ACUTE NEPHRITIC

SYNDROME

By Hakimah Khani Binti Suhaimi

Third Year Medical Student
MARA University of Technology, Malaysia

(this is the edited slides, after discussions with Assoc. Prof. Dr. Rashid =)
and bedsite teaching with Dr Faisal =)
 Introduction
• Synonyms: acute nephritis,
acute nephritic syndrome

• An immunologic mechanism (Type III hypersensitivity)

resulted from an immune process that injures the
glomeruli of the kidney, leading to reduced renal
function characterized as follows:

• Clinical features:
– A sudden onset of hematuria,
– Proteinuria,
– Edema,
– Oliguria and volume overload,
– Hypertension,
– Azotemia is another common but inconstant finding.
 Acute post
strep GN

IgA
Nephropathy

Membrano
Proliferative
Henoch
Acute Schonlein
Nephritic Purpura
Glomerulo syndrome
nephritis Alport
Nephrotic Syndrome
Syndrome
SLE
nephritis
Other post
infectious
glomerulo
nephritis
 ACUTE
POST-STREPTOCOCCAL
GLOMERULONEPHRITIS
 Outline
• Definition
• Epidemiology
• Etiology and Pathogenesis
• Clinical Features
• Complication
• Investigation
• Diagnosis
• Differential Diagnosis
• Treatment and Management
• 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
 Epidemiology
• 121 of the 124 patients had poststreptococcal
nephritis.
Department of Pediatrics, HUSM, July 1987- June 1988

• Globally - incidence has decreased in the past

three decades.
• Most commonly – sporadic.
• Despite that, epidemics and clusters of cases - in
some poor or rural communities
© 2008 American Society of Nephrology

• Peak incidence - age 5-12 y/o, uncommon <3y/o.

• Male : female ratio is 2 : 1.
VERY VERY
Nelson Textbook of Pediatrics, 7th Edition
IMPORTANT TO
REMEMBER. TO
EVALUATE THE
DX
Etiology and Pathogenesis
• The child gets gets throat or skin infection by
nephritogenic strain of group A beta hemolytic
streptococci - serotype 12 , 4 and 1

• Antibodies to streptoccocus (eg antistreptolysin

O) are formed in his circulation

• 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 Low serum

complement
immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

activation of R-A-A-S retention of water & sodium

blood volume 

edema
hypertension
Streptococcal pharyngitis Streptococcal impetigo

CLINICAL FEATURES
Typical Manifestation
Develop acute nephritic syndrome 1–2 wk after an
antecedent streptococcal pharyngitis or 3–6 wk
after a streptococcal pyoderma.

1. Edema
75% of the patients
Face, periorbital area  lower extremities 
generalized (ascites, pleural effusions) *c/o: dyspnea
o/percussion: stony dullness
o/auscultation: crepitations
2. Proteinuria – usu. normalize after 4 weeks
Not as severe as nephrotic syndrome (only <1g/m 2 /day. In nephrotic, >1g/m2 /day)

3. Oliguria
school child < 400ml/day
preschool child < 300ml/day Normal urine output of a
childL 1-2ml/kg/hour
infant & toddler < 200ml/day
 Typical Manifestation (2)
3. Gross hematuria (65% of patients)
Don’t forget to
Smoky, tea-colored, cola-colored, or fresh
ask about the bloody urine
symptoms of
hypertension in
Microscopical hematuria (almost all
hx ok! Blurred patients)
vission? The urine appears normal, but >3 RBCs/HP
Headache?
Dizziness? are found in centrifuged urine sediment
examined microscopically.

4. Hypertension (50%) – mild to moderate,

typically subsides promptly after diuresis

5. Nonspecific symptoms:
Such as anorexia but with weight gain,
vomiting, general malaise, lethargy,
Meaning, renal function
restores after 5-10days

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
• Circulatory hypervolemia / Congestive heart
failure Sx: Distended JVP,
dyspnea

• Encephalopathy Sx: blurred vision,

confusion, coma,
headache

• Acute renal failure

Laboratory Investigations
1. Urinalysis
2. Bacteriological and Serological test
3. Renal function test
4. Full blood count
5. Serum complement levels
6. Kidney ultrasound
 Laboratory Investigations
Defined as >3-5 RBCs
per high power field,
Urinalysis in a freshly voided and
• Macroscopic hematuria: Rusty or tea-color. centrifuged urine

