Psgn nephrotic syndrome

Acute Nephritis and Nephrotic
syndrome
DR SHIV DUBEY

CASE SCENARIO
A 7 year old Male boy was admitted with the chief complaints
of facial puffiness and passing smoky and frothy urine for 1
week. The facial puffiness initially started off as periorbital
oedema. Urinary output was also decreased. He also
complaint of fever for one week which was of low grade.
There is no history of sore throat, flu, blood transfusion,
nausea, and vomiting, rashes, dyspnoea and chest pain.
General examinations revealed pallor, blood pressure of
139/96 mmHg, and an erythmatous scaly circular skin lesion
on his right elbow. Urine biochemistry revealed protein 3+,
RBC 4+. Blood urea was raised to 500 umol/L.C3 levels were
low, ASO –ve, Anti DNAse B +VE. USG showed B/L enlarged
and pale kidneys.

Definition
• An acute inflammation of renal glomerular
paranchyma due to deposition of immune
complexes characterized by sudden onset of
 oliguria,
 hematuria,
 Proteinuria,
 hypertension and
 edema

Ac Nephritic Syndrome
Clinical Presentation
● History
● Age 5-12 yr (uncommon< 3 yrs)
A history suggestive of preceding streptococcal infection may include
a preceding infective episode such as pharyngitis, tonsillitis, or
pyoderma.
In general, the latent period is 1-2 weeks after a throat infection and 3-
6 weeks after a skin infection.
The onset of signs and symptoms at the same time as pharyngitis
(also called synpharyngitic nephritis) is more likely to be
immunoglobulin A (IgA) nephropathy rather than APSGN.

pathogenesis
Throat/skin infection by gp A beta hemolytic
streptococci (serotype 12, 4 and 1)
Antibodies to streptococcus (antistreptolysin
O) are formed in the circulation
Antigen-antibody circulating immune
complexes are subsequently deposited along
the glomerular basement membrane (GBM)

Etiology
• Streptococcal infection
• Non streptococcal
– Bacterial : Infective endocarditis, shunt nephritis,
typhoid, syphilis, S.pneumoniae, meningococcal
– Viral : HBV, mumps, varicella, ECHO, coxsackie,
measles, infective mononucleosis.
– Fungal and parasitic infections
• Autoimmune
– Goodpasture syndrome, HSP, SLE, IgA nephropathy
• Miscellaneous
– GBS, Irradiation to Wilms tumor

Aetiology of Ac Nephritic
Synd

Pathology
• Gross:
- Both kidneys enlarged
- Ischemic
• Microscopy:
- Glomeruli enlarged, infiltrated by polymorphs
- Epithelial crescents
• Immunofluorescence:
- Lumpy-bumpy deposits of IgG, antigen and C3
• Electron microscopy:
- Mesangial proliferation and mesangial matrix
deposition
- Humps of immune complex depositions seen on
the epithelial side of GBM

Understanding Serology In Ac
Nephritis

Post Streptococcal Glomerulonephritis
- Etiology
• Streptococcal serotypes involved
– Pharyngitis :
• Types 1, 3, 4, 12, 25, 49
– Pyoderma
• Types 2,47, 49, 55, 57, 60
• Streptococcal antigens involved in immune response :
– Zymogen precursor of exotoxin
– Glutaraldehyde phosphate dehydrogenase
Usually occurs 7-14 days after pharyngitis and
2 wks – 6 wks after skin infection

Age group
5 – 12 yrs (Rare before 3 yrs)
Sex
Male predominance
Socioeconomic group
Common in low socioeconomic group
Seasonal variation
During winter and rainy season serotype 12 causes
Ac. pharyngitis
During summer – serotype 49 causes skin infections

Clinical Features
• Puffiness of face – more in the mornings
• Edema feet
• Oliguria ( < 400 ml / m2)
• Hematuria (cola coloured urine)
• Breathlessness due to hypertensive heart failure
• Fever
• Hypertension
• Abdominal pain
• Atypical presentations:
– Hypertensive encephalopathy – confusion, convulsions, etc.
– Pulomonary edema – due to CHF
– Acute renal failure

Investigations - For kidney injury :
Urine analysis
• Proteinuria – non selective
• Hematuria :
- Macroscopic : Plenty of RBC &
RBC casts in urine
- Microscopic : > 5 RBC / HPF in 10 ml
centrifuged urine
• Hypocomplementemia
Kidney function Tests
• Blood urea
• S. creatinine (h due to i GFR)

