fitness for such use and shall not be
liable for any direct, indirect or consequential loss; personal injury; special or punitive damages; loss of profits,
loss of savings and loss of revenue; loss of business, loss of reputation and loss of goodwill; and loss of data
arising from the use of the Coding system and/or the related web‐based PRD search tool.
I GLOMERULAR DISEASE
1
3749 Glomerulonephritis ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Immunology ‐ Urine analysis ‐ Imaging
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined
SNOMED CT concept identifier and fully specified name ‐ 36171008 ‐ Glomerulonephritis (disorder)
ICD‐10 code and term
‐ N059 ‐ Unspecified nephritic syndrome, Unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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2
1003 Adult nephrotic syndrome ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ A history of heavy proteinuria at some point is required. Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 52254009 ‐ Nephrotic syndrome (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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3
1019 Nephrotic syndrome of childhood ‐ steroid sensitive ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ Defined by response to steroid therapy. Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 445119005 ‐ Steroid sensitive nephrotic syndrome of childhood (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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4
3604 Nephrotic syndrome of childhood ‐ steroid resistant ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/600995 about nephrotic syndrome, type 2; NPHS2 = nephrotic syndrome, steroid‐resistant, autosomal recessive; SRN1
‐ http://omim.org/entry/610725 about nephrotic syndrome, type 3; NPHS3 = nephrotic syndrome, early‐onset, type 3
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ No remission despite a therapeutic trial of corticosteroids. Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 800991000000107 ‐ Steroid resistant nephrotic syndrome of childhood (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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5
3615 Nephrotic syndrome of childhood ‐ no trial of steroids ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ Nephrotic syndrome in childhood. No trial of steroids, no histology, no information to allow a more detailed PRD to be selected.
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 445119005 ‐ Steroid sensitive nephrotic syndrome of childhood (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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6
1026 Congenital nephrotic syndrome (CNS) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/256300 about nephrotic syndrome, type 1; NPHS1 ‐ http://omim.org/entry/602716 about nephrin; NPHS1 ‐ http://omim.org/entry/600995 about nephrotic syndrome, type 2; NPHS2 = nephrotic
syndrome, steroid‐resistant, autosomal recessive; SRN1 ‐ http://omim.org/entry/604766 about podocin; NPHS2 ‐ http://omim.org/entry/610725 about nephrotic syndrome, type 3; NPHS3 = nephrotic
syndrome, early‐onset, type 3 ‐ http://omim.org/entry/256370 about nephrotic syndrome, type 4; NPHS4 ‐ http://omim.org/entry/614199 about nephrotic syndrome, type 5, with or without
ocular abnormalities; NPHS5 ‐ http://omim.org/entry/614196 about nephrotic syndrome, type 6; NPHS6
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ Defined by response to steroid therapy. ‐ Clinical history: Onset in 1st 3 months after birth.
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 48796009 ‐ Congenital nephrotic syndrome (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
I GLOMERULAR DISEASE
7
1035 Congenital nephrotic syndrome (CNS) ‐ Finnish type ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/256300 about nephrotic syndrome, type 1; NPHS1 ‐ http://omim.org/entry/602716 about nephrin; NPHS1
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ Mutation in the gene encoding nephrin (NPHS1) on chromosome 19q13.1. ‐ Clinical history: Onset in 1st 3 months after birth.
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 197601003 ‐ Finnish congenital nephrotic syndrome (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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8
1042 Congenital nephrotic syndrome (CNS) ‐ Finnish type ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/256300 ‐ http://omim.org/entry/602716 about nephrin; NPHS1
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ Mutation in the gene encoding nephrin (NPHS1) on chromosome 19q13.1. ‐ Clinical history: Onset in 1st 3 months after birth.
Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 197601003 ‐ Finnish congenital nephrotic syndrome (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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9
1057 Congenital nephrotic syndrome (CNS) ‐ diffuse mesangial sclerosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/256370 about nephrotic syndrome, type 4; NPHS4 ‐ http://omim.org/entry/607102 about WT1 gene; WT1
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ Often associated with mutation in the Wilms tumor suppressor gene (WT1) on chromosome 11p13.
‐ Clinical history: Onset in 1st 3 months after birth. Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 48796009 ‐ Congenital nephrotic syndrome (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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10
1061 Congenital nephrotic syndrome (CNS) ‐ focal segmental glomerulosclerosis (FSGS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/600995 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ Clinical history: Onset in 1st 3 months after birth. ‐ Often associated with NPHS2 mutations.
Old ERA‐EDTA PRD code
‐ 11 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in children SNOMED CT concept identifier and fully specified name
‐ 236384008 ‐ Congenital nephrotic syndrome with focal glomerulosclerosis (disorder) ICD‐10 code and term
‐ N071 ‐ Focal and segmental glomerular lesions
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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11
1074 Denys‐Drash syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/194080 ‐ http://omim.org/entry/607102 about WT1 gene; WT1
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Gene test
Additional notes/criteria
‐ Mutation in the WT1 gene. ‐ Clinical history: Onset in 1st 3 months after birth.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 236385009 ‐ Drash syndrome (disorder) ICD‐10 code and term
‐ N048 ‐ Nephrotic syndrome, other
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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12
1088 Congenital nephrotic syndrome (CNS) ‐ congenital infection Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ Clinical history: Onset in 1st 3 months after birth. Infection as a cause of congenital nephrotic syndrome is rare, especially in Europe.
‐ This PRD should not be used for hepatitis C related nephropathy or autoimmune disease, both of which are coded elsewhere.
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 48796009 ‐ Congenital nephrotic syndrome (disorder) ICD‐10 code and term
‐ N049 ‐ Nephrotic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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13
1090 Minimal change nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ A history of heavy proteinuria at some point is required. Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 44785005 ‐ Minimal change disease (disorder) ICD‐10 code and term
‐ N050 ‐ Unspecified nephritic syndrome, minor glomerular abnormality
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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14
1100 Minimal change nephropathy ‐ histologically proven Criteria for diagnosis [all to be satisfied] ‐ Histology ‐ Biochemistry Additional notes/criteria ‐ A history of heavy proteinuria at some point is required. Old ERA‐EDTA PRD code ‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name ‐ 44785005 ‐ Minimal change disease (disorder) ICD‐10 code and term ‐ N050 ‐ Unspecified nephritic syndrome, minor glomerular abnormality
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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15
1116 IgA nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/161950 ‐ http://omim.org/entry/613944
Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ In the absence of a renal histopathology, consider alternative PRDs (e.g. postinfection glomerulonephritis) before choosing this code.
‐ Note that Henoch‐Schönlein nephritis has a separate PRD. Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 236407003 ‐ IgA nephropathy (disorder) ICD‐10 code and term
‐ N028 ‐ Recurrent and persistent haematuria, other
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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16
1128 IgA nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/161950 ‐ http://omim.org/entry/613944
Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ IgA must be demonstrated by renal histopathology. ‐ IgA in skin histopathology or raised serum IgA concentration are not sufficient for this
PRD. ‐ Note that Henoch‐Schönlein nephritis is coded separately.
Old ERA‐EDTA PRD code
‐ 12 ‐ IgA nephropathy (proven by immunofluorescence, not code 76 or 85) SNOMED CT concept identifier and fully specified name
‐ 236407003 ‐ IgA nephropathy (disorder) ICD‐10 code and term
‐ N028 ‐ Recurrent and persistent haematuria, other
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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17
1137 Familial IgA nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/161950 ‐ http://omim.org/entry/613944
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history
Additional notes/criteria
‐ With histological evidence in a first degree relative and a compatible clinical setting in the patient, this PRD allows nephrologists to record their preferred diagnosis of IgA nephropathy even in the absence of a renal histopathology.
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 445404003 ‐ Familial immunoglobulin A nephropathy (disorder) ICD‐10 code and term
‐ N028 ‐ Recurrent and persistent haematuria, other
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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18
1144 Familial IgA nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/161950 ‐ http://omim.org/entry/613944
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history
Additional notes/criteria
‐ IgA must be demonstrated by renal histopathology. ‐ IgA in skin histopathology or raised serum IgA concentration are not sufficient for this
PRD. Old ERA‐EDTA PRD code
‐ 12 ‐ IgA nephropathy (proven by immunofluorescence, not code 76 or 85) SNOMED CT concept identifier and fully specified name
‐ 445404003 ‐ Familial immunoglobulin A nephropathy (disorder) ICD‐10 code and term
‐ N028 ‐ Recurrent and persistent haematuria, other
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
I GLOMERULAR DISEASE
19
1159 IgA nephropathy secondary to liver cirrhosis ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 282364005 ‐ IgA nephropathy associated with liver disease (disorder) ICD‐10 code and term
‐ N028 ‐ Recurrent and persistent haematuria, other AND ‐ K746 ‐ Other and unspecified cirrhosis of liver
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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20
1163 IgA nephropathy secondary to liver cirrhosis ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 12 ‐ IgA nephropathy (proven by immunofluorescence, not code 76 or 85) SNOMED CT concept identifier and fully specified name
‐ 282364005 ‐ IgA nephropathy associated with liver disease (disorder) ICD‐10 code and term
‐ N028 ‐ Recurrent and persistent haematuria, other AND ‐ K746 ‐ Other and unspecified cirrhosis of liver
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21
1171 IgM ‐ associated nephropathy Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ The presence of IgM should be demonstrated by IF and the presence of deposits compatible with Immunoglobulins in the same region of the glomerulus should be seen on EM.
Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 236411009 ‐ IgM nephropathy (disorder) ICD‐10 code and term
‐ N053 ‐ Diffuse mesangial proliferative glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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22
1185 Membranous nephropathy ‐ idiopathic Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/604939 about phospholipase A2 receptor 1; PLA2R1 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Additional notes/criteria
‐ May be associated with antibodies to the phospholipase A2 receptor. ‐ This PRD should not be used for SLE related nephropathies which are coded
elsewhere. Old ERA‐EDTA PRD code
‐ 14 ‐ Membranous nephropathy SNOMED CT concept identifier and fully specified name
‐ 197590001 ‐ Nephrotic syndrome with membranous glomerulonephritis (disorder) ICD‐10 code and term
‐ N042 ‐ Nephrotic syndrome, diffuse membranous glomerulonephritis
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1192 Membranous nephropathy ‐ malignancy associated Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 14 ‐ Membranous nephropathy SNOMED CT concept identifier and fully specified name
‐ 197590001 ‐ Nephrotic syndrome with membranous glomerulonephritis (disorder) ICD‐10 code and term
‐ N042 ‐ Nephrotic syndrome, diffuse membranous glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1205 Membranous nephropathy ‐ drug induced Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 14 ‐ Membranous nephropathy SNOMED CT concept identifier and fully specified name
‐ 197590001 ‐ Nephrotic syndrome with membranous glomerulonephritis (disorder) ICD‐10 code and term
‐ N042 ‐ Nephrotic syndrome, diffuse membranous glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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25
1214 Membranous nephropathy ‐ infection associated Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 14 ‐ Membranous nephropathy SNOMED CT concept identifier and fully specified name
‐ 197590001 ‐ Nephrotic syndrome with membranous glomerulonephritis (disorder) ICD‐10 code and term
‐ N042 ‐ Nephrotic syndrome, diffuse membranous glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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26
1222 Mesangiocapillary glomerulonephritis type 1 Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/305800 Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Often associated with cryoglobulinaemia. Old ERA‐EDTA PRD code
‐ 15 ‐ Membrano‐proliferative GN; type I (proven by immunofluorescence / electron microscopy, not code 84 or 89)
SNOMED CT concept identifier and fully specified name
‐ 75888001 ‐ Mesangiocapillary glomerulonephritis, type I (disorder) ICD‐10 code and term
‐ N055 ‐ Diffuse mesangiocapillary glomerulonephritis
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1233 Mesangiocapillary glomerulonephritis type 2 (dense deposit disease) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/305800 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 13 ‐ Dense deposit disease; membrano‐proliferative GN; type II (proven by immunofluorescence / electron microscopy)
SNOMED CT concept identifier and fully specified name
‐ 59479006 ‐ Mesangiocapillary glomerulonephritis, type II (disorder) ICD‐10 code and term
‐ N056 ‐ Unspecified nephritic syndrome, dense deposit disease
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1246 Mesangiocapillary glomerulonephritis type 3 Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/305800 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 236409000 ‐ Mesangiocapillary glomerulonephritis type III (disorder) ICD‐10 code and term
‐ N055 ‐ Diffuse mesangiocapillary glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1251 Idiopathic rapidly progressive (crescentic) glomerulonephritis Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ This PRD should not be used for other specific PRDs e.g. IgA, vasculitis or Goodpasture's syndrome.
Old ERA‐EDTA PRD code
‐ 16 ‐ Crescentic (extracapillary) glomerulonephritis (type I, II, III) SNOMED CT concept identifier and fully specified name
‐ 236398000 ‐ Crescentic glomerulonephritis (disorder) ICD‐10 code and term
‐ N057 ‐ Diffuse concentric glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1267 Primary focal segmental glomerulosclerosis (FSGS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6
Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 17 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in adults SNOMED CT concept identifier and fully specified name
‐ 236403004 ‐ Focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
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1279 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal recessive ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Gene test
Additional notes/criteria
‐ This PRD can be used if there is a compatible family history with renal histological evidence in a first degree relative but not in the patient.
