Nephrology Division King Khalid University tutorial to course441 Tutorial Case.
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Transcript of Nephrology Division King Khalid University tutorial to course441 Tutorial Case.
Nephrology DivisionKing Khalid Universitytutorial to course441
Tutorial Case
14 year old Saudi young female student
C/0 fever- Headache for 10 days
General malaise
Dark urine x 3 days
HPI
Fever intermittentFever intermittent
Muscular joint painMuscular joint pain
no skin rashno skin rash
urine characterurine character
edemaedema
PH & Med PH & Med
antibiotic & NSAIDantibiotic & NSAID
Social HxSocial Hx
S. reviewS. review
FHFH
ExaminationExamination
Bp : 160/90 &Pulse 120/MIN Bp : 160/90 &Pulse 120/MIN Temp 39 C Resp 25/MIN .paleTemp 39 C Resp 25/MIN .pale
Look SickLook Sick
Puffiness in facePuffiness in face
Head& neckHead& neck
JVP highJVP high
Chest: Bronchial breathing
decrease Breath Rt sideIncrease TVFIncrease Dull percussionS1 increaseS2 NS3 Positivepan systolic murmurradiation to axillagrade 3
Abd tend epigastictend lionsBS + ve
CNS Drawsy
M.S tender joint &muscle
Initial DiagnosisFever? Infection RT lung consolidationM R HEAMATURIA: Renal stone (ARF –AKI- ATN) Acute glomerulonephritis (post infection)GN RPGN IgAHemolytic anemiahemolytic uremic syndromeNSAID papillary necrosis
Investigation
WBC 15000 cells per microliter Hb 100 g/L Plat 150 g/L
PT normal PPT normal Sec Bl film Sec
U & E
Scr 210 μmml
urea 20 mmol/L
K6 mmol/L Na 125 mmol/L
Ca 1.9 mmol/L album 28 g/L
Urine Analysis
few RBC
No cast
Protein 1.2g/24h
U/S : kid size ENLARGE 12.2CMU/S : kid size ENLARGE 12.2CM
Treatment
Patient receive ceftrixone IV and IV fluid TREAT HYPERKALEMIA
Follow up:
S cr 300 μmml/L
JVP
Oliguria
Edema
Management:
IV lasixRepeat urine analysis RBC CAST ANCA(P) positive Kidney Biopsy RPGN
Final diagnosis microscopic polyangitis
Glomerular Disease – Glomerular Disease – Acute GlomerulonephritisAcute Glomerulonephritis
Post infectious glomerulonephritis
Group A Strep Infection Infective endcarditis
Membranoproliferative glomerulonephritis:
Systemic lupus erythematosusHepatitis C virus
Rapidly progressive glomerulonephritis Vasculitis (cryoglobulinemia
Type I RPGN (direct antibody) Good Posture syndrome
Type II RPGN (immune complex) Post infectious Systematic lupus erythematosus Henoch – Schonlein pupura (IgA) Others
Type III RPGN (pauci-immune)
o Wegener granulomatosiso Microscopic polyarteritis
Investigations for Investigations for GlomerulonephritisGlomerulonephritis
Regular Follow-up and U&ERegular Follow-up and U&EAntistreptolysin O (ASO)Antistreptolysin O (ASO) ANA, Anti DNAANA, Anti DNA C3-C4C3-C4 ANCA (p, c)ANCA (p, c) HCV Antibody HBsAgHCV Antibody HBsAgHIVHIVRFRFCryoglobulinCryoglobulinAnti-basement Membrane Antibody (With lung Anti-basement Membrane Antibody (With lung hemorrhage)hemorrhage)
Acute postinfection Acute postinfection GlomerulonephritisGlomerulonephritis
Often associated with group A Often associated with group A
B-hemolytic streptococcal type 12 infectionB-hemolytic streptococcal type 12 infection
Abrupt onset 10-12 days after strept throat Abrupt onset 10-12 days after strept throat or 14-21 skin infection (impetigo)or 14-21 skin infection (impetigo)
Also staphylococcus or virusesAlso staphylococcus or viruses
Acute GlomerulonephritisAcute Glomerulonephritis
Symptoms occur 10-21 days after infection
HematuriaProteinuriaDecreased GFR, oliguriaHypertensionEdema around eyes, feet and anklesAscites or pleural effusion
Low C3, normal C4, Antistreptolysin O (ASO) – Kidney biopsy immune complexes and proliferation,
Proliferative GN- poststretococcal
This glomerulus is hypercellular and capillary loops are poorly defined.
This is a type of proliferative glomerulonephritis known as post- streptococcal glomerulonephritis.
