1. Nephrotic Syndrome
Presented by
Dr. Huma Daniel

2. Characteristic Features
Heavy proteinuria > 40mg/m2/hr
Hypoalbuminemia <2.5g/dl
Edema
Hyperlipidema >250mg/dl

3. Epidemiology 15 times more common in children than adults
incidence is 2-3/ 100,000 children per year
incidence higher is Asian population 16/100,000 children

4. Etiology IDIOPATHIC NEPHROTIC SYNDROME (90%)
Minimal change disease 85%
Mesengial proliferation 5%
Focal segmental glomerulosclerosis 10%

5. Etiology SECONDARY NEPHROTIC SYNDROME (10%): 1. Renal Causes:
Membranous nephropathy
Membranoproliferative glomerulonephtritis
2. Extra Renal Causes:
Infection
Drugs
Neoplasia
Systemic diseases
Allergic reactions
Familial disorders
Circulatory disorders

6. Pathophysiology

7. Pathophysiology

8. IDIOPATHIC NEPHROTIC SYNDROME

9. SECONDARY NEPHROTIC SYNDROME

10. Clinical Features HISTORY Preceding flu-like illness General health
(anorexia, wt. gain ,lethargy)
Edema
Urinary symptoms
(hematuria, oliguria)
Infection, diarrhea, abd. pain
Drug intake
Past history

11. Clinical Features EXAMINAITON Vital & bp Height & weight for age
Anemia
Periorbital puffiness
Lymphadenopathy
Pleural effusion, ascites
Ankle, sacral, genital edema

12. Clinical Features

13. Diagnosis URINE ANALYSIS: PROTEINURIA: 3+ Or 4+
24HRS URINARY PROTEIN EXCRETION: Children : >40mg/m2/hr
URINARY PROTEIN TO CREATININE RATIO: >2.0
MICROSCOPIC HEMATURIA: 20%
PUS CELLS: underlying UTI
CELLULAR CASTS: not in minimal change disease, common in other forms

14. Diagnosis SERUM: S. CREATININE: Normal S. CHOLESTROL: Elevated
S. ALBUMIN:
<2.5g/dl
C3 & C4:
TOTAL CALCIUM:
Decreased

15. Diagnosis OTHERS: X-RAY CHEST: VITRAL SEROLOGY: BLOOD COUNTS:
HBV associated with membranous nephritis &
HCV with mesengial proliferation
BLOOD COUNTS:
TLC & DLC Normal
ESR raised
X-RAY CHEST:
R/O pulmonary pathology or pleural effusion

16. Diagnosis MANTOUX TEST: RENAL BIOPSY R/O Tb before starting steroids
ANA: R/O SLE

17. SCHEME FOR MANAGEMENT OF CHILDREN WITH NEPHROTIC SYNDROME

18. SCHEME FOR MANAGEMENT OF CHILDREN WITH NEPHROTIC SYNDROME

19. SCHEME FOR MANAGEMENT OF CHILDREN WITH NEPHROTIC SYNDROME

20. Management of Nephrotic Syndrome
DIETARY ADVICE:
A balanced diet adequate in proteins and calories is recommended
Edema no added salt
foods high in sodium avoided

21. Management of Nephrotic Syndrome
DIURETICS:
INDICATIONS:
Severe symptomatic edema
Steroid toxicity or steroid contraindicated
DOSAGE & ADMINISTRATION:
Chlorothiazide 10mg/kg/doze I/V 12hrly or
Metolazome 0.1mg/kg/doze PO bid followed by Furosemide 30mins later 1-2mg/kg/doze I/V 12 hrly

22. Management of Nephrotic Syndrome
ROLE OF INTRAVENOUS ALBUMIN
INDICATIONS:
Signs of hypovolemia
DOSAGE & ADMINISTRATION:
I/V salt poor 25% albumin infusion
0.5-1 gm/kg/doze over 6-12 hrs followed by Frusemide 1-2 mg/kg/doze I/V

23. Management of Nephrotic Syndrome
CORTICOSTEROID THERAPY:
DOSAGE & ADMINISTRATION:
Prednisolone 60mg/m2/day (max 80mg) divided into 2-3 doses for 4 consecutive wks
80-90% remission in 10days
after 4wks course, prednisolone tapered to 40mg/m2/day on alternate days as single morning dose
Alternate day dose tapered slowly & discontinued over 2-3 months