• Microscopy: leukocytes, red blood cell casts

(pathognomonic), and granular casts.
• Proteinuria 2+. (Nephrotic-range proteinuria occurs in <5% of
patients.)
• Pyuria. The urine contains large amounts of fibrin degradation
products, and fibrinopeptides.
Why I say it’s pathognomonic? Hehee, because, in some
other cases, there may be red urine + positive blood on
dipstick and microscopy, but there will BE NO RBC
CASTS FOUND! It suggests that there is a urinary tract
bleeding from a site beyond the renal tubules. Still
however, if there is no casts, we can’t exclude a
glomerular etiology!

Read more in Nelson page 757, interestingggg =)

 Laboratory Investigations
• Evidences of streptococcal infection
– Throat or skin cultures.
– Antistreptolysin O (ASO) titer. Pharyngitis (80%), skin infections
(<50%).
– Anti-deoxyribonuclease (DNase) B level. Pharyngitis (98%), skin
infections (80%). NOT DONE IN MSIA. Not
• Renal function Test needed anyway. If nak buat
jugak, pi try dkt spore
– 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.
– Hyperkalemia, hypocalcaemia, hyponatremia, and metabolic
acidosis are seen only in severe patients.
 (side note)
• Treatment of Hyperkalemia
1. Insulin + glucose (will cause
transcellular shift of K+ into the cell)
2. Sodium bicarbonate
3. Calcium gluconate (as a cardioprotective
agent.Though it does not have an effect on potassium levels in
the blood, it reduces the excitability of cardiomyocytes thus
lowering the likelihood of developing cardiac arrhythmias. )–
IV slow infusion
4. Chelating agent
5. Lastly, consider dialysis (peritoneal
dialysis in children)
 Laboratory Investigations
• Full Blood Count
– A mild normochromic anemia may be present from
hemodilution and low-grade hemolysis.
– Leococytosis maybe present.

• Activation of complements
– Serum C3 level, decrease (90%), return to normal within 6
weeks.
– Serum C4 levels are typically normal.

• Kidney ultrasound
– Not necessary if patient has clear cut acute nephritic
syndrome
 Renal Biopsy
• Patients whose clinical presentation,
laboratory findings, or course is atypical.
• Delay resolution
o oliguria > 2 weeks
o Azotaemia > 3 weeks
o Gross haematuria > 3 weeks
o Persistent proteinuria > 6 months

Paediatric Protocols, 12th Edition

 Light microscope
Not specific for post streptococcal nephritis

• Glomeruli appear enlarged and hypercellular.

• Diffuse mesangial cell proliferation with an increase in mesangial matrix.
• Polymorphonuclear leukocytes are common in glomeruli during the
early stage of the disease.
Electron microscope

• Subepithelial electron-dense deposits or “humps” are present which are

observed on the epithelial side of the glomerular basement membrane
(GBM).
• Gaps or discontinuities of GBM, which is likely indicative of proteinuria
and hematuria.
Immunofluorescence microscopy