Poststreptococcal GN
Post-streptococcal glomerulonephritis is immunologically mediated, and the immune deposits are distributed in the capillary loops in a
granular, bumpy pattern because of the focal nature of the deposition process.
Post-Streptococcal GlomerulonephritisPost-Streptococcal Glomerulonephritis
CONSERATIVE TREATMENT (acute kidney CONSERATIVE TREATMENT (acute kidney injury)injury)
Improves 1-4 weeks, C3 normalizes in 1-3 Improves 1-4 weeks, C3 normalizes in 1-3 months, hypertension improves 1-3 months, months, hypertension improves 1-3 months, intermittent hematuria x 3 yearsintermittent hematuria x 3 years
99% complete recovery in children and 85% 99% complete recovery in children and 85% in adultin adult
IgA Nephropathy (Berger's Disease)IgA Nephropathy (Berger's Disease)
Most common acute glomerulonephritis in US ;South East Asia Most common acute glomerulonephritis in US ;South East Asia Associated with H.S. PurpuraAssociated with H.S. Purpura Upper respiratory(50%) IN 1—2 Day(Synpharyngitic hematuria)Upper respiratory(50%) IN 1—2 Day(Synpharyngitic hematuria) Primary versus secondary (IBD, Liver Disease, SLE, vasculitis)Primary versus secondary (IBD, Liver Disease, SLE, vasculitis) 50% risk of CRF50% risk of CRF Proteinuria, hypertension, renal insufficiency predict worse prognosisProteinuria, hypertension, renal insufficiency predict worse prognosis 50% increased IgA, normal compliments 50% increased IgA, normal compliments
TREATEMENT COSERVATIVE; ACEI TREATEMENT COSERVATIVE; ACEI
HIGH RISK: patient prednisone & alkylating agentHIGH RISK: patient prednisone & alkylating agent(cyclophosphamide-azothroprim) &ASA&ACEI&tosilectomy(cyclophosphamide-azothroprim) &ASA&ACEI&tosilectomy
Rapidly Progressive GN
Develops over a period of days or weeksDevelops over a period of days or weeks
Primarily adults in 50’s and 60’sPrimarily adults in 50’s and 60’s
Progresses to renal failure in a few weeks or Progresses to renal failure in a few weeks or monthsmonths
Hematuria is common, may see proteinuria, Hematuria is common, may see proteinuria, edema or hypertensionedema or hypertension
Rapidly Progressive (Crescentic) Glomerulonephritis
Morphology crescent formation
crescents are formed by proliferation of parietal cells
infiltrates of WBC’s & fibrin deposition in Bowman’s space
EM reveals focal ruptures in the GBM
MICROSCOPIC POLYANGITIS
Necrotizing vasculitis of small- and medium- sized vessels in both the arterial and venous circulations Frequently involves the lung and the kidneys
with typical complications of hemorrhage and glomerulonephritis Associated with ANCA in three-fourths of all
cases, usually anti-myeloperoxidase antibodies (MPO-ANCA) that cause a P-ANCA pattern on immuno-flourescence testing. ANCA directedf againsts proteinae-3 (PR3-ANCA) can also be observed in microscopic polyangiitis
Treatment
Initial TherapyInitial TherapyCombination cyclophosphamide-Combination cyclophosphamide-corticosteroid therapy corticosteroid therapy A slow steroid taper, with the goal of A slow steroid taper, with the goal of reaching 20 mg of prednisone per day reaching 20 mg of prednisone per day by the end of two months and an overall by the end of two months and an overall glucocorticoid course of between 6 and glucocorticoid course of between 6 and 9 months. 9 months. Either daily oral or monthly intravenous Either daily oral or monthly intravenous cyclophosphamide.cyclophosphamide.
Treatment
Plasmapheresis Plasmapheresis Severe manifestations of pulmonary Severe manifestations of pulmonary hemorrhage on presentation. hemorrhage on presentation.
Dialysis-dependent renal failure upon Dialysis-dependent renal failure upon presentation.presentation.
Concurrent anti-GBM antibodies. Concurrent anti-GBM antibodies.
Goodpasture Syndrome
Antibody formation against pulmonary and glomerular capillary basement membranes
Damage glomerular basement membrane Men 20 - 30 years of age Pulmonary hemorrhage and renal failure
TREATMENT:Early treatment is essential Pulse Steroid (10 mg/ kg/day for 3-5 days)CyclophosphamidePlasmaphorisis
Goodpasture’s syndrome
This immunofluorescence micrograph shows positivity with antibody to IgG has a smooth, diffuse, linear pattern that is characteristic for glomerular basement membrane antibody with Goodpasture's syndrome.