Old ERA‐EDTA PRD code
‐ 50 ‐ Hereditary/Familial nephropathy ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 445388002 ‐ Autosomal recessive focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
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1280 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal recessive ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history ‐ Gene test
Additional notes/criteria
‐ This PRD should be used if there is a compatible family history and renal histological evidence in the patient.
Old ERA‐EDTA PRD code
‐ 11 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in children SNOMED CT concept identifier and fully specified name
‐ 445388002 ‐ Autosomal recessive focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
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1298 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal dominant ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6 ‐ http://omim.org/entry/604638 about Actinin
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Gene test
Additional notes/criteria
‐ This PRD can be used if there is a compatible family history with renal histological evidence in a first degree relative but not in the patient.
Old ERA‐EDTA PRD code
‐ 50 ‐ Hereditary/Familial nephropathy ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 444977005 ‐ Autosomal dominant focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
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1308 Familial focal segmental glomerulosclerosis (FSGS) ‐ autosomal dominant ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6 ‐ http://omim.org/entry/604638 about Actinin
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history ‐ Gene test
Additional notes/criteria
‐ This PRD should be used if there is a compatible family history and renal histological evidence in the patient.
Old ERA‐EDTA PRD code
‐ 11 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in children SNOMED CT concept identifier and fully specified name
‐ 444977005 ‐ Autosomal dominant focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1312 Focal segmental glomerulosclerosis (FSGS) secondary to obesity ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6
Criteria for diagnosis [all to be satisfied]
‐ Clinical exam ‐ Biochemistry
Additional notes/criteria
‐ Obesity on clinical examination. ‐ This PRD should be used with great caution. ‐ It allows a nephrologist to give an opinion in the absence of the histological proof
which is required to substantiate a diagnosis of FSGS. ‐ An alternative PRD e.g. 'Adult Nephrotic syndrome ‐ no histology' should be
considered. Old ERA‐EDTA PRD code
‐ 17 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in adults SNOMED CT concept identifier and fully specified name
‐ 236403004 ‐ Focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1320 Focal segmental glomerulosclerosis (FSGS) secondary to obesity ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603278 about FSGS1 ‐ http://omim.org/entry/603965 about FSGS2 ‐ http://omim.org/entry/607832 about FSGS3 ‐ http://omim.org/entry/612551 about FSGS4 ‐ http://omim.org/entry/613237 about FSGS5 ‐ http://omim.org/entry/614131 about FSGS6
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical exam ‐ Biochemistry
Additional notes/criteria
‐ Obesity on clinical examination. Old ERA‐EDTA PRD code
‐ 17 ‐ Focal segmental glomerulosclerosis with nephrotic syndrome in adults SNOMED CT concept identifier and fully specified name
‐ 236403004 ‐ Focal segmental glomerulosclerosis (disorder) ICD‐10 code and term
‐ N051 ‐ Focal and segmental glomerular lesions
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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37
1331 Diffuse endocapillary glomerulonephritis Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Typical of post‐infectious glomerulonephritis. Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 3704008 ‐ Diffuse endocapillary proliferative glomerulonephritis (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1349 Mesangial proliferative glomerulonephritis Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ This PRD should not be used for SLE related nephropathies which are coded elsewhere.
Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 35546006 ‐ Mesangial proliferative glomerulonephritis (disorder) ICD‐10 code and term
‐ N033 ‐ Diffuse mesangial proliferative glomerulonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1354 Focal and segmental proliferative glomerulonephritis Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 83866005 ‐ Focal and segmental proliferative glomerulonephritis (disorder) ICD‐10 code and term
‐ N071 ‐ Focal and segmental glomerular lesions
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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40
1365 Glomerulonephritis ‐ secondary to other systemic disease Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Examples of the systemic conditions include malignancy and liver disease but this PRD should not be used if a more accurate one is available.
Old ERA‐EDTA PRD code
‐ 10 ‐ Glomerulonephritis; histologically NOT examined SNOMED CT concept identifier and fully specified name
‐ 36171008 ‐ Glomerulonephritis (disorder) ICD‐10 code and term
‐ N059 ‐ Unspecified nephritic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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41
1377 Glomerulonephritis ‐ histologically indeterminate Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Use this PRD if no other seems appropriate even after considering all the evidence and the renal histopathology report.
Old ERA‐EDTA PRD code
‐ 19 ‐ Glomerulonephritis; histologically examined SNOMED CT concept identifier and fully specified name
‐ 36171008 ‐ Glomerulonephritis (disorder) ICD‐10 code and term
‐ N059 ‐ Unspecified nephritic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1383
Systemic vasculitis ‐ ANCA negative ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Old ERA‐EDTA PRD code
‐ 70 ‐ Renal vascular disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 46956008 ‐ Systemic vasculitis (disorder) ICD‐10 code and term
‐ I776 ‐ Arteritis, unspecified
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1396 Systemic vasculitis ‐ ANCA positive ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Immunology
Additional notes/criteria
‐ Must be clinical evidence to suggest a vasculitis. Old ERA‐EDTA PRD code
‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name
‐ 46956008 ‐ Systemic vasculitis (disorder) ICD‐10 code and term
‐ I776 ‐ Arteritis, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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44
1401 Granulomatosis with polyangiitis ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/177020 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Immunology
Additional notes/criteria
‐ Involvement of upper or lower respiratory tract or the oral cavity in addition to renal involvement.
Old ERA‐EDTA PRD code
‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name
‐ 195353004 ‐ Wegener's granulomatosis (disorder) ICD‐10 code and term
‐ M313 ‐ Wegener's granulomatosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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45
1417 Granulomatosis with polyangiitis ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/177020 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Additional notes/criteria
‐ In a compatible clinical setting, histological diagnosis from nasal or respiratory tract can support this PRD if renal histology is not available.
Old ERA‐EDTA PRD code
‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name
‐ 195353004 ‐ Wegener's granulomatosis (disorder) ICD‐10 code and term
‐ M313 ‐ Wegener's granulomatosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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46
1429 Microscopic polyangiitis ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Old ERA‐EDTA PRD code
‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name
‐ 239928004 ‐ Microscopic polyarteritis nodosa (disorder) ICD‐10 code and term
‐ M300 ‐ Polyarteritis nodosa
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47
1438 Churg‐Strauss syndrome ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Immunology
Additional notes/criteria
‐ Peripheral blood eosinophilia is often found. Old ERA‐EDTA PRD code
‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name
‐ 82275008 ‐ Allergic granulomatosis angiitis (disorder) ICD‐10 code and term
‐ M301 ‐ Polyarteritis with lung involvement [Churg‐Strauss]
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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1440 Churg‐Strauss syndrome ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Old ERA‐EDTA PRD code
‐ 74 ‐ Wegener's granulomatosis SNOMED CT concept identifier and fully specified name
‐ 82275008 ‐ Allergic granulomatosis angiitis (disorder) ICD‐10 code and term
‐ M301 ‐ Polyarteritis with lung involvement [Churg‐Strauss]
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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49
1455 Polyarteritis nodosa Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ ANCA may be positive but specific tests for anti MPO and anti PR3 are negative. ‐ PAN can be secondary to other disorders. ‐ Use this PRD for classical PAN proven with imaging. ‐ Do not use this PRD for any type of microscopic polyangiitis.
Old ERA‐EDTA PRD code
‐ 73 ‐ Renal vascular disease due to polyarteritis SNOMED CT concept identifier and fully specified name
‐ 155441006 ‐ Polyarteritis nodosa (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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50
1464 Anti‐Glomerular basement membrane (GBM) disease / Goodpasture's syndrome ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/233450 Criteria for diagnosis [all to be satisfied]
‐ Immunology Old ERA‐EDTA PRD code
‐ 86 ‐ Goodpasture's Syndrome SNOMED CT concept identifier and fully specified name
‐ 236506009 ‐ Goodpasture's disease (disorder) ICD‐10 code and term
‐ M310 ‐ Hypersensitivity angiitis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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51
1472 Anti‐Glomerular basement membrane (GBM) disease / Goodpasture's syndrome ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/233450 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 86 ‐ Goodpasture's Syndrome SNOMED CT concept identifier and fully specified name
‐ 50581000 ‐ Goodpasture's syndrome ICD‐10 code and term
‐ M310 ‐ Hypersensitivity angiitis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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52
1486 Systemic lupus erythematosus / nephritis ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/152700 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Immunology
Additional notes/criteria
‐ Evidence of renal disease manifested by at least either proteinuria or haematuria. Old ERA‐EDTA PRD code
‐ 84 ‐ Lupus erythematosus SNOMED CT concept identifier and fully specified name
‐ 68815009 ‐ Systemic lupus erythematosus glomerulonephritis syndrome (disorder) ICD‐10 code and term
‐ M321D ‐ Systemic lupus erythematosus with organ or sys involv
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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53
1493 Systemic lupus erythematosus / nephritis ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/152700 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 84 ‐ Lupus erythematosus SNOMED CT concept identifier and fully specified name
‐ 68815009 ‐ Systemic lupus erythematosus glomerulonephritis syndrome (disorder) ICD‐10 code and term
‐ M321D ‐ Systemic lupus erythematosus with organ or sys involv
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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54
1504 Henoch‐Schönlein purpura / nephritis ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam
Additional notes/criteria
‐ Evidence of renal disease manifested by at least either proteinuria or haematuria. ‐ Must be clinical evidence or history compatible with HSP.
Old ERA‐EDTA PRD code
‐ 85 ‐ Henoch‐Schoenlein purpura SNOMED CT concept identifier and fully specified name
‐ 191306005 ‐ Henoch‐Schönlein purpura (disorder) ICD‐10 code and term
‐ D690 ‐ Allergic purpura
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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55
1515 Henoch‐Schönlein purpura / nephritis ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Clinical exam
Additional notes/criteria
‐ Must be clinical evidence or history compatible with HSP. Old ERA‐EDTA PRD code
‐ 85 ‐ Henoch‐Schoenlein purpura SNOMED CT concept identifier and fully specified name
‐ 191306005 ‐ Henoch‐Schönlein purpura (disorder) ICD‐10 code and term
‐ D690 ‐ Allergic purpura
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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56
1527 Renal scleroderma / systemic sclerosis ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ Cutaneous and systemic symptoms with autoantibodies. Old ERA‐EDTA PRD code
‐ 87 ‐ Systemic sclerosis (scleroderma) SNOMED CT concept identifier and fully specified name
‐ 89155008 ‐ Systemic sclerosis ICD‐10 code and term
‐ M349 ‐ Systemic sclerosis, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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57
1536 Renal scleroderma / systemic sclerosis ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ Cutaneous and systemic symptoms with autoantibodies. Old ERA‐EDTA PRD code
‐ 87 ‐ Systemic sclerosis (scleroderma) SNOMED CT concept identifier and fully specified name
‐ 236502006 ‐ Renal involvement in scleroderma (disorder) ICD‐10 code and term
‐ M348 ‐ Other forms of systemic sclerosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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58
1543 Essential mixed cryoglobulinaemia ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/123550 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Old ERA‐EDTA PRD code
‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name
‐ 239947001 ‐ Essential mixed cryoglobulinemia (disorder) ICD‐10 code and term
‐ D891 ‐ Cryoglobulinaemia
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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59
1558 Essential mixed cryoglobulinaemia ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name
‐ 239947001 ‐ Essential mixed cryoglobulinemia (disorder) ICD‐10 code and term
‐ D891 – Cryoglobulinaemia
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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60
1562 Cryoglobulinaemia secondary to hepatitis C ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/609532 Criteria for diagnosis [all to be satisfied]
‐ Immunology Old ERA‐EDTA PRD code
‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name
‐ 30911005 ‐ Cryoglobulinemia (disorder) ICD‐10 code and term
‐ D891 ‐ Cryoglobulinaemia AND B171 ‐ Acute hepatitis C OR ‐ D891 ‐ Cryoglobulinaemia AND B182 ‐ Chronic hepatitis C
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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61
1570 Cryoglobulinaemia secondary to hepatitis C ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/609532 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Old ERA‐EDTA PRD code
‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name
‐ 30911005 ‐ Cryoglobulinemia (disorder) ICD‐10 code and term
‐ D891 ‐ Cryoglobulinaemia AND B171 ‐ Acute hepatitis C OR ‐ D891 ‐ Cryoglobulinaemia AND B182 ‐ Chronic hepatitis C
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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62
1589 Cryoglobulinaemia secondary to systemic disease ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Immunology Additional notes/criteria
‐ This PRD should not be used for hepatitis C related nephropathy or autoimmune disease. Alternative PRDs are available.
Old ERA‐EDTA PRD code
‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name
‐ 30911005 ‐ Cryoglobulinemia (disorder) ICD‐10 code and term
‐ D891 ‐ Cryoglobulinaemia
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
63
1591 Cryoglobulinaemia secondary to systemic disease ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Immunology
Additional notes/criteria
‐ This PRD should not be used for hepatitis C related nephropathy or autoimmune disease. Alternative PRDs are available.