• Lumpy-bumpy deposits of immunoglobulin G and complement 3 along

the capillary loops and within the mesangium.
• It is helpful in the differential diagnosis of other entities that may mimic
APSGN clinically, particularly IgA nephropathy.
 Diagnosis
• Acute onset
• Symptoms: edema, oliguria, dark urine,
hypertension
• Urinalysis: RBCs, protein, casts
• Evidences of streptococcal infection:
– Prodromes
– Elavated serum titers of Abs to
streptozymes(ASO)
• Serum C3 - Reduced
 Differential Diagnosis
Points to support Points against
Membrano- a) Nephritic syndrome a) Persistent nephritic syndrome
proliferative b) Elevated ASO titer b) Hypocomplementemia (C3) - >6-8w
GN c) Hypocomplement- c) Marked reduced renal fn.
emia d) Dx – by renal biopsy – “tram-tracking”
GBM
IgA a) Hematuria a) Recurrent
nephropathy b) Associated with b) No period of latency
(Berger’s respiratory illness c) Hypertension & edema – uncommon
disease) (following viral d) ASO – not elevated
syndromes) e) ↑ Serum IgA (15%)
f) Normal serum complement values
g) Focal proliferation, diffuse mesangial
IgA deposits
Henoch- a) Hematuria (80%) a) Characteristic skin rash
Schönlein b) Mild proteinuria b) Assoc. symptoms: Abdominal pain,
Purpura c) Preceding by URTI diarrhea (due to intussusceptions),
(only 50%) athritis, athralgia
c) Normal serum complement values
 Differential Diagnosis
Points to support Points against
Familial a) Nephritic syndrome a) Lack of expected complete
nephritis b) 1-2 days following URTI resolution
(Alport b) Progressive proteinuria after 2nd decade
Syndrome) of life
c) Association with non-renal
manifestations: hearing deficits &
ocular abnormalities
SLE a) Occurs in 30–70% of a) Lack of expected complete
nephritis children resolution
(Nephritic- b) Hematuria b) Association with non-renal
Grade I & II, c) Mild proteinuria manifestations
Nephrotic- d) Hypocomplement-emia c) C4 also depressed.
Grade III)
(C3) d) Detection of anti-nuclear antibodies.
Other a) Acute nephritic a) Missing evidence of a prior
chronic syndrome streptococcal infection
infections: b) Similar histopathologic b) Treatment is unresponsive
findings
c) Hypocomplementemia
*Additional notes after bedsite with dr faisal

Some other differential diagnosis “vomited”

by the medical students were:

1. Allergic
Apart from swelling + rashes, in history taking:
• Any shortness of breath?
• Any itchiness?
• Any history of allergy?
• Any family history of allergy?
2. Nephrotic syndrome (the ones with hematuria. Homework! =P)
3. Rheumatic fever?
• Meet the Jones criteria?
 Treatment
• Treatment of APSGN is largely that of
supportive care.
• Usually, patients undergo a spontaneous
diuresis within 7 to 10 days after the onset
of their illness - strict monitoring –
nephrotic chart + fluid restriction until
diuresis
• Management is directed at treating the
acute effects of renal insufficiency and
hypertension
 • Diuretics
Due to renal
insufficiency
• Diet
Fluid restriction– during oliguric phase
Sodium restriction is necessary
Protein restriction is unnecessary

• Antibiotics
Post strep GN is a A 10-day course of systemic antibiotic therapy with
sequelae after 2-
penicillin V is recommended to limit the spread of the
3weeks (not antigen-
mediated, but immune- nephritogenic organisms.
mediated). Hence the Antibiotic therapy does not affect the natural history of
need to administer
glomerulonephritis.
antibiotic, to get rid of
any strep left and
prevent further
immune-mediated cx Paediatric Protocols, 12th Edition
 Treatment for complications
Recheck Oral Other
Bed BP ½ Add oral
nifedipine
rest hour Furosemide AHT
later

Significant asymptomatic hypertension

Look for Reduce BP Next

Emergency Target BP by 25% of reduction of
signs and management
indicated control target BP 75% over 48
symptoms over 3-12 hrs hrs

symptomatic, severe hypertension or hypertensive emergency / encephalopathy

Paediatric Protocols, 12th Edition

Paediatric Protocols, 12th Edition
Treatment for complications
-Give O2 Acute
-prop patient up
-ventilatory support if necessary pulmonary
edema
IV
furosemide

Fluid restriction
-withhold fluid for 24 Hrs if
possiible

Consider dialysis if no
response to diuretics

Paediatric Protocols, 12th Edition

Treatment for complications
Acute Renal Failure
Correcting
electrolyte
Monitor BP & Limiting fluid
imbalance ie
managing intake in
life
hypertension oliguric state
threatening
hyperkalemia

Implementing Optimizing nutritional

acute dialysis intake (since ARF pt
usually hypercatabolic)

Paediatric Protocols, 12th Edition

 Follow-up
• for at least 1 year.
• monitor BP at every visit
• do urinalysis and renal func􀆟 on
to evaluate recovery.
• repeat C3 levels 6 weeks later if not
already normalised by time of discharge

Paediatric Protocols, 12th Edition

 Prognosis
• short term outcome: excellent, mortality
<0.5%.
• long term outcome: 1.8% of children
develop chronic kidney disease
• following post streptococcal AGN. These
children should be referred to the
paediatric nephrologists for further
evaluation and management.

Paediatric Protocols, 12th Edition