Post on 20-May-2015
ACUTE NEPHRITIC SYNDROME
By Hakimah Khani Binti Suhaimi
Introduction
bull Synonyms acute nephritis acute nephritic
syndromebull An immunologic mechanism the
result of an immune process that injures the glomeruli of the kidney
bull Clinical features ndash A sudden onset of hematuria ndash Proteinuriandash Edema ndash Oliguria and volume overloadndash Hypertension ndash Azotemia is another common but
inconstant finding
Glomerulo
nephritis
Acute Nephritic syndrome
Acute post strep GN
IgA Nephropath
y
MembranoProliferative
Henoch Schonlein Purpura
Alport Syndrome
SLE nephritis
Other post infectious glomerulonephritis
Nephrotic Syndrome
ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
Outline
bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis
Definition
AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci
The classic example of the acute nephritic syndrome
Nelson Textbook of Pediatrics 7th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Introduction
bull Synonyms acute nephritis acute nephritic
syndromebull An immunologic mechanism the
result of an immune process that injures the glomeruli of the kidney
bull Clinical features ndash A sudden onset of hematuria ndash Proteinuriandash Edema ndash Oliguria and volume overloadndash Hypertension ndash Azotemia is another common but
inconstant finding
Glomerulo
nephritis
Acute Nephritic syndrome
Acute post strep GN
IgA Nephropath
y
MembranoProliferative
Henoch Schonlein Purpura
Alport Syndrome
SLE nephritis
Other post infectious glomerulonephritis
Nephrotic Syndrome
ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
Outline
bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis
Definition
AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci
The classic example of the acute nephritic syndrome
Nelson Textbook of Pediatrics 7th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Glomerulo
nephritis
Acute Nephritic syndrome
Acute post strep GN
IgA Nephropath
y
MembranoProliferative
Henoch Schonlein Purpura
Alport Syndrome
SLE nephritis
Other post infectious glomerulonephritis
Nephrotic Syndrome
ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
Outline
bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis
Definition
AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci
The classic example of the acute nephritic syndrome
Nelson Textbook of Pediatrics 7th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
Outline
bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis
Definition
AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci
The classic example of the acute nephritic syndrome
Nelson Textbook of Pediatrics 7th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Outline
bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis
Definition
AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci
The classic example of the acute nephritic syndrome
Nelson Textbook of Pediatrics 7th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Definition
AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci
The classic example of the acute nephritic syndrome
Nelson Textbook of Pediatrics 7th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Epidemiologybull 121 of the 124 patients had
poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988
bull Globally - incidence has decreased in the past three decades
bull Most commonly ndash sporadicbull Despite that epidemics and clusters of
cases - in some poor or rural communitiescopy 2008 American Society of Nephrology
bull Peak incidence - age 5-12 yo uncommon lt3yo
bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Etiology and Pathogenesis
bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1
bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation
bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Streptococcal infection
immune complex formation + deposited in GBM
complement system activated
immune injuries
cellular proliferation GBM fracture
capillary lumen narrowed hematuria
glomerular blood flow decreased proteinuria
oliguria GFR distal sodium reabsorption
retention of water amp sodium
blood volume
edema hypertension
Low serum complement
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
CLINICAL FEATURES
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma
1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)
2 Proteinuria ndash usu normalize after 4 weeks
3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Typical Manifestation (2)3 Gross hematuria (65 of
patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically
4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis
5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Clinical course
Spontaneous improvement
typically begins within 1 wk with
resolution of edema in 5-10 days
and hypertension in 2-3 wk but
urinalysis may be abnormal
(persistent microscopic
hematuria) for a year
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Complications in severe cases
bull Circulatory hypervolemia Congestive heart failure
bull Encephalopathy
bull Acute renal failure
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Laboratory Investigations1 Urinalysis2 Bacteriological and Serological
test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Laboratory Investigations
Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts
(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria
occurs in lt5 of patients) bull Pyuria The urine contains large amounts of
fibrin degradation products and fibrinopeptides
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Laboratory Investigations
bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)
skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level
Pharyngitis (98) skin infections (80) bull Renal function Test
ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children
ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Laboratory Investigations
bull Full Blood Countndash A mild normochromic anemia may be
present from hemodilution and low-grade hemolysis
ndash Leococytosis maybe present
bull Activation of complementsndash Serum C3 level decrease (90) return to
normal within 6 weeks ndash Serum C4 levels are typically normal
bull Kidney ultrasoundndash Not necessary if patient has clear cut acute
nephritic syndrome
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Renal Biopsy
bull Patients whose clinical presentation laboratory findings or course is atypical
bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Light microscopeNot specific for post streptococcal nephritis
bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix
bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Electron microscope
bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)
bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Immunofluorescence microscopy
bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium
bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Diagnosis
bull Acute onsetbull Symptoms edema oliguria dark
urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal
infectionndash Prodromesndash Elavated serum titers of Abs to
streptozymes(ASO)bull Serum C3 - Reduced
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Differential DiagnosisPoints to support Points against
Membrano-proliferative GN
a) Nephritic syndrome
b) Elevated ASO titer
c) Hypocomplement-emia
a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -
gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-
trackingrdquo GBM
IgA nephropathy (Bergerrsquos disease)
a) Hematuriab) Associated with
respiratory illness (following viral syndromes)
a) Recurrentb) No period of latencyc) Hypertension amp edema ndash
uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement
valuesg) Focal proliferation diffuse
mesangial IgA deposits
Henoch- Schoumlnlein Purpura
a) Hematuria (80)
b) Mild proteinuriac) Preceding by
URTI (only 50)
a) Characteristic skin rashb) Assoc symptoms Abdominal
pain athritis athralgiac) Normal serum complement
valuescopy Hakimah
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Differential DiagnosisPoints to support Points against
Familial nephritis (Alport Syndrome)
a) Nephritic syndromeb) 1-2 days following
URTI
a) Lack of expected complete resolution
b) Progressive proteinuria after 2nd decade of life
c) Association with non-renal manifestations hearing deficits amp ocular abnormalities
SLE nephritis
a) Occurs in 30ndash70 of children
b) Hematuriac) Mild proteinuriad) Hypocomplement-
emia (C3)
a) Lack of expected complete resolution
b) Association with non-renal manifestations
c) C4 also depressedd) Detection of anti-nuclear
antibodies
Other chronic infections
a) Acute nephritic syndrome
b) Similar histopathologic findings
c) Hypocomplementemia
a) Missing evidence of a prior streptococcal infection
b) Treatment is unresponsive
copy Hakimah
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Treatment
bull Treatment of APSGN is largely that of supportive care
bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis
bull Management is directed at treating the acute effects of renal insufficiency and hypertension
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
bull Diuretics
bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary
bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Treatment for complications
Bed rest
Recheck BP frac12 hour later
Oral nifedipine
Add
Furosemide
Other oral AHT
Significant asymptomatic hypertension
Look for signs and symptoms
Emergency management
indicated
Target BP control
Reduce BP by 25 of target BP
over 3-12 hrs
Next reduction of 75 over 48
hrs
symptomatic severe hypertension or hypertensive emergency encephalopathy
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Treatment for complications
-Give O2
-prop patient up
-ventilatory support if necessary
IV
furosemide
Fluid restriction
-withhold fluid for 24 Hrs if possiible
Consider dialysis if no response to diuretics
Acute pulmon
ary edema
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Treatment for complications
Monitor BP amp managing
hypertension
Limiting fluid intake in
oliguric state
Correcting electrolyte imbalance
ie life threatening hyperkalemi
a
Optimizing nutritional intake
(since ARF pt usually hypercatabolic)
Implementing acute dialysis
Acute Renal Failure
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Follow-up
bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if
not already normalised by time of discharge
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition
Prognosis
bull short term outcome excellent mortality lt05
bull long term outcome 18 of children develop chronic kidney disease
bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management
Paediatric Protocols 12th Edition