Old ERA‐EDTA PRD code
‐ 78 ‐ Cryoglobulinaemic glomerulonephritis SNOMED CT concept identifier and fully specified name
‐ 30911005 ‐ Cryoglobulinemia (disorder) ICD‐10 code and term
‐ D891 – Cryoglobulinaemia
II TUBULOINTERSTITIAL DISEASE
64
1602 Primary reflux nephropathy ‐ sporadic Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ A positive family history excludes this diagnosis. ‐ This PRD should not normally be used if there is a positive family history. In that case
use PRD 'Familial reflux nephropathy'. Old ERA‐EDTA PRD code
‐ 24 ‐ Pyelonephritis due to vesico‐ureteric reflux without obstruction SNOMED CT concept identifier and fully specified name
‐ 197764002 ‐ Non‐obstructive reflux‐associated chronic pyelonephritis (disorder) ICD‐10 code and term
‐ N110 ‐ Nonobstructive reflux‐associated chronic pyelonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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65
1618 Familial reflux nephropathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/193000 about VUR1 ‐ http://omim.org/entry/610878 about VUR2 ‐ http://omim.org/entry/613674 about VUR3
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Imaging
Additional notes/criteria
‐ A positive family history is required for this PRD. Old ERA‐EDTA PRD code
‐ 24 ‐ Pyelonephritis due to vesico‐ureteric reflux without obstruction SNOMED CT concept identifier and fully specified name
‐ 522551000000101 ‐ Familial non‐obstructive reflux‐associated chronic pyelonephritis (disorder)
ICD‐10 code and term
‐ N110 ‐ Nonobstructive reflux‐associated chronic pyelonephritis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
66
1625 Congenital dysplasia / hypoplasia Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 60 ‐ Renal hypoplasia (congenital) ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 204949001 ‐ Renal dysplasia (disorder) ICD‐10 code and term
‐ Q614 ‐ Renal dysplasia
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
67
1639 Multicystic dysplastic kidneys Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/143400 ‐ http://omim.org/entry/602868 about cell division cycle 5, s. pombe, homolog of;
CDC5L Criteria for diagnosis [all to be satisfied]
‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Associated with the HNF1B gene. Old ERA‐EDTA PRD code
‐ 40 ‐ Cystic kidney disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 82525005 ‐ Congenital cystic kidney disease (disorder) ICD‐10 code and term
‐ Q619 ‐ Cystic kidney disease, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
68
1641 Dysplasia due to fetal ACE‐inhibitor exposure Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/106180 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Imaging
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 519331000000100 ‐ Renal dysplasia due to fetus affected by maternal use of angiotensin converting enzyme inhibitor (disorder)
ICD‐10 code and term
‐ Q614 ‐ Renal dysplasia
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
69
3627 Renal cysts and diabetes syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/137920 Criteria for diagnosis [all to be satisfied]
‐ Immunology ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Associated with the HNF1B gene. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 446641003 ‐ Renal cysts and diabetes syndrome (disorder) ICD‐10 code and term
‐ E142 ‐ Unspecified diabetes mellitus with renal complications
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
70
1656 Glomerulocystic disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/609886 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Gene test
Additional notes/criteria
‐ Associated with the HNF1B gene. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 253864004 ‐ Familial hypoplastic, glomerulocystic kidney (disorder) ICD‐10 code and term
‐ Q605 ‐ Renal hypoplasia, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
71
1660 Congenital pelvi‐ureteric junction obstruction Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/143400 Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 373584008 ‐ Congenital pelviureteric junction obstruction (disorder) ICD‐10 code and term
‐ Q621 ‐ Atresia and stenosis of ureter
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
72
1673 Congenital vesico‐ureteric junction obstruction Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/193000 about VUR1 ‐ http://omim.org/entry/610878 about VUR2 ‐ http://omim.org/entry/613674 about VUR3
Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 373585009 ‐ Congenital ureterovesical obstruction (disorder) ICD‐10 code and term
‐ Q621 ‐ Atresia and stenosis of ureter
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
73
1687 Posterior urethral valves Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 253900005 ‐ Congenital posterior urethral valves (disorder) ICD‐10 code and term
‐ Q643 ‐ Other atresia and stenosis of urethra and bladder neck
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
74
1694 Syndrome of agenesis of abdominal muscles ‐ prune belly syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/100100 ‐ http://omim.org/entry/264140
Criteria for diagnosis [all to be satisfied]
‐ Clinical exam ‐ Imaging
Old ERA‐EDTA PRD code
‐ 66 ‐ Syndrome of agenesis of abdominal muscles (Prune Belly) SNOMED CT concept identifier and fully specified name
‐ 5187006 ‐ Prune belly syndrome ICD‐10 code and term
‐ Q794 ‐ Prune belly syndrome
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
75
1706 Congenital neurogenic bladder Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ Investigation will usually include urodynamic studies. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 445387007 ‐ Congenital neurogenic urinary bladder (finding) ICD‐10 code and term
‐ N319 ‐ Neuromuscular dysfunction of bladder, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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76
1710 Bladder exstrophy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/600057 Criteria for diagnosis [all to be satisfied]
‐ Clinical exam Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 61758007 ‐ Exstrophy of bladder sequence (disorder) ICD‐10 code and term
‐ Q641 ‐ Exstrophy of urinary bladder
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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77
1723 Megacystis‐megaureter Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ Bilateral hydroureteronephrosis; large, smooth, thin‐walled bladder without urethral obstruction, gross reflux.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 253904001 ‐ Megacystis‐megaureter syndrome (disorder) ICD‐10 code and term
‐ Q622 ‐ Congenital megaloureter
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
78
1734 Oligomeganephronia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/246560 ‐ http://omim.org/entry/137920
Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Reduced number of enlarged nephrons. Old ERA‐EDTA PRD code
‐ 61 ‐ Oligomeganephronic hypoplasia SNOMED CT concept identifier and fully specified name
‐ 18417009 ‐ Oligomeganephronic hypoplasia of kidney (disorder) ICD‐10 code and term
‐ Q605 ‐ Renal hypoplasia, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
79
1747 Renal papillary necrosis ‐ cause unknown Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorder SNOMED CT concept identifier and fully specified name
‐ 90241004 ‐ Papillary necrosis (disorder) ICD‐10 code and term
‐ N172 ‐ Acute renal failure with medullary necrosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
80
1752 Acquired obstructive uropathy / nephropathy Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term
‐ N138 ‐ Other obstructive and reflux uropathy OR
‐ N139 ‐ Obstructive and reflux uropathy, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
81
1768 Acquired obstructive nephropathy due to neurogenic bladder Criteria for diagnosis [all to be satisfied]
‐ Clinical exam ‐ Imaging
Additional notes/criteria
‐ The diagnosis is made by imaging plus either clinical examination or urodynamic studies.
Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term
‐ N319 ‐ Neuromuscular dysfunction of bladder, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
82
1775 Obstructive nephropathy due to prostatic hypertrophy Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term
‐ N138 ‐ Other obstructive and reflux uropathy AND N40X ‐ Hyperplasia of prostate
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
83
1781 Obstructive nephropathy due to prostate cancer Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/176807 Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term
‐ N138 ‐ Other obstructive and reflux uropathy AND C61X ‐ Malignant neoplasm of prostate
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
84
1799 Obstructive nephropathy due to bladder cancer Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/109800 Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term
‐ N138 ‐ Other obstructive and reflux uropathy AND C679 ‐ Malignant neoplasm of bladder, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
85
1809 Obstructive nephropathy due to other malignancies Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 522591000000109 ‐ Nephropathy due to acquired urinary tract obstruction (disorder) ICD‐10 code and term
‐ N138 ‐ Other obstructive and reflux uropathy
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
86
1813 Idiopathic retroperitoneal fibrosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/228800 Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 197808006 ‐ Idiopathic retroperitoneal fibrosis (disorder) ICD‐10 code and term
‐ N135 ‐ Kinking and stricture of ureter without hydronephrosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
87
1821 Retroperitoneal fibrosis secondary to malignancies Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Imaging
Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 236017004 ‐ Malignant retroperitoneal fibrosis (disorder) ICD‐10 code and term
‐ N135 ‐ Kinking and stricture of ureter without hydronephrosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
88
3689 Retroperitoneal fibrosis secondary to drugs Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Imaging
Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 236015007 ‐ Drug‐induced retroperitoneal fibrosis (disorder) ICD‐10 code and term
‐ N135 ‐ Kinking and stricture of ureter without hydronephrosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
89
3670 Retroperitoneal fibrosis secondary to peri‐aortitis Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 23 ‐ Pyelonephritis due to acquired obstructive uropathy SNOMED CT concept identifier and fully specified name
‐ 49120005 ‐ Retroperitoneal fibrosis (disorder) ICD‐10 code and term
‐ N135 ‐ Kinking and stricture of ureter without hydronephrosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
90
1832 Calculus nephropathy / urolithiasis Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 25 ‐ Pyelonephritis due to urolithiasis SNOMED CT concept identifier and fully specified name
‐ 95566004 ‐ Urolithiasis (disorder) ICD‐10 code and term
‐ N209 ‐ Urinary calculus, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
91
1845 Calcium oxalate urolithiasis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/167030 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Imaging
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 444717006 ‐ Calcium oxalate urolithiasis (disorder) ICD‐10 code and term
‐ N209 ‐ Urinary calculus, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
92
1850 Enteric hyperoxaluria Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 37497004 ‐ Enteric hyperoxaluria (disorder) ICD‐10 code and term
‐ E748 ‐ Other specified disorders of carbohydrate metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
93
1866 Magnesium ammonium phosphate (struvite) urolithiasis Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Imaging
Additional notes/criteria
‐ Associated with chronic infections with urease producing organisms. Old ERA‐EDTA PRD code
‐ 25 ‐ Pyelonephritis due to urolithiasis SNOMED CT concept identifier and fully specified name
‐ 444690001 ‐ Magnesium ammonium phosphate urolithiasis (disorder) ICD‐10 code and term
‐ N209 ‐ Urinary calculus, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
94
1878 Uric acid urolithiasis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/191700 ‐ http://omim.org/entry/191540
Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Imaging
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 267441009 ‐ Uric acid urolithiasis (disorder) ICD‐10 code and term
‐ M100D ‐ Idiopathic gout
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
95
1884 Tubulointerstitial nephritis ‐ no histology Additional notes/criteria
‐ This PRD can be used in an appropriate clinical setting, if there is convincing evidence of tubular dysfunction, e.g. Fanconi syndrome, tubular proteinuria.
Old ERA‐EDTA PRD code
‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name
‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
96
1897 Tubulointerstitial nephritis ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name
‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
97
1907 Familial interstitial nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/161900 about progression of renal failure Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Gene test
Additional notes/criteria
‐ This PRD may be used if the diagnosis has been confirmed with a renal histology in at least one affected family member.
‐ This PRD should not be used for familial nephropathies which have a more accurate PRD.
‐ A genetic test may help to make the diagnosis but is not mandatory for this PRD. Old ERA‐EDTA PRD code
‐ 50 ‐ Hereditary/Familial nephropathy ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 28689008 ‐ Interstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
98
1911 Familial interstitial nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/161900 about progression of renal failure Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history ‐ Gene test
Additional notes/criteria
‐ This PRD should not be used for familial nephropathies which have a more accurate PRD.
Old ERA‐EDTA PRD code
‐ 50 ‐ Hereditary/Familial nephropathy ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 28689008 ‐ Interstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
99
1924 Tubulointerstitial nephritis associated with autoimmune disease ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ A genetic test may help to make the diagnosis but is not mandatory for this PRD. Old ERA‐EDTA PRD code
‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name
‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND M359 ‐ Systemic involvement of connective tissue, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
100
1930 Tubulointerstitial nephritis associated with autoimmune disease ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name
‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND M359 ‐ Systemic involvement of connective tissue, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
101
1948 Tubulointerstitial nephritis with uveitis (TINU) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/607665 Criteria for diagnosis [all to be satisfied]
‐ Clinical exam Additional notes/criteria
‐ To use this PRD, there must be evidence of past or current uveitis. Old ERA‐EDTA PRD code
‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name
‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND H209 ‐ Iridocyclitis, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
102
1953 Tubulointerstitial nephritis with uveitis (TINU) ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/607665 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical exam
Additional notes/criteria
‐ To use this PRD, there must be evidence of past or current uveitis. Old ERA‐EDTA PRD code
‐ 30 ‐ Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified SNOMED CT concept identifier and fully specified name
‐ 428255004 ‐ Tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N12X ‐ Tubulo‐interstitial nephritis not spec as acute or chronic AND H209 ‐ Iridocyclitis, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
103
1969 Renal sarcoidosis ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/181000 Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ Any standard method of diagnosing sarcoid is acceptable. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 37061001 ‐ Granulomatous sarcoid nephropathy (disorder) ICD‐10 code and term
‐ D868D ‐ Sarcoidosis of other and combined sites
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
104
1976 Renal sarcoidosis ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/181000 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ Histology from non renal tissue is acceptable. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 37061001 ‐ Granulomatous sarcoid nephropathy (disorder) ICD‐10 code and term
‐ D868D ‐ Sarcoidosis of other and combined sites
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
105
1982 Aristolochic acid nephropathy (Balkan / Chinese herb / endemic nephropathy) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/124100 Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 94 ‐ Balkan nephropathy SNOMED CT concept identifier and fully specified name
‐ 236514003 ‐ Toxic nephropathy (disorder) ICD‐10 code and term
‐ N144 ‐ Toxic nephropathy, not elsewhere classified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
106
1995 Aristolochic acid nephropathy (Balkan / Chinese herb / endemic nephropathy) ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/124100 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ Compatible renal histopathology evidence is required to use this PRD but alone is insufficient because the appearances are not diagnostic.