24. Management of Nephrotic Syndrome
REPONSE TO STEROID:
10% respond by first week
70% by second week
85% by third week
92% by forth week

25. Management of Nephrotic Syndrome
CORTICOSTEROID THERAPY
RESPONSE TO STEROIDS:
STEROID RESPONSIVE PATIENTS:
70-90% pts . Responsive
>75% at least 1 relapse
Treated using protocol already described
FREQUENT RELAPSER:
4 or more relapses in 12 months
Alternate day prednisolone tapered over 6 months
Alternative therapy

26. Management of Nephrotic Syndrome
CORTICOSTEROID THERAPY
RESPONSE TO STEROIDS:
STEROID DEPENDENT:
Relapses on 2 consective occasion as prenisolone is being decreased or within 28daysof stopping prednisolone
Alternative therapy
STEROID RESISTANT:
Fail to respond to corticosteroid therapy within 8 wks

27. Management of Nephrotic Syndrome
ALTERNATIVE THERAPY:
INDICATIONS:
steroid dependent
frequent relapsers
steroid responsive
unwanted effects of steroids
CYCLOPHOSPAMIDE:
Prolong duration of remission & reduce no. of relapses
DOSE: 2-3 mg/kg/24hrs OD For 8-12 wks
Alternate day prednisolone often continued

28. Management of Nephrotic Syndrome
METHYLPREDNISOLONE:
DOSE:30mg/kg I/V bolus (max 1 gm), first 6 doses on alternate day followed by tapering regimen for 18 months
Cyclophosphamide may be added
CYCLOSPORIN:
DOSE: 3-6mg/kg/24hrs in 12hrly
ACE INHIBITORS:
adjunct therapy to reduce proteinuria is steroid resistant pts

29. Complications INFECTIONS:
SBP, pneumonia, cellulitis, UTI, disseminated varicella
THROMBOEMBOLISM:
Renal vein thrombosis, pulmonary embolism, saggital sinus thrombosis of arterial & venous catheters

30. Complications OTHERS: Deficiencies of coagulation factors 1X, X1,& X11
Reduced levels of vitamin D
Acute renal failure
Hypertension
Malnutrition
Flare up of tuberculosis
Steroid & anti-metabolite related toxicity
Exacerbation by immunization

31. Differential Diagnosis
Other forms of glomerulonephritis including post streptococcal glomerulonephritis
Pyelonephritis
Obstructive Uropathies
Hemolytic Uremic Syndrome
Fever, Exercise, Orthostatic protein urea
Renal Failure
Congestive cardiac failure
Liver failure

32. Follow-up Blood CP Urine RE Growth parameters General examination
Blood Pressure
Eye examination
RFTs
Serum electrolytes
BSR

33. Follow-up
Serum calcium
X-Ray wrist
X-Ray spine
Chest X-Ray
PT/APTT

34. Prognosis
Children responding to steroid rapidly & have no relapses in first 6 months infrequently relapsing
steroid responsiveness, no underlying pathology better outcome in INS
children with steroid resistant nephrotic syndrome poor prognosis
Mortality rate 1-2 %

35. Congenital Nephrotic Syndrome
Infants who develop nephrotic syndrome within first 3 months of life
ETIOLOGY:
Finish type congenital nephrotic syndrome
Congenital infections
HIV/HBV
Diffused mesengial sclerosis
Drash syndrome
Minimal change disease
Focal segmental glomerulosclerosis

36. Congenital Nephrotic Syndrome
CLINICAL FEATURES
Massive proteinuria ( alpha fetoprotein)
Large placenta
marked edema
prematurity
respiratory distress
separation of cranial
sutures
Recurrent infections

37. Congenital Nephrotic Syndrome
TREATMENT:
ACE inhibitors + Indomethacin + unilateral neprectomy
B/L nephrectomy chronic dialysis & kidney transplant
no role of steroid or immunosuppressive agents
PROGNOSIS:
Poor
Progressive renal failure
Death by 5 yrs age

38. Thank You