Investigations - For Etiological Factores
1. Culture of organisms in throat or skin
2. Antistreptokinase, antihyalironidase – increased
3. ASO titer is increased if the disease is due to sero
type 12 (throat infection) but not increased if the
disease is due to type 49 because subcutaneous lipids
prevent the percolation of ASO titer in blood
4. Single most specific test : Anti DNAse – B

Complications
1. Hypertensive encephalopathy
Failure of autoregulatory system of the vessels of brain
due to acute rise of blood pressure
– Altered sensorium, convulsions, etc,
2. Hypertensive heart failure
3. Hypocalcemia
4. Hyperphosphatemia
5. Hyperkalemia
6. Acute renal failure

NATURAL COURSE
• The acute phase generally resolves within 6–8
wk.
• The serum C3 level, urinary protein excretion
and hypertension usually normalize by 6–8 wk
after onset
• Persistent microscopic hematuria may persist
for 1–2 yr after the initial presentation.

INDICATIONS FOR BIOPSY
• Renal biopsy should be considered
– presence of acute renal failure or nephrotic
syndrome.
– absence of evidence of streptococcal infection
– normal complement levels.
– when hematuria and proteinuria, diminished renal
function, and/or a low C3 level persist more than
2 mo after onset.

Management Principles
• Eliminate Strep. infection with antibiotics
• Supportive therapy
• Diuretics and antihypertensives to control BP
and extra-cellular fluid volume

Prevention
Any streptococcal sore throat or skin infection should be
treated with
• Benzyl Penicilline IM single dose
6 Lakh IU - for < 6 yrs age
12 Lakh IU - for > 6 yrs age (or)
• Oral Penicilline : 125 mg BID for 10 days
• Ampicilline 100 mg / kg / d twice daily for 10 days
• Amoxicilline 50 mg / kg /d for 19 days
There is no role for long term prophylaxis in acute nephritis

Does the treatment of streptococcal skin or
pharyngeal infection prevent APSGN?
• No study has ever demonstrated that
treatment of impetigo or pharyngitis prevents
renal complications in the index case.
• However, treatment lessens the likelihood of
contagious spread to hosts who may be
susceptible to renal complications

Some facts about APSGN
• Acute rheumatic fever does not occur after
the skin infection
• About 80-85% of children with APSGN
develop elevated ASO titers.
• Streptolysin O is bound to lipids in the skin
so that the % of individual with streptococcal
impetigo who develop +ve ASO titers is much
lower. So normal ASO titer does not rule out
recent strep. Infection
• Streptozyme test will be positive in > 95% of
children with documented Strep. infection

D/D
● Membranoproliferative glomerulonephritis (MPGN) — The
presentation of MPGN may be indistinguishable initially from
PSGN. It typically presents with hematuria, hypertension,
proteinuria, and hypocomplementemia following an upper
respiratory infection. However, patients with MPGN continue to
have persistent nephritis and hypocomplementemia beyond four to
six weeks and possibly a further elevation in serum creatinine.
● IgA nephropathy — Patients with IgA nephropathy often present
after an upper respiratory infection, similar to the presentation of
patients with PSGN. Potential distinguishing features from PSGN
include a shorter time between the antecedent illness and
hematuria is (less than 5 versus more than 10 days in PSGN) and a
history of prior episodes of gross hematuria since recurrence is rare
in PSGN
● Lupus nephritis and Henoch-Schönlein purpura (IgA vasculitis)
nephritis share similar features to PSGN. However, extrarenal
manifestations of the underlying systemic diseases and laboratory
testing should differentiate them from PSGN..

NEPHROTIC SYNDROME
THE SWOLLEN CHILD

DEFINITION
It is a clinical syndrome of:
1. Nephrotic range proteinuria
 >1 g/m2/day or 40 mg/kg/hour
2. Hypoproteinemia
 ↓ serum albumin < 2.5 g/dL
3. Oedema
4. Hypercholestrolnemia
 > 220 mg/dL

CLASSIFICATION
Primary or Idiopathic Secondary
• no known aetiology
• Minimal change disease (MCD)
• Focal Segmental Glomerulosclerosis
(FSGS)
• Membranous nephropathy
• congenital nephrotic syndrome
• Systemic disease
- SLE
- HSP
- DM
• Infections
- Post-infectious GN
- Hepatitis B
- Syphilis
- Malaria
• Drugs
• Toxins and allergen
- bee sting
- food allergy

Minimal Change Disease
 most common (70-80%)
 M:F = 2:1
 < 7 years old
 steroid-sensitive nephrotic syndrome
 do not progress to renal failure
 often precipitated by respiratory infections
 Features:
• age between 1 and 10 years
• no macroscopic haematuria
• normal blood pressure
• normal complement levels
• normal renal function.