‐ A clinical history consistent with exposure to Aristolochia or residence in an area in which this condition is endemic is also necessary.
Old ERA‐EDTA PRD code
‐ 94 ‐ Balkan nephropathy SNOMED CT concept identifier and fully specified name
‐ 236514003 ‐ Toxic nephropathy (disorder) ICD‐10 code and term
‐ N144 ‐ Toxic nephropathy, not elsewhere classified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
107
2005 Drug‐induced tubulointerstitial nephritis ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 439990003 ‐ Drug‐induced tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N142 ‐ Nephropathy induced by unspec drug medicament or biol subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
108
2014 Drug‐induced tubulointerstitial nephritis ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 439990003 ‐ Drug‐induced tubulointerstitial nephritis (disorder) ICD‐10 code and term
‐ N142 ‐ Nephropathy induced by unspec drug medicament or biol subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
109
2022 Nephropathy due to analgesic drugs ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Imaging
Old ERA‐EDTA PRD code
‐ 31 ‐ Nephropathy (interstitial) due to analgesic drugs SNOMED CT concept identifier and fully specified name
‐ 59400006 ‐ Analgesic nephropathy (disorder) ICD‐10 code and term
‐ N140 ‐ Analgesic nephropathy
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
110
2033 Nephropathy due to analgesic drugs ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Imaging
Old ERA‐EDTA PRD code
‐ 31 ‐ Nephropathy (interstitial) due to analgesic drugs SNOMED CT concept identifier and fully specified name
‐ 59400006 ‐ Analgesic nephropathy (disorder) ICD‐10 code and term
‐ N140 ‐ Analgesic nephropathy
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
111
2046 Nephropathy due to ciclosporin ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 33 ‐ Nephropathy (interstitial) due to cyclosporin A SNOMED CT concept identifier and fully specified name
‐ 519481000000106 ‐ Nephropathy induced by ciclosporin (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
112
2051 Nephropathy due to ciclosporin ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 33 ‐ Nephropathy (interstitial) due to cyclosporin A SNOMED CT concept identifier and fully specified name
‐ 519481000000106 ‐ Nephropathy induced by ciclosporin (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
113
2067 Nephropathy due to tacrolimus ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519491000000108 ‐ Nephropathy induced by tacrolimus (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
114
2079 Nephropathy due to tacrolimus ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519491000000108 ‐ Nephropathy induced by tacrolimus (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
115
2080 Nephropathy due to aminoglycosides ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519501000000102 ‐ Nephropathy induced by aminoglycosides (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
116
2098 Nephropathy due to aminoglycosides ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519501000000102 ‐ Nephropathy induced by aminoglycosides (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
117
2108 Nephropathy due to amphotericin ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519511000000100 ‐ Nephropathy induced by amphotericin (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
118
2112 Nephropathy due to amphotericin ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519511000000100 ‐ Nephropathy induced by amphotericin (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
119
2120 Nephropathy due to cisplatin ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 32 ‐ Nephropathy (interstitial) due to cis‐platinum SNOMED CT concept identifier and fully specified name
‐ 53556002 ‐ Cis‐platinum nephropathy (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
II TUBULOINTERSTITIAL DISEASE
120
2131 Nephropathy due to cisplatin ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 32 ‐ Nephropathy (interstitial) due to cis‐platinum SNOMED CT concept identifier and fully specified name
‐ 53556002 ‐ Cis‐platinum nephropathy (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
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2149 Nephropathy due to lithium ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 4390004 ‐ Lithium nephropathy (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
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2154 Nephropathy due to lithium ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 39 ‐ Drug induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 4390004 ‐ Lithium nephropathy (disorder) ICD‐10 code and term
‐ N141 ‐ Nephropathy induc by other drugs meds and biolog subs
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2165 Lead induced nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ To use this PRD there should be biochemical evidence of chronic lead intoxication (e.g. EDTA chelation) plus hyperuricaemia and hypertension.
‐ A single measurement of blood lead is insufficient. Old ERA‐EDTA PRD code
‐ 34 ‐ Lead induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519521000000106 ‐ Nephropathy induced by lead (disorder) ICD‐10 code and term
‐ N143 ‐ Nephropathy induced by heavy metals
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2177 Lead induced nephropathy ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Additional notes/criteria
‐ To use this PRD there should be biochemical evidence of chronic lead intoxication (e.g. EDTA chelation) plus hyperuricaemia and hypertension.
‐ A single measurement of blood lead is insufficient. Old ERA‐EDTA PRD code
‐ 34 ‐ Lead induced nephropathy (interstitial) SNOMED CT concept identifier and fully specified name
‐ 519521000000106 ‐ Nephropathy induced by lead (disorder) ICD‐10 code and term
‐ N143 ‐ Nephropathy induced by heavy metals
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2183 Acute urate nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/191700 ‐ http://omim.org/entry/191540
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 92 ‐ Gout SNOMED CT concept identifier and fully specified name
‐ 236496000 ‐ Acute urate nephropathy (disorder) ICD‐10 code and term
‐ E790 ‐ Hyperuricaem without sign inflamm arthritis+tophaceous dis
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2196 Acute urate nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/191700 ‐ http://omim.org/entry/191540
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 92 ‐ Gout SNOMED CT concept identifier and fully specified name
‐ 236496000 ‐ Acute urate nephropathy (disorder) ICD‐10 code and term
‐ E790 ‐ Hyperuricaem without sign inflamm arthritis+tophaceous dis
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3636 Chronic urate nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/191700 ‐ http://omim.org/entry/191540
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 92 ‐ Gout SNOMED CT concept identifier and fully specified name
‐ 190829000 ‐ Chronic urate nephropathy (disorder) ICD‐10 code and term
‐ M100D ‐ Idiopathic gout
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2203 Chronic urate nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/191700 ‐ http://omim.org/entry/191540
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ Requires the presence of parenchymal kidney damage resulting from urate deposits. ‐ Should be distinguished from Uromodulin‐associated nephropathy (PRD 2827).
Old ERA‐EDTA PRD code
‐ 92 ‐ Gout SNOMED CT concept identifier and fully specified name
‐ 190829000 ‐ Chronic urate nephropathy (disorder) ICD‐10 code and term
‐ M100D ‐ Idiopathic gout
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3662 Hypercalcaemic nephropathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/143880 ‐ http://omim.org/entry/145000
Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ Use a more accurate PRD if appropriate. Old ERA‐EDTA PRD code
‐ 93 ‐ Nephrocalcinosis and hypercalcaemic nephropathy SNOMED CT concept identifier and fully specified name
‐ 33763006 ‐ Hypercalcemic nephropathy (disorder) ICD‐10 code and term
‐ E835 ‐ Disorders of calcium metabolism
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2219 Radiation nephritis Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ To use this PRD, there should be a history of radiation with the radiation field including the kidneys.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 7725007 ‐ Radiation nephritis (disorder) ICD‐10 code and term
‐ T66X ‐ Unspecified effects of radiation
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2226 Renal / perinephric abscess Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 3321001 ‐ Renal abscess (disorder) OR
‐ 80640009 ‐ Perirenal abscess (disorder) ICD‐10 code and term
‐ N151 ‐ Renal and perinephric abscess
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2235 Renal tuberculosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/607948 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Imaging
Additional notes/criteria
‐ A diagnosis of past or present tuberculosis of the kidney must have been made. Old ERA‐EDTA PRD code
‐ 91 ‐ Tuberculosis SNOMED CT concept identifier and fully specified name
‐ 44323002 ‐ Tuberculosis of kidney (disorder) ICD‐10 code and term
‐ A181D ‐ Tuberculosis of genitourinary system
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2242 Leptospirosis Additional notes/criteria
‐ A diagnosis of leptospirosis must have been made. The diagnosis is normally supported by serology, culture or PCR.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 77377001 ‐ Leptospirosis (disorder) ICD‐10 code and term
‐ A279 ‐ Leptospirosis, unspecified
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2257 Hantavirus nephropathy Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ Diagnosis of hantavirus infection should have been confirmed by PCR. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 102455002 ‐ Hemorrhagic nephroso‐nephritis (disorder) ICD‐10 code and term
‐ A985D ‐ Haemorrhagic fever with renal syndrome
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2261 Xanthogranulomatous pyelonephritis Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 38898003 ‐ Xanthogranulomatous pyelonephritis (disorder) ICD‐10 code and term
‐ N119 ‐ Chronic tubulo‐interstitial nephritis, unspecified
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2274 Nephropathy related to HIV ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/609423 ‐ http://omim.org/entry/612551 ‐ http://omim.org/entry/607832
Additional notes/criteria
‐ This PRD requires evidence of HIV infection. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 90708001 ‐ Kidney disease (disorder) ICD‐10 code and term
‐ N289 ‐ Disorder of kidney and ureter, unspecified AND B24X ‐ Unspecified human immunodefiency virus [HIV] disease
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2288 Nephropathy related to HIV ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/609423 ‐ http://omim.org/entry/612551 ‐ http://omim.org/entry/607832
Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 90708001 ‐ Kidney disease (disorder) ICD‐10 code and term
‐ N289 ‐ Disorder of kidney and ureter, unspecified AND B24X ‐ Unspecified human immunodefiency virus [HIV] disease
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2290 Schistosomiasis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/181460 Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ This PRD requires microbiological evidence of urinary tract infection with Schistosomiasis.
‐ This PRD should not be used for immune complex nephropathy secondary to Schistosomiasis.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 236706006 ‐ Urinary schistosomiasis (disorder) ICD‐10 code and term
‐ B650D ‐ Schistosom due Schis haematobium [urin schistosom]
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2300 Other specific infection Additional notes/criteria
‐ This PRD requires evidence of infection with the putative organism. ‐ This PRD should only be used if there is evidence of infection directly causing renal
impairment not listed elsewhere. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 40733004 ‐ Infectious disease (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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2316 Diabetic nephropathy in type I ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/222100 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ A diagnosis of type I diabetes mellitus must have been made. ‐ For a diagnosis of diabetic nephropathy without evidence from renal histopathology,
proteinuria must have been documented at some point in the patient's history. ‐ A PRD of diabetic nephropathy is not mandatory in the presence of DM with
proteinuria and alternative diagnoses can be considered. ‐ In the absence of renal histopathology the differential diagnosis will include 'Chronic
kidney disease (CKD) / chronic renal failure (CRF) aetiology uncertain / unknown (with or without histology)', Ischaemic nephropathy, renovascular disease and atheroembolic renal disease.
Old ERA‐EDTA PRD code
‐ 80 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type I SNOMED CT concept identifier and fully specified name
‐ 421893009 ‐ Renal disorder associated with type I diabetes mellitus (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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2328 Diabetic nephropathy in type I diabetes ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/222100 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Additional notes/criteria
‐ A diagnosis of type I diabetes mellitus must have been made. ‐ Histopathological features most compatible with diabetic nephropathy must be
present. Old ERA‐EDTA PRD code
‐ 80 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type I SNOMED CT concept identifier and fully specified name
‐ 421893009 ‐ Renal disorder associated with type I diabetes mellitus (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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2337 Diabetic nephropathy in type II diabetes ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/125853 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ A diagnosis of type II diabetes mellitus must have been made. ‐ For a diagnosis of diabetic nephropathy, proteinuria must have been documented at
some point in the patient's history. ‐ A PRD of diabetic nephropathy is not mandatory in the presence of DM with
proteinuria and alternative diagnoses can be considered. ‐ In the absence of renal histology the differential diagnosis will include 'Chronic kidney
disease (CKD) / chronic renal failure (CRF) aetiology uncertain / unknown (with or without histology)', ischaemic nephropathy, renovascular disease and atheroembolic renal disease.
‐ Distinguish from: Inherited / genetic diabetes mellitus type II. Old ERA‐EDTA PRD code
‐ 81 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type II SNOMED CT concept identifier and fully specified name
‐ 420279001 ‐ Renal disorder associated with type II diabetes mellitus (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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2344 Diabetic nephropathy in type II diabetes ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/125853 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Additional notes/criteria
‐ A diagnosis of type II diabetes mellitus must have been made. ‐ Histopathological features most compatible with diabetic nephropathy must be
present. Old ERA‐EDTA PRD code
‐ 81 ‐ Diabetes glomerulosclerosis or diabetic nephropathy ‐ Type II SNOMED CT concept identifier and fully specified name
‐ 420279001 ‐ Renal disorder associated with type II diabetes mellitus (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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2359 Chronic hypertensive nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/145500 about essential hypertension ‐ http://omim.org/entry/161900 about progression of renal failure
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam
Additional notes/criteria
‐ The patient must have a history of hypertension. ‐ Other PRDs will usually have been considered before accepting this PRD.
Old ERA‐EDTA PRD code
‐ 72 ‐ Renal vascular disease due to hypertension SNOMED CT concept identifier and fully specified name
‐ 38481006 ‐ Hypertensive renal disease (disorder) ICD‐10 code and term
‐ I120 ‐ Hypertensive renal disease with renal failure
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2363 Chronic hypertensive nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/145500 about essential hypertension ‐ http://omim.org/entry/161900 about progression of renal failure
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Clinical exam
Old ERA‐EDTA PRD code
‐ 72 ‐ Renal vascular disease due to hypertension SNOMED CT concept identifier and fully specified name
‐ 38481006 ‐ Hypertensive renal disease (disorder) ICD‐10 code and term
‐ I120 ‐ Hypertensive renal disease with renal failure
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2371 Malignant hypertensive nephropathy / accelerated hypertensive nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical exam ‐ Biochemistry
Additional notes/criteria
‐ Lacks evidence of any other PRD responsible for the hypertension and renal failure. Old ERA‐EDTA PRD code
‐ 71 ‐ Renal vascular disease due to malignant hypertension SNOMED CT concept identifier and fully specified name
‐ 65443008 ‐ Malignant hypertensive renal disease (disorder) ICD‐10 code and term
‐ I120 ‐ Hypertensive renal disease with renal failure
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2385 Malignant hypertensive nephropathy / accelerated hypertensive nephropathy ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical exam
Additional notes/criteria
‐ Lacks evidence of systemic sclerosis. Old ERA‐EDTA PRD code
‐ 71 ‐ Renal vascular disease due to malignant hypertension SNOMED CT concept identifier and fully specified name
‐ 65443008 ‐ Malignant hypertensive renal disease (disorder) ICD‐10 code and term
‐ I120 ‐ Hypertensive renal disease with renal failure
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2392 Ageing kidney ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/502000 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Imaging
Additional notes/criteria
‐ If the patient develops stage 4 CKD, consider a PRD of ischaemic nephropathy. Old ERA‐EDTA PRD code
‐ 70 ‐ Renal vascular disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 445108007 ‐ Age related reduction of renal function (finding) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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2407 Ischaemic nephropathy ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ Lacks evidence of any other PRD responsible for the hypertension and renal failure. Old ERA‐EDTA PRD code
‐ 75 ‐ Ischaemic renal disease/cholesterol embolism SNOMED CT concept identifier and fully specified name
‐ 519581000000107 ‐ Ischaemic nephropathy (disorder) ICD‐10 code and term
‐ N280 ‐ Ischaemia and infarction of kidney
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2411 Ischaemic nephropathy / microvascular disease ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ Lacks evidence of any other PRD responsible for the hypertension and renal failure. Old ERA‐EDTA PRD code
‐ 75 ‐ Ischaemic renal disease/cholesterol embolism SNOMED CT concept identifier and fully specified name
‐ 519581000000107 ‐ Ischaemic nephropathy (disorder) ICD‐10 code and term
‐ N280 ‐ Ischaemia and infarction of kidney
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2424 Renal artery stenosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/135580 ‐ http://omim.org/entry/108725
Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 70 ‐ Renal vascular disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 302233006 ‐ Renal artery stenosis (disorder) ICD‐10 code and term
‐ I701 ‐ Atherosclerosis of renal artery
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2430 Atheroembolic renal disease ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/108725 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Imaging
Additional notes/criteria
‐ Eosinophilia or complement consumption are suggestive. Old ERA‐EDTA PRD code
‐ 75 ‐ Ischaemic renal disease/cholesterol embolism SNOMED CT concept identifier and fully specified name
‐ 51677000 ‐ Atheroembolism of renal arteries (disorder) ICD‐10 code and term
‐ I701 ‐ Atherosclerosis of renal artery
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2448 Atheroembolic renal disease ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/108725 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 75 ‐ Ischaemic renal disease/cholesterol embolism SNOMED CT concept identifier and fully specified name
‐ 51677000 ‐ Atheroembolism of renal arteries (disorder) ICD‐10 code and term
‐ I701 ‐ Atherosclerosis of renal artery
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2453 Fibromuscular dysplasia of renal artery Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/135580 Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 70 ‐ Renal vascular disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 2900003 ‐ Hyperplasia of renal artery (disorder) ICD‐10 code and term
‐ I773 ‐ Arterial fibromuscular dysplasia
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2469 Renal arterial thrombosis / occlusion Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/188050 Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ A source of emboli or a hypercoagulable state are usually identified. Old ERA‐EDTA PRD code
‐ 70 ‐ Renal vascular disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 236488005 ‐ Renal artery occlusion (disorder) ICD‐10 code and term
‐ N280 ‐ Ischaemia and infarction of kidney
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2476 Renal vein thrombosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/188050 Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ If there is an underlying nephrotic syndrome causing the thrombosis, the underlying renal diagnosis should take precedence.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 15842009 ‐ Thrombosis of renal vein (disorder) ICD‐10 code and term
‐ I823 ‐ Embolism and thrombosis of renal vein
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2482 Cardiorenal syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/232200 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ Intractable heart failure with severe renal impairment without evidence of parenchymal kidney disease or renal vascular disease.
‐ Corresponds to CardioRenal Syndrome type 2 as described by the Acute Dialysis Quality Initiative group (Ronco C et al J Am Coll Cardiol 2008; 52; 1527‐1539).
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 445236007 ‐ Cardiorenal syndrome (disorder) ICD‐10 code and term
‐ I139 ‐ Hypertensive heart and renal disease, unspecified
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2495 Hepatorenal syndrome Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ A renal biopsy is not normally done in this clinical situation. ‐ Renal histology is essentially normal. ‐ Patients with liver disease are predisposed to IgA nephropathy and where this is
found, the alternative PRD 'IgA nephropathy secondary to liver cirrhosis' should be considered.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 51292008 ‐ Hepatorenal syndrome (disorder) ICD‐10 code and term
‐ K767 ‐ Hepatorenal syndrome
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159
2509 Renal amyloidosis Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Histological proof of amyloid from another tissue is an adequate substitute for renal histology.
Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 48713002 ‐ Amyloid nephropathy (disorder) ICD‐10 code and term
‐ E854 ‐ Organ‐limited amyloidosis
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2513 AA amyloid secondary to chronic inflammation Criteria for diagnosis [all to be satisfied]
‐ Histology Additional notes/criteria
‐ Histological proof of amyloid from another tissue is an adequate substitute for renal histology.
Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 274945004 ‐ AA amyloidosis (disorder) ICD‐10 code and term
‐ E853 ‐ Secondary systemic amyloidosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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2521 AL amyloid secondary to plasma cell dyscrasia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/254500 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Additional notes/criteria
‐ There should be evidence of a monoclonal light chain (e.g. biochemical or immunological).
‐ Histological proof of amyloid from another tissue is a substitute for renal histology. Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 23132008 ‐ AL amyloidosis (disorder) ICD‐10 code and term
‐ E859 ‐ Amyloidosis, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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162
2532 Familial amyloid secondary to protein mutations ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/105200 Criteria for diagnosis [all to be satisfied]
‐ Family history Additional notes/criteria
‐ Amyloid should have been demonstrated histologically in at least one affected family member.
Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 66451004 ‐ Familial visceral amyloidosis, Ostertag type (disorder) ICD‐10 code and term
‐ E850 ‐ Non‐neuropathic heredofamilial amyloidosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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163
2545 Familial amyloid secondary to protein mutations ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/105200 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history
Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 66451004 ‐ Familial visceral amyloidosis, Ostertag type (disorder) ICD‐10 code and term
‐ E850 ‐ Non‐neuropathic heredofamilial amyloidosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
164
2550 Familial AA amyloid secondary to familial Mediterranean fever / TRAPS (Hibernian fever) ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/142680; for background information see: ‐ http://omim.org/entry/608107
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 367528006 ‐ Amyloid of familial Mediterranean fever (disorder) ICD‐10 code and term
‐ E850 ‐ Non‐neuropathic heredofamilial amyloidosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
165
2566 Familial AA amyloid secondary to familial Mediterranean fever / TRAPS (Hibernian fever) ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/142680; for background information see: ‐ http://omim.org/entry/608107
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 83 ‐ Amyloid SNOMED CT concept identifier and fully specified name
‐ 367528006 ‐ Amyloid of familial Mediterranean fever (disorder) ICD‐10 code and term
‐ E850 ‐ Non‐neuropathic heredofamilial amyloidosis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
166
2578 Myeloma kidney ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/254500 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ A diagnosis of myeloma must have been made with biochemical, haematological or immunological confirmation.
Old ERA‐EDTA PRD code
‐ 82 ‐ Myelomatosis / light chain deposit disease SNOMED CT concept identifier and fully specified name
‐ 32278006 ‐ Myeloma kidney (disorder) ICD‐10 code and term
‐ C900D ‐ Multiple myeloma
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
167
2584 Myeloma cast nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/254500 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Additional notes/criteria
‐ A diagnosis of myeloma must have been made with biochemical, haematological or immunological confirmation.
Old ERA‐EDTA PRD code
‐ 82 ‐ Myelomatosis / light chain deposit disease SNOMED CT concept identifier and fully specified name
‐ 32278006 ‐ Myeloma kidney (disorder) ICD‐10 code and term
‐ C900D ‐ Multiple myeloma
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
168
2597 Light chain deposition disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/254500 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry
Additional notes/criteria
‐ A diagnosis of light chain disease must have been made with biochemical, haematological or immunological confirmation.
Old ERA‐EDTA PRD code
‐ 82 ‐ Myelomatosis / light chain deposit disease SNOMED CT concept identifier and fully specified name
‐ 373604002 ‐ Light chain deposition disease (disorder) ICD‐10 code and term
‐ D808 ‐ Other immunodeficiencies with predominantly antibody defects
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
169
2606 Immunotactoid / fibrillary nephropathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/137950 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 73305009 ‐ Fibrillary glomerulonephritis (disorder) ICD‐10 code and term
‐ N059 ‐ Unspecified nephritic syndrome, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
170
2610 Haemolytic uraemic syndrome (HUS) ‐ diarrhoea associated Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ This PRD is usually associated with E. coli 0157 infection. Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 373421000 ‐ Diarrhea‐associated hemolytic uremic syndrome (disorder) ICD‐10 code and term
‐ D593 ‐ Haemolytic‐uraemic syndrome
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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171
2623 Atypical haemolytic uraemic syndrome (HUS) ‐ diarrhoea negative Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/235400 about AHUS1 ‐ http://omim.org/entry/612922 about AHUS2 ‐ http://omim.org/entry/612923 about AHUS3 ‐ http://omim.org/entry/612924 about AHUS4 ‐ http://omim.org/entry/612925 about AHUS5 ‐ http://omim.org/entry/612926 about AHUS6
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ If there is evidence of a genetic mutation, or of a family history of haemolytic uraemic syndrome, then the code 'Congenital haemolytic uraemic syndrome' or 'Familial haemolytic uraemic syndrome' should be used.
Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 373422007 ‐ Diarrhea‐negative hemolytic uremic syndrome (disorder) ICD‐10 code and term
‐ D593 ‐ Haemolytic‐uraemic syndrome
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
172
2634 Thrombotic thrombocytopenic purpura (TTP) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/274150 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ ADAMTS13 deficiency or autoantibodies to ADAMTS13 may be demonstrated (not mandatory).
Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 78129009 ‐ Thrombotic thrombocytopenic purpura (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
173
2647 Haemolytic uraemic syndrome (HUS) secondary to systemic disease Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ This PRD includes HUS secondary to drugs or malignancies. Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 111407006 ‐ Hemolytic uremic syndrome (disorder) ICD‐10 code and term
‐ D593 ‐ Haemolytic‐uraemic syndrome
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
174
2652 Congenital haemolytic uraemic syndrome (HUS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/274150 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 444976001 ‐ Congenital hemolytic uremic syndrome (disorder) ICD‐10 code and term
‐ D593 ‐ Haemolytic‐uraemic syndrome
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
175
2668 Familial haemolytic uraemic syndrome (HUS) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/274150 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ This PRD includes complement factor H or I abnormalities. Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 373420004 ‐ Upshaw‐Schulman syndrome (disorder) ICD‐10 code and term
‐ M311 ‐ Thrombotic microangiopathy
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
176
2675 Familial thrombotic thrombocytopenic purpura (TTP) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/274150 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Gene test
Additional notes/criteria
‐ ADAMTS13 deficiency (not mandatory). Old ERA‐EDTA PRD code
‐ 88 ‐ Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome) SNOMED CT concept identifier and fully specified name
‐ 373420004 ‐ Upshaw‐Schulman syndrome (disorder) ICD‐10 code and term
‐ M311 ‐ Thrombotic microangiopathy
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
177
2681 Nephropathy due to pre‐eclampsia / eclampsia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/189800 about PEE1 ‐ http://omim.org/entry/609402 about PEE2 ‐ http://omim.org/entry/609403 about PEE3 ‐ http://omim.org/entry/609404 about PEE4
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 90708001 ‐ Kidney disease (disorder) ICD‐10 code and term
‐ N289 ‐ Disorder of kidney and ureter, unspecified AND O159 ‐ Eclampsia, unspecified as to time period OR
‐ N289 ‐ Disorder of kidney and ureter, unspecified AND O149 ‐ Pre‐eclampsia, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
178
2699 Sickle cell nephropathy ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603903 Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 13886001 ‐ Sickle cell nephropathy (disorder) ICD‐10 code and term
‐ D571D ‐ Sickle‐cell anaemia without crisis
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
III SYSTEMIC DISEASE AFFECTING THE KIDNEY
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
179
2702 Sickle cell nephropathy ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/603903 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 13886001 ‐ Sickle cell nephropathy (disorder) ICD‐10 code and term
‐ D571D ‐ Sickle‐cell anaemia without crisis
IV FAMILIAL / HEREDITARY NEPHROPATHIES
180
2718 Autosomal dominant (AD) polycystic kidney disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/173900 about PKD1 ‐ http://omim.org/entry/613095 about PKD2 ‐ http://omim.org/entry/600666 about PKD3
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 41 ‐ Polycystic kidneys; adult type (dominant) SNOMED CT concept identifier and fully specified name
‐ 28728008 ‐ Polycystic kidney disease, adult type (disorder) ICD‐10 code and term
‐ Q612 ‐ Polycystic kidney, adult type
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
181
2725 Autosomal dominant (AD) polycystic kidney disease type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/173900 about PKD1 ‐ http://omim.org/entry/601313 about polycystin 1 ‐ http://omim.org/entry/606702 about PKDH1
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 41 ‐ Polycystic kidneys; adult type (dominant) SNOMED CT concept identifier and fully specified name
‐ 253878003 ‐ Adult type polycystic kidney disease type 1 (disorder) ICD‐10 code and term
‐ Q612 ‐ Polycystic kidney, adult type
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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182
2739 Autosomal dominant (AD) polycystic kidney disease type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/613095 about PKD2 ‐ http://omim.org/entry/173910 about polycystin 2
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 41 ‐ Polycystic kidneys; adult type (dominant) SNOMED CT concept identifier and fully specified name
‐ 253879006 ‐ Adult type polycystic kidney disease type 2 (disorder) ICD‐10 code and term
‐ Q612 ‐ Polycystic kidney, adult type
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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183
2741 Autosomal recessive (AR) polycystic kidney disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/263200 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 42 ‐ Polycystic kidneys; infantile (recessive) SNOMED CT concept identifier and fully specified name
‐ 28770003 ‐ Polycystic kidney disease, infantile type (disorder) ICD‐10 code and term
‐ Q611 ‐ Polycystic kidney, infantile type
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
184
2756 Alport syndrome ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/301050 about alport syndrome, X‐ linked; ATS ‐ http://omim.org/entry/303630 about collagen, type IV, alpha‐5; COL4A5 ‐ http://omim.org/entry/203780 about alport syndrome, autosomal recessive ‐ http://omim.org/entry/104200 about alport syndrome, autosomal dominant ‐ http://omim.org/entry/308940 about leiomyomatosis, diffuse, with alport syndrome;
DL‐ATS ‐ http://omim.org/entry/300194 about alport syndrome, mental retardation, midface
hypoplasia, and elliptocytosis for background information see: ‐ http://omim.org/entry/609469 about nephropathy, progressive, with deafness
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test
Old ERA‐EDTA PRD code
‐ 51 ‐ Hereditary nephritis with nerve deafness (Alport's Syndrome) SNOMED CT concept identifier and fully specified name
‐ 399340005 ‐ Hereditary nephritis (disorder) ICD‐10 code and term
‐ N079 ‐ Unspecified morphological changes
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
185
2760 Alport syndrome ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/301050 about alport syndrome, X‐ linked; ATS ‐ http://omim.org/entry/303630 about collagen, type IV, alpha‐5; COL4A5 ‐ http://omim.org/entry/203780 about alport syndrome, autosomal recessive ‐ http://omim.org/entry/104200 about alport syndrome, autosomal dominant ‐ http://omim.org/entry/308940 about leiomyomatosis, diffuse, with alport syndrome;
DL‐ATS ‐ http://omim.org/entry/300194 about alport syndrome, mental retardation, midface
hypoplasia, and elliptocytosis for background information see: ‐ http://omim.org/entry/609469 about nephropathy, progressive, with deafness
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test
Old ERA‐EDTA PRD code
‐ 51 ‐ Hereditary nephritis with nerve deafness (Alport's Syndrome) SNOMED CT concept identifier and fully specified name
‐ 399340005 ‐ Hereditary nephritis (disorder) ICD‐10 code and term
‐ N079 ‐ Unspecified morphological changes
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
186
2773 Benign familial haematuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/141200 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Urine analysis
Additional notes/criteria
‐ The clinical history can be useful because it does NOT suggest any other nephropathy. ‐ Haematuria can be demonstrated by any means e.g. dip stick or urine microscopy.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 236421001 ‐ Benign familial hematuria (disorder) ICD‐10 code and term
‐ N029 ‐ Recurrent and persistent haematuria, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
187
2787 Thin basement membrane disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/141200 Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 236418003 ‐ Thin basement membrane disease (disorder) ICD‐10 code and term
‐ N050 ‐ Unspecified nephritic syndrome, minor glomerular abnormality
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
188
2794 Cystic kidney disease Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 40 ‐ Cystic kidney disease ‐ type unspecified SNOMED CT concept identifier and fully specified name
‐ 82525005 ‐ Congenital cystic kidney disease (disorder) ICD‐10 code and term
‐ Q619 ‐ Cystic kidney disease, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
189
2804 Medullary cystic kidney disease type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/174000 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Urine analysis ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Usually autosomal dominant family history, bland urine and without heavy proteinuria.
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 444699000 ‐ Medullary cystic kidney disease type 1 (disorder) ICD‐10 code and term
‐ Q615 ‐ Medullary cystic kidney
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
190
2815 Medullary cystic kidney disease type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/603860 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Genetically identical to uromodulin‐associated nephropathy, but characterised by presence of cysts plus gout.
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 445503007 ‐ Medullary cystic kidney disease type 2 (disorder) ICD‐10 code and term
‐ Q615 ‐ Medullary cystic kidney
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
191
2827 Uromodulin‐associated nephropathy (familial juvenile hyperuricaemic nephropathy) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/162000 about HNFJ1 ‐ http://omim.org/entry/613092 about HNFJ2 ‐ http://omim.org/entry/614227 about HNFJ3 for background information see: ‐ http://omim.org/entry/191845 about uromodulin
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Autosomal dominant inheritance, high incidence of gout, but no cysts. Old ERA‐EDTA PRD code
‐ 49 ‐ Cystic kidney disease ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 46785007 ‐ Familial juvenile gout (disorder) ICD‐10 code and term
‐ E798 ‐ Other disorders of purine and pyrimidine metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
192
2836 Nephronophthisis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/609254 about senior‐loken syndrome 5; SLSN5 and NPHP ‐ http://omim.org/entry/612013 about coiled‐coil and C2 domains‐containing protein
2A; CC2D2A ‐ http://omim.org/entry/613159 about NPHPL1 ‐ http://omim.org/entry/609237 about IQ motif‐containing protein B1; IQCB1 =
nephrocystin 5; NPHP5 ‐ http://omim.org/entry/610142 about centrosomal protein, 290‐KD; CEP290 =
nephrocystin 6; NPHP6 ‐ http://omim.org/entry/611498 about NPHP7 ‐ http://omim.org/entry/610937 about RPGRIP1‐like; RPGRIP1L = nephrocystin 8;
NPHP8 ‐ http://omim.org/entry/613824 about NPHP9 ‐ http://omim.org/entry/613524 about serologically defined colon cancer antigen 8;
SDCCAG8 = NPHP10 gene ‐ http://omim.org/entry/613550 about NPHP11 ‐ http://omim.org/entry/613820 about NPHP12
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 204958008 ‐ Nephronophthisis (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
193
2843 Nephronophthisis ‐ type 1 (juvenile type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/256100 about NPHP1 ‐ http://omim.org/entry/607100 about nephrocystin 1; NPHP
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 444830001 ‐ Juvenile nephronophthisis (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
194
2858 Nephronophthisis ‐ type 2 (infantile type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/602088 about NPHP2 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 444558002 ‐ Infantile nephronophthisis (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
195
2862 Nephronophthisis ‐ type 3 (adolescent type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/604387 about NPHP3 ‐ http://omim.org/entry/608002 about nephrocystin 3; NPHP3
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 444749006 ‐ Adolescent nephronophthisis (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
196
2870 Nephronophthisis ‐ type 4 (juvenile type) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/606966 about NPHP4 ‐ http://omim.org/entry/607215 about nephrocystin 4; NPHP4
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 446989009 ‐ Nephronophthisis type 4 (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
197
2889 Nephronophthisis ‐ type 5 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 446991001 ‐ Nephronophthisis type 5 (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
198
2891 Nephronophthisis ‐ type 6 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Gene test
Old ERA‐EDTA PRD code
‐ 43 ‐ Medullary cystic disease; including nephronophthisis SNOMED CT concept identifier and fully specified name
‐ 447335007 ‐ Nephronophthisis type 6 (disorder) ICD‐10 code and term
‐ Q618 ‐ Other cystic kidney diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
199
2901 Primary Fanconi syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/134600 about FRTS1 ‐ http://omim.org/entry/613388 about FRTS2
Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Additional notes/criteria
‐ Clinical syndrome associated with genetic mutations. Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 236466005 ‐ Congenital Fanconi syndrome (disorder) ICD‐10 code and term
‐ E720 ‐ Disorders of amino‐acid transport
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
200
2917 Tubular disorder as part of inherited metabolic diseases Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/551200 about chronic tubulointerstitial nephropathy Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 197744007 ‐ Renal tubulo‐interstitial disorders in metabolic diseases (disorder) ICD‐10 code and term
‐ N163A ‐ Renal tubulo‐interstitial disorders in metabolic diseases
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
201
2929 Dent disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/300009 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ X linked recessive nephrolithiasis + mutation in gene CLCN5. ‐ Clinical: low MW proteinuria, raised urinary calcium:creatinine ratio. ‐ May have nephrocalcinosis, nephrolithiasis, hypophosphatemic rickets.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 444645005 ‐ Dent's disease (disorder) ICD‐10 code and term
‐ N398 ‐ Other specified disorders of urinary system
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
202
2938 Lowe syndrome (oculocerebrorenal syndrome) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/309000 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Genetic mutation in OCR1. ‐ Similar renal phenotype to that seen in Dent disease.