PATHOPHYSIOLOGY
• Primary disorder:
Loss of glomerular
BM sialoprotein
Loss of normal
negative charge
Increase glomerular
permeability
Massive proteinuria
Decrease serum
protein
Decrease plasma
oncotic pres.
Fluid shift from
vascular to interstitial
Contraction of
plasma volume

COMPLICATION
• Infection
 Spontaneous bacterial peritonitis, cellulitis, bacteriemia (S.pneumoniae, E.coli)
 Steroid and immunosuppressant toxicity
• Hypovolaemia
 abdominal pain and may feel faint, cold peripheries, poor pulse volume,
hypotension, and haemoconcentration.
 A low urinary sodium (<20mmol/L) and a high packed cell volume
• Thromboembolism
 hypercoagulable state due to urinary losses of antithrombin, thrombocytosis
 exacerbated by steroid therapy
 increased synthesis of clotting factors
 increased blood viscosity from the raised haematocrit,
 This is usually arterial and may affect the brain, limbs and splanchnic circulation
• Hypercholesteroleamia
• Acute renal failure (rare)

CLINICAL MANIFESTATION
• Sudden onset of dependent pitting oedema
- periorbital
- scrotal or vulva
- ankle or leg
• Weight gain
• Ascites
- abdominal pain
- malaise
• Diarrhoea (dt intestinal oedema)
• Respiratory distress (dt pulm. oedema)

DIFFERENTIAL DIAGNOSIS
 Main ddx:
1. Anaphylaxis
2. Cellulitis (orbital,periorbital)
3. Angioedema
4. Nephrotic synd.
5. Other causes of hypoalbuminemia
 Transient proteinuria
 Protein losing enteropathy
 CLD

HISTORY TAKING
• First time or relapse???
• History of edema noted on awakening in the morning or sudden swelling??
 Distribution
 Colour changes
 Initiating factor? (bee sting)
 Painful??
• Weight gain (edema)
• Respiratory distress
 Breathlessness
• Diarrhea
• Urine: frothy
• Pass medical and drug history: recent illness, allergies, asthma
• Family history

PHYSICAL EXAMINATION
• Assessment of hydration status identifies fluid imbalances (dehydration,
overhydration)
• Blood pressure: hypertension
• Henoch-Schönlein purpura (purpura)
• Systemic lupus erythematosus (malar rash)
• Rales heard on lung auscultation suggest extravascular fluid from overload or
hypoalbuminemia
• Palpation and percussion of the abdomen may reveal ascites or masses
• Liver enlargement is present in several multisystem diseases (systemic lupus
erythematosus, infections, polycystic disease) and in glomerulosclerosis

DEFINITION FOR DX & TX OF IDIOPATHIC
NS
REMISSION:
Morning Urine dipstix nil/trace for 3 consecutive days in a patient , in relapse.
RELAPSE:
Morning urine dipstix ++ or more for 3 consecutive days, in a child earlier in
remission.
FREQUENT RELAPSES:
Two or more relapses within 6 months of initial response or four or more
relapses within any 12 month period.
STEROID DEPENDENCE:
relapse occurring during the period of steroid taper or within 14 days of its
cessation.
STEROID RESISTANCE:
Failure to achieve remission in spite of 4 weeks of standard prednisolone
therapy.

• Urinalysis - 3+ to 4+ proteinuria
• Renal Function
–Spot UPC ratio > 2.0
–UPE > 40 mg/m2/hr
• Serum Creatinine – normal or elevated
• Serum albumin - < 2.5 gm/dl
• Serum Cholesterol/ TGA levels – elevated
• Serum Complement levels – Normal or low
- Nelson Textbook of Paediatrics, Vol 2, 19th Edition, page 1804

Additional Tests
• C3 and antistreptolysin O
• Chest X ray and tuberculin test
• ANA
• Hepatitis B surface antigen
Ghai Essential Paediatrics,8th edition, page 478
Indications for Biopsy
• Age below 12 months or >12 yr
• Gross or persistent microscopic hematuria
• Low blood C3
• Hypertension
• Impaired renal Function
• SRNS

Initial Episode
• High protein diet
• Salt moderation
• Treatment of infections
• If significant edema – diuretics Aldosterone
antagonist ( Furosemide, spironolactone )
• Corticosteroid therapy with Prednisolone or
prednisone
– ( 2mg/kg per day for 6 weeks followed by
1.5 mg/kg single morning dose on alternate days for
6 weeks )
Ghai Essential Paediatrics,8th edition, page 476, 477