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 79385002 ‐ Lowe syndrome (disorder) ICD‐10 code and term
‐ E720 ‐ Disorders of amino‐acid transport
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
203
2940 Inherited aminoaciduria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/ search for [amino AND aciduria] Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 522601000000103 ‐ Inherited aminoaciduria (disorder) ICD‐10 code and term
‐ E729 ‐ Disorder of amino‐acid metabolism, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
204
2955 Cystinuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/220100 ‐ http://omim.org/entry/606407 about hypotonia‐cystinuria syndrome
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Urinary cystine concentration / nephrolithiasis / genetic mutations. Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 85020001 ‐ Cystinuria (disorder) ICD‐10 code and term
‐ E720 ‐ Disorders of amino‐acid transport
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
205
2964 Cystinosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/219800 ‐ http://omim.org/entry/219900 about cystinosis late‐onset juvenile or adolescent
nephropathic type Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ Systemic deposit cystine / mutation in CTNS gene. Old ERA‐EDTA PRD code
‐ 52 ‐ Cystinosis SNOMED CT concept identifier and fully specified name
‐ 190681003 ‐ Cystinosis (disorder) ICD‐10 code and term
‐ E720 ‐ Disorders of amino‐acid transport
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
206
2972 Inherited renal glycosuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/233100 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Urine analysis
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 226309007 ‐ Familial renal glucosuria (disorder) ICD‐10 code and term
‐ E748 ‐ Other specified disorders of carbohydrate metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
207
2986 Hypophosphataemic rickets X‐linked (XL) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/307800 about hypophosphatemic rickets, X‐linked dominant; XLHR
‐ http://omim.org/entry/300554 about hypophosphatemic rickets, X‐linked recessive Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 82236004 ‐ Familial x‐ linked hypophosphatemic vitamin D refractory rickets (disorder) ICD‐10 code and term
‐ E833 ‐ Disorders of phosphorus metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
208
2993 Hypophosphataemic rickets autosomal recessive (AR) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/241520 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Imaging shows rickets. Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 90505000 ‐ Autosomal recessive hypophosphatemic vitamin D refractory rickets (disorder)
ICD‐10 code and term
‐ E833 ‐ Disorders of phosphorus metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
209
3000 Primary renal tubular acidosis (RTA) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/267200 about renal tubular acidosis III Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 1776003 ‐ Renal tubular acidosis (disorder) ICD‐10 code and term
‐ N258 ‐ Other disorders resulting from impaired renal tubular funct
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
210
3016 Proximal renal tubular acidosis (RTA) ‐ type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/179830 ‐ http://omim.org/entry/604278 about renal tubular acidosis, proximal, with ocular
abnormalities and mental retardation Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 24790002 ‐ Proximal renal tubular acidosis (disorder) ICD‐10 code and term
‐ N258 ‐ Other disorders resulting from impaired renal tubular funct
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
211
3028 Distal renal tubular acidosis (RTA) ‐ type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/179800 about renal tubular acidosis, distal, autosomal dominant
‐ http://omim.org/entry/602722 about renal tubular acidosis, distal, autosomal recessive; RTADR
‐ http://omim.org/entry/611590 about renal tubular acidosis, distal, with hemolytic anemia
Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ May have nephrocalcinosis. Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 236461000 ‐ Distal renal tubular acidosis (disorder) ICD‐10 code and term
‐ N258 ‐ Other disorders resulting from impaired renal tubular funct
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
212
3037 Distal renal tubular acidosis with sensorineural deafness ‐ gene mutations Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/267300 about renal tubular acidosis, distal, with progressive nerve deafness
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ May have nephrocalcinosis. Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 236461000 ‐ Distal renal tubular acidosis (disorder) ICD‐10 code and term
‐ N258 ‐ Other disorders resulting from impaired renal tubular funct AND H905 ‐ Sensorineural hearing loss, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
213
3044 Nephrogenic diabetes insipidus Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/304800 about diabetes insipidus, nephrogenic, X‐ linked ‐ http://omim.org/entry/125800 about diabetes insipidus, nephrogenic, autosomal ‐ http://omim.org/entry/221995 about diabetes insipidus, nephrogenic, with mental
retardation and intracerebral calcification Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 111395007 ‐ Nephrogenic diabetes insipidus (disorder) ICD‐10 code and term
‐ N251 ‐ Nephrogenic diabetes insipidus
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
214
3059 Lesch Nyhan syndrome ‐ hypoxanthine guanine phosphoribosyl transferase deficiency Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/300322 ‐ http://omim.org/entry/300323 for background information see: ‐ http://omim.org/entry/308000 ‐ http://omim.org/entry/308950
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 10406007 ‐ Lesch‐Nyhan syndrome (disorder) ICD‐10 code and term
‐ E791 ‐ Lesch‐Nyhan syndrome
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
215
3063 Phosphoribosyl pyrophosphate synthetase (PRPPS) superactivity Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/300661 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Biochemistry ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 35759001 ‐ Ribose‐phosphate pyrophosphokinase overactivity (disorder) ICD‐10 code and term
‐ E798 ‐ Other disorders of purine and pyrimidine metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
216
3071 Alagille syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/118450 about alagille syndrome 1; ALGS1 ‐ http://omim.org/entry/610205 about alagille syndrome 2; ALGS2
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Renal dysplasia, renal artery stenosis, and cystic kidney disease have been described in this condition.
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 31742004 ‐ Arteriohepatic dysplasia (disorder) ICD‐10 code and term
‐ Q447 ‐ Other congenital malformations of liver
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
217
3085 Bartter syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/601678 about type 1 ‐ http://omim.org/entry/241200 about type 2 ‐ http://omim.org/entry/607364 about type 3 ‐ http://omim.org/entry/602522 about type 4a ‐ http://omim.org/entry/613090 about type 4b
Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 71275003 ‐ Pseudoprimary aldosteronism (disorder) ICD‐10 code and term
‐ E268 ‐ Other hyperaldosteronism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
218
3092 Gitelman syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/263800 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 3188003 ‐ Familial hypokalemia‐hypomagnesemia (disorder) ICD‐10 code and term
‐ E876 ‐ Hypokalaemia
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
219
3102 Liddle syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/177200 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 71275003 ‐ Pseudoprimary aldosteronism (disorder) ICD‐10 code and term
‐ E268 ‐ Other hyperaldosteronism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
220
3118 Apparent mineralocorticoid excess Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/218030 Criteria for diagnosis [all to be satisfied]
‐ Biochemistry Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 237770005 ‐ Syndrome of apparent mineralocorticoid excess (disorder) ICD‐10 code and term
‐ E278 ‐ Other specified disorders of adrenal gland
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
221
3125 Glucocorticoid suppressible hyperaldosteronism Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/103900 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 237743003 ‐ Glucocorticoid‐suppressible hyperaldosteronism (disorder) ICD‐10 code and term
‐ E268 ‐ Other hyperaldosteronism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
222
3139 Inherited / genetic diabetes mellitus type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/125853 about DM II and its rene relationships Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Family history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ Multiple / variable mutations. ‐ Previously called 'Maturity onset diabetes in young people (MODY)'. ‐ MODY types 1‐6 (HNF‐1alpha mutation accounts for 65%).
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 237604008 ‐ Diabetes mellitus autosomal dominant type II (disorder) ICD‐10 code and term
‐ E139 ‐ Other specified diabetes mellitus without complications
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
223
3141 Pseudohypoaldosteronism type 1 Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/177735 about Pseudohypoaldosteronism type I, autosomal dominant PHA1A
‐ http://omim.org/entry/264350 about Pseudohypoaldosteronism type I, autosomal recessive PHA1B
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 43941006 ‐ Pseudohypoaldosteronism, type 1 (disorder) ICD‐10 code and term
‐ E274 ‐ Other and unspecified adrenocortical insufficiency
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
224
3156 Pseudohypoaldosteronism type 2 (Gordon syndrome) Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/145260 Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 15689008 ‐ Pseudohypoaldosteronism, type 2 (disorder) ICD‐10 code and term
‐ E878 ‐ Other disorders of electrolyte and fluid balance NEC
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
225
3160 Familial hypocalciuric hypercalcaemia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/145980 about HHC1 ‐ http://omim.org/entry/145981 about HHC2 ‐ http://omim.org/entry/600740 about HHC3
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 237885008 ‐ Familial hypocalciuric hypercalcemia (disorder) ICD‐10 code and term
‐ E835 ‐ Disorders of calcium metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
226
3173 Familial hypercalciuric hypocalcaemia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/145980 ‐ http://omim.org/entry/146200
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 237885008 ‐ Familial hypocalciuric hypercalcemia (disorder) ICD‐10 code and term
‐ E835 ‐ Disorders of calcium metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
227
3187 Familial hypomagnesaemia Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/602014 about HOMG1 ‐ http://omim.org/entry/154020 about HOMG2 ‐ http://omim.org/entry/248250 about HOMG3 ‐ http://omim.org/entry/611718 about HOMG4 ‐ http://omim.org/entry/248190 about HOMG5 ‐ http://omim.org/entry/613882 about HOMG6
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Biochemistry ‐ Gene test
Additional notes/criteria
‐ A number of syndromes with different mutations have been identified. Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 80710001 ‐ Primary hypomagnesemia (disorder) ICD‐10 code and term
‐ E834 ‐ Disorders of magnesium metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
228
3194 Primary hyperoxaluria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/259900 about hyperoxaluria, primary, type I; HP1 ‐ http://omim.org/entry/260000 about hyperoxaluria, primary, type II; HP2 ‐ http://omim.org/entry/613616 about hyperoxaluria, primary, type III; HP3
Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry ‐ Imaging
Old ERA‐EDTA PRD code
‐ 53 ‐ Primary oxalosis SNOMED CT concept identifier and fully specified name
‐ 17901006 ‐ Primary hyperoxaluria (disorder) ICD‐10 code and term
‐ E748 ‐ Other specified disorders of carbohydrate metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
229
3207 Primary hyperoxaluria type I Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/259900 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 53 ‐ Primary oxalosis SNOMED CT concept identifier and fully specified name
‐ 65520001 ‐ Primary hyperoxaluria type I (disorder) ICD‐10 code and term
‐ E748 ‐ Other specified disorders of carbohydrate metabolism
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
IV FAMILIAL / HEREDITARY NEPHROPATHIES
230
3211 Primary hyperoxaluria type II Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/260000 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 53 ‐ Primary oxalosis SNOMED CT concept identifier and fully specified name
‐ 40951006 ‐ Primary hyperoxaluria, type II (disorder) ICD‐10 code and term
‐ E748 ‐ Other specified disorders of carbohydrate metabolism
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3731 Primary hyperoxaluria type III Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/613616 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Biochemistry ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Associated with mutations in an uncharacterized gene (DHDPSL) on chromosome 10. Old ERA‐EDTA PRD code
‐ 53 ‐ Primary oxalosis SNOMED CT concept identifier and fully specified name
‐ 828971000000101 ‐ Primary hyperoxaluria, type III (disorder) ICD‐10 code and term
‐ E748 ‐ Other specified disorders of carbohydrate metabolism
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3224 Fabry disease ‐ no histology Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/301500 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Immunology ‐ Gene test
Old ERA‐EDTA PRD code
‐ 54 ‐ Fabry's disease SNOMED CT concept identifier and fully specified name
‐ 16652001 ‐ Fabry's disease (disorder) ICD‐10 code and term
‐ E752 ‐ Other sphingolipidosis
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3230 Fabry disease ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/301500 Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Biochemistry ‐ Immunology ‐ Gene test
Old ERA‐EDTA PRD code
‐ 54 ‐ Fabry's disease SNOMED CT concept identifier and fully specified name
‐ 16652001 ‐ Fabry's disease (disorder) ICD‐10 code and term
‐ E752 ‐ Other sphingolipidosis
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3248 Xanthinuria Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/278300 about xanthinuria, type I ‐ http://omim.org/entry/603592 about xanthinuria, type II
Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 190919008 ‐ Xanthinuria (disorder) ICD‐10 code and term
‐ E798 ‐ Other disorders of purine and pyrimidine metabolism
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3253 Nail‐patella syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/161200 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 236527004 ‐ Nail patella‐like renal disease (disorder) ICD‐10 code and term
‐ N078 ‐ Hereditary nephropathy, not elsewhere classified, other
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3269 Rubinstein‐Taybi syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/180849 about Rubinstein‐Taybi syndrome 1; RSTS1 ‐ http://omim.org/entry/613684 about Rubinstein‐Taybi syndrome 2; RSTS2
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 45582004 ‐ Rubinstein‐Taybi syndrome (disorder) ICD‐10 code and term
‐ Q872 ‐ Cong malformation syndromes predominantly involving limbs
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3276 Tuberous sclerosis Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/191100 about tuberous sclerosis 1; TSC1 ‐ http://omim.org/entry/605284 about TSC1 gene; TSC1 ‐ http://omim.org/entry/613254 about tuberous sclerosis 2; TSC2 ‐ http://omim.org/entry/191092 about TSC2 gene; TSC2
Criteria for diagnosis [all to be satisfied]
‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 7199000 ‐ Tuberous sclerosis syndrome (disorder) ICD‐10 code and term
‐ Q851 ‐ Tuberous sclerosis
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238
3282 Von Hippel‐Lindau disease Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/193300 about Von Hippel‐Lindau syndrome; VHL ‐ http://omim.org/entry/608537 about VHL gene
Criteria for diagnosis [all to be satisfied]
‐ Clinical exam ‐ Imaging
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 46659004 ‐ Von Hippel‐Lindau syndrome (disorder) ICD‐10 code and term
‐ Q858 ‐ Other phakomatoses, not elsewhere classified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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239
3295 Medullary sponge kidneys Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 236443009 ‐ Medullary sponge kidney (disorder) ICD‐10 code and term
‐ Q615 ‐ Medullary cystic kidney
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240
3305 Horse‐shoe kidney Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 41729002 ‐ Horseshoe kidney (disorder) ICD‐10 code and term
‐ Q631 ‐ Lobulated, fused and horseshoe kidney
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241
3314 Frasier syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/136680 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Clinical exam ‐ Imaging ‐ Gene test
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 445431000 ‐ Frasier syndrome (disorder) ICD‐10 code and term
‐ Q998 ‐ Other specified chromosome abnormalities
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3658 Renal coloboma syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/120330 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Imaging ‐ Gene test
Additional notes/criteria
‐ Associated with the Pax2 gene. Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 446449009 ‐ Renal coloboma syndrome (disorder) ICD‐10 code and term
‐ Q605 ‐ Renal hypoplasia, unspecified
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243
3322 Branchio‐oto‐renal syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/113650 about branchiootorenal syndrome 1; BOR1 ‐ http://omim.org/entry/610896 about branchiootorenal syndrome 2; BOR2
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 290006 ‐ Melnick‐Fraser syndrome (disorder) ICD‐10 code and term
‐ Q870 ‐ Cong malform syndromes predom affect facial appearance
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3333 Williams syndrome Criteria for diagnosis [all to be satisfied]
‐ Clinical exam ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 63247009 ‐ Williams syndrome (disorder) ICD‐10 code and term
‐ Q878 ‐ Other specified congenital malformation syndromes NEC
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3346 Townes‐Brocks syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/107480 Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test
Additional notes/criteria
‐ SALL1 is the only gene known to be associated with TBS (2011). Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 523411000000105 ‐ Townes‐Brocks syndrome (disorder) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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3351 Lawrence‐Moon‐Biedl / Bardet‐Biedl syndrome Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/245800 about Laurence‐Moon syndrome ‐ http://omim.org/entry/209900 about Bardet‐Biedl syndrome; BBS
Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Family history ‐ Clinical exam ‐ Gene test
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 232059000 ‐ Laurence‐Moon syndrome (disorder) ICD‐10 code and term
‐ Q878 ‐ Other specified congenital malformation syndromes NEC
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3367 Mitochondrial cytopathy Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov; for background information see:
‐ http://omim.org/entry/251900 about mitochondrial myopathy Criteria for diagnosis [all to be satisfied]
‐ Gene test Additional notes/criteria
‐ Usually diagnosed by muscle histopathology and mitochondrial enzyme complex functional assay.