Subsequent course
• Relapse
– Infrequent Relapsers : 3 or less relapses per year
– Frequent Relapsers : 4 or more relapses per year
• Steroid therapy
– Steroid dependant : relapse following dose
reduction or discontinuation
– Steroid resistant : Partial or no response to initial
treatment

Management of Relapse
• Parent Education
• Symptomatic therapy for infections in case of
low grade proteinuria
• Persistent proteinuria ( 3 - 4+ ) –
– Prednisolone
( 2mg/kg/day until protein is negative for 3 days )
1.5 mg/kg on alternate days for 4 weeks )

Frequent Relapses
• Alternate Day prednisolone
• Steroid sparing agents
– Levamisole ( 2 – 2.5 mg/kg )
– Cyclophosphamide ( 2 – 2.5 mg/kg/day)
– Mycophenolate Mofetil ( 20 – 25 mg/kg/day )
– Cyclosporin ( 4 – 5 mg/kg/day )
– Tacrolimus (0.1 – 0.2 mg/kg/day )
– Rituximab ( 375mg/m2 IV once a week )

Complications
• Edema
• Infections
• Thrombotic complications
• Hypovolaemia and Acute renal Failure
• Steroid Toxicity

Steroid Resistant Nephrotic Syndrome
• Diagnosis – Lack of response to prednisolone
therapy for 4 weeks
• Indication for renal biopsy
• FSGS

TREATING FRNS/SRNS
ALKYLATING AGENTS
Cyclophospamide
Chlorambucil
CALCINEURINE
INHIBITORS
Cyclosporin
Tacrolimus
LEVAMISOLE MYCOPHENOLATE
MOFETIL

Congenital Nephrotic Syndrome
• Presents in first 3 months of life
• Anasarca, hypoalbuminaemia, oliguria
‘Finnish’ Type Nephrotic Syndrome
• Antenatally detectable :
– Raised AFP in maternal serum and amniotic fluid
• Complications
– Failure to thrive
– Infections
– Hypothyroidism
– Renal Failure ( 2 – 3 yrs )

Due to loss of
proteins in the urine
Due to ↓ oncotic
pressure
•Immunoglobulin
↑susceptibility to infection
•antithrombin III and proteins C
and S Thromboembolism
•vit D–binding protein 
vit D deficiency
•Transferrin
Iron deficiency anemia
•Hyperlipidaemia
•Hypovolemia
Acute renal failure
•Anasarca
risk of cellulitis, bacterial
peritonitis with ascites
,large pleural effusions or
pulmonary edema

Steroid toxicity
•Stunting of growth
•Cataracts
•Striae
•Severe cushingoid features - behavioural changes, a
rounded face, central obesity and the tendency to bruise
more easily, hirsutism
•Osteoporosis
•Proximal myopathy
•Recurrent infection dt low immunity

MANAGEMENT
• Mx of oedematous state
Bed rest to be avoided as there is a tendency of
hrpercoagulability
Dietary advice: no added salt, normal protein with
adequate calories
Hypovolaemia: infuse salt poor albumin or 5%
albumin, plasma protein derivatives or human
plasma
Diuretics

• Mx complication:
 Infection: parenteral penicillin and a third
generation cephalosporin (in primary peritonitis)
 If exposed to chickenpox and measles varicella-
zoster immunoglobulin (VZIG) should be given
within 72 hours after exposure to chickenpox /
single dose of intravenous immunoglobulin.
 Thrombosis : Warfarin, low-dose aspirin all have
been used to minimize the risk of clots.

– URINE ALBUMIN MONITORING
– It is advocated that monitoring of urine albumin
excretion be done regularly either at home with
urinary dipstix or at the nearest health centre.
–NEPHROTIC DIARY

diary
date Urine dipstick medication
1.3.16 4+ Tab pred BD
2.3.16 3+ TAB PRED BD
5.3.16 trace TAB PRED BD
8.3.16 trace No medication

EDUCATION
– Education:
Parents and school teachers should be provided
with information regarding the disease which
includes:
1. Advice and precaution of infection
2. Danger of sudden steroid withdrawal (adrenal
crisis)

VACCINATION
Yearly influenza vaccination
Pneumococcal vaccination should be administered to all patients
with INS to reduce the risk of pneumococcal infection.
Routine childhood vaccines with live virus strains are
contraindicated in patients taking steroids and until off steroid
treatment for a minimum of 1 month.
varicella vaccination
Routine, nonlive viral vaccines should be administered
according to their recommended schedules.
Hepatitis B vaccination
Hib vaccination

Psgn nephrotic syndrome

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