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 240096000 ‐ Mitochondrial cytopathy (disorder) ICD‐10 code and term
‐ G713 ‐ Mitochondrial myopathy, not elsewhere classified
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3379 Familial nephropathy Criteria for diagnosis [all to be satisfied]
‐ Family history Additional notes/criteria
‐ A clear and compatible family history must be present that cannot be assigned to any more specific familial disorder.
Old ERA‐EDTA PRD code
‐ 59 ‐ Hereditary nephropathy ‐ other specified type SNOMED CT concept identifier and fully specified name
‐ 236419006 ‐ Progressive hereditary glomerulonephritis without deafness (disorder) ICD‐10 code and term
‐ N079 ‐ Unspecified morphological changes
V MISCELLANEOUS RENAL DISORDERS
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3380 Acute kidney injury Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ For a brief definition of acute and chronic renal failure when RRT is required, see 'Notes for users'.
‐ This super concept PRD should only be used if it is not possible to choose a more accurate one.
Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 14669001 ‐ Acute renal failure syndrome (disorder) ICD‐10 code and term
‐ N179 ‐ Acute renal failure, unspecified
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3398 Acute kidney injury due to hypovolaemia Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 14669001 ‐ Acute renal failure syndrome (disorder) ICD‐10 code and term
‐ N179 ‐ Acute renal failure, unspecified AND E86X ‐ Volume depletion
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3403 Acute kidney injury due to circulatory failure Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 14669001 ‐ Acute renal failure syndrome (disorder) ICD‐10 code and term
‐ N179 ‐ Acute renal failure, unspecified AND R579 ‐ Shock, unspecified
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3419 Acute kidney injury due to sepsis Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 14669001 ‐ Acute renal failure syndrome (disorder) ICD‐10 code and term
‐ N179 ‐ Acute renal failure, unspecified
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3426 Acute kidney injury due to rhabdomyolysis Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 14669001 ‐ Acute renal failure syndrome (disorder) ICD‐10 code and term
‐ N179 ‐ Acute renal failure, unspecified AND M628 ‐ Other specified disorders of muscle
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3435 Acute kidney injury due to nephrotoxicity Criteria for diagnosis [all to be satisfied]
‐ Clinical history Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 236428007 ‐ Nephrotoxic acute renal failure (disorder) ICD‐10 code and term
‐ N144 ‐ Toxic nephropathy, not elsewhere classified
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3442 Acute cortical necrosis Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Imaging
Additional notes/criteria
‐ Either imaging or histological evidence is acceptable. Old ERA‐EDTA PRD code
‐ 90 ‐ Tubular necrosis (irreversible) or cortical necrosis (different from 88) SNOMED CT concept identifier and fully specified name
‐ 197650009 ‐ Acute cortical necrosis (disorder) ICD‐10 code and term
‐ N171 ‐ Acute renal failure with acute cortical necrosis
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3457 Acute pyelonephritis Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ Either evidence of urinary tract infection with white cell casts or histology. Old ERA‐EDTA PRD code
‐ Not available in previous coding system SNOMED CT concept identifier and fully specified name
‐ 36689008 ‐ Acute pyelonephritis (disorder) ICD‐10 code and term
‐ N10X ‐ Acute tubulo‐interstitial nephritis
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3461 Kidney tumour Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/144700 about renal cell carcinoma, nonpapillary; RCC ‐ http://omim.org/entry/150800 about hereditary leiomyomatosis and renal cell
cancer; HLRCC ‐ http://omim.org/entry/609322 about rhabdoid tumor predisposition syndrome 1;
RTPS1 ‐ http://omim.org/entry/267000 about renal hamartomas, nephroblastomatosis, and
fetal gigantism Criteria for diagnosis [all to be satisfied]
‐ Imaging Old ERA‐EDTA PRD code
‐ 95 ‐ Kidney tumour SNOMED CT concept identifier and fully specified name
‐ 126880001 ‐ Neoplasm of kidney (disorder) ICD‐10 code and term
‐ D410 ‐ Neoplasm uncert / unkn behav kidney
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3474 Renal cell carcinoma ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/605074 about renal cell carcinoma, papillary, 1; RCCP1 ‐ http://omim.org/entry/613253 about hereditary renal cancer, associated 1; HRCA1 ‐ http://omim.org/entry/606423 about disrupted in renal carcinoma 1; DIRC1 ‐ http://omim.org/entry/602773 about disrupted in renal carcinoma 2; DIRC2 ‐ http://omim.org/entry/608262 about disrupted in renal carcinoma 3; DIRC3 ‐ http://omim.org/entry/300854 about renal cell carcinoma, xp11‐associated; RCCX1 ‐ http://omim.org/entry/179755 about papillary renal cell carcinoma translocation‐
associated gene; PRCC ‐ http://omim.org/entry/605642 about thyroid carcinoma, papillary, with papillary renal
neoplasia ‐ http://omim.org/entry/144700 about renal cell carcinoma, nonpapillary; RCC ‐ http://omim.org/entry/150800 about hereditary leiomyomatosis and renal cell
cancer; HLRCC Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 95 ‐ Kidney tumour SNOMED CT concept identifier and fully specified name
‐ 254915003 ‐ Clear cell carcinoma of kidney (disorder) ICD‐10 code and term
‐ C64X ‐ Malignant neoplasm of kidney, except renal pelvis
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3488 Transitional cell carcinoma ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/109800 about bladder cancer. Refers to TCC of the renal pelvis Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 95 ‐ Kidney tumour SNOMED CT concept identifier and fully specified name
‐ 408642003 ‐ Transitional cell carcinoma of kidney (disorder) ICD‐10 code and term
‐ C64X ‐ Malignant neoplasm of kidney, except renal pelvis
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3490 Wilms tumour ‐ histologically proven Online Mendelian Inheritance in Man ‐ link from the National Center for Biotechnology Information www.ncbi.nlm.nih.gov
‐ http://omim.org/entry/194070 about Wilms tumor 1; WT1 ‐ http://omim.org/entry/605442 about Wilms tumor 1‐associating protein; WTAP ‐ http://omim.org/entry/194071 about Wilms tumor 2; WT2 ‐ http://omim.org/entry/194090 about Wilms tumor 3; WT3 ‐ http://omim.org/entry/601363 about Wilms tumor 4; WT4 = familial wilms tumor 1;
FWT1 ‐ http://omim.org/entry/601583 about Wilms tumor 5; WT5 ‐ http://omim.org/entry/194072 about Wilms tumor, aniridia, genitourinary anomalies,
and mental retardation syndrome; WAGR ‐ http://omim.org/entry/612469 about Wilms tumor, aniridia, genitourinary anomalies,
mental retardation, and obesity syndrome; WAGRO Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 95 ‐ Kidney tumour SNOMED CT concept identifier and fully specified name
‐ 302849000 ‐ Nephroblastoma (disorder) ICD‐10 code and term
‐ C64X ‐ Malignant neoplasm of kidney, except renal pelvis
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3501 Mesoblastic nephroma ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology Old ERA‐EDTA PRD code
‐ 95 ‐ Kidney tumour SNOMED CT concept identifier and fully specified name
‐ 307604008 ‐ Mesoblastic nephroma (disorder) ICD‐10 code and term
‐ D410 ‐ Neoplasm uncert / unkn behav kidney
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3517 Single kidney identified in adulthood Criteria for diagnosis [all to be satisfied]
‐ Imaging Additional notes/criteria
‐ Diagnosis codes expressing the presence of a kidney (left or right) are not suitable because they do not imply absence of the contralateral kidney.
Old ERA‐EDTA PRD code
‐ 99 ‐ Other identified renal disorders SNOMED CT concept identifier and fully specified name
‐ 824131000000108 ‐ Solitary kidney (finding) ICD‐10 code and term
‐ ICD‐10 code is not available. When required, users should discuss the clinical features with local ICD‐10 coding staff and select the most appropriate code.
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3529 Chronic kidney disease (CKD) / chronic renal failure (CRF) caused by tumour nephrectomy Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ Causing kidney disease due to loss of renal mass in the absence of another identified primary renal disease.
Old ERA‐EDTA PRD code
‐ 96 ‐ Traumatic or surgical loss of kidney SNOMED CT concept identifier and fully specified name
‐ 236425005 ‐ Chronic renal impairment (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified
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3538 Chronic kidney disease (CKD) / chronic renal failure (CRF) due to traumatic loss of kidney Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ Causing kidney disease due to loss of renal mass in the absence of another identified primary renal disease.
Old ERA‐EDTA PRD code
‐ 96 ‐ Traumatic or surgical loss of kidney SNOMED CT concept identifier and fully specified name
‐ 236425005 ‐ Chronic renal impairment (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified
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3540 Chronic kidney disease (CKD) / chronic renal failure (CRF) due to donor nephrectomy Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ Causing kidney disease due to loss of renal mass in the absence of another identified primary renal disease.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 236425005 ‐ Chronic renal impairment (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified
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3555 Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Clinical history Additional notes/criteria
‐ This PRD should only be used after careful history, clinical examination and appropriate investigation.
‐ It does not mean that no information is available. In that case, consider using PRD 3643 'Chronic renal failure'.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 236425005 ‐ Chronic renal impairment (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified
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3564 Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ histologically proven Criteria for diagnosis [all to be satisfied]
‐ Histology ‐ Clinical history
Additional notes/criteria
‐ This PRD should only be used after careful history, clinical examination and appropriate investigation.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 236425005 ‐ Chronic renal impairment (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified
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3572 Haematuria and proteinuria ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Urine analysis
Additional notes/criteria
‐ This PRD should only be used after careful history, clinical examination and appropriate investigation.
‐ It does not mean that no information is available. In that case, consider using PRD 3643 'Chronic renal failure'.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 53298000 ‐ Hematuria syndrome (disorder) AND 29738008 ‐ Proteinuria (disorder) ICD‐10 code and term
‐ R31X ‐ Unspecified haematuria AND N391 ‐ Persistent proteinuria, unspecified
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3712 Isolated haematuria ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Urine analysis
Additional notes/criteria
‐ This PRD should only be used after careful history, clinical examination and appropriate investigation.
‐ It does not mean that no information is available. In that case, consider using PRD 3643 'Chronic renal failure'.
‐ Alternative PRDs are available for 'Isolated proteinuria ‐ no histology' and for 'Haematuria and proteinuria ‐ no histology'.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 53298000 ‐ Hematuria syndrome (disorder) ICD‐10 code and term
‐ R31X ‐ Unspecified haematuria
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3720 Isolated proteinuria ‐ no histology Criteria for diagnosis [all to be satisfied]
‐ Biochemistry ‐ Urine analysis
Additional notes/criteria
‐ This PRD should only be used after careful history, clinical examination and appropriate investigation.
‐ It does not mean that no information is available. In that case, consider using PRD 3643 'Chronic renal failure'.
‐ Alternative PRDs are available for 'Isolated haematuria ‐ no histology' and for 'Haematuria and proteinuria ‐ no histology'.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 29738008 ‐ Proteinuria (disorder) ICD‐10 code and term
‐ N391 ‐ Persistent proteinuria, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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3643 Chronic renal failure due to systemic infection Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry ‐ Imaging
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 90688005 ‐ Chronic renal failure syndrome (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
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3691 Renal failure Criteria for diagnosis [all to be satisfied]
‐ Clinical history ‐ Biochemistry
Additional notes/criteria
‐ This super concept PRD should only be used if it is not possible to specify even whether the patient has acute or chronic renal failure.
Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 42399005 ‐ Renal failure syndrome (disorder) ICD‐10 code and term
‐ N19X ‐ Unspecified renal failure
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
V MISCELLANEOUS RENAL DISORDERS
2012 ERA‐EDTA Primary Renal Disease (PRD) Coding System ‐ version 1.0
273
3708 Chronic renal failure Additional notes/criteria
‐ This super concept PRD should be used: 1) when the investigations that have been undertaken do not allow any more
granular PRD to be selected or 2) very infrequently when a full diagnosis has been made which can not be assigned
to another PRD. In this case, please send an email to the ERA‐EDTA Registry office, highlighting the PRD that is missing.
‐ Where a patient has been investigated appropriately but it is not possible to choose a more granular PRD, consider using: ‐ PRD 3555 = 'Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ no histology, or ‐ PRD 3564 'Chronic kidney disease (CKD) / chronic renal failure (CRF) ‐ aetiology uncertain / unknown ‐ histologically proven'.
‐ See 'Notes for users'. Old ERA‐EDTA PRD code
‐ 00 ‐ Chronic renal failure; aetiology uncertain SNOMED CT concept identifier and fully specified name
‐ 90688005 ‐ Chronic renal failure syndrome (disorder) ICD‐10 code and term
‐ N189 ‐ Chronic renal failure, unspecified