Nephrotic Syndrome

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Nephrotic Syndrome
Presented by Dr. Huma Daniel
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Characteristic Features

• Heavy proteinuria gt 40mg/m2/hr
• Hypoalbuminemia lt2.5g/dl
• Edema
• Hyperlipidema gt250mg/dl

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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

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Etiology

• IDIOPATHIC NEPHROTIC SYNDROME (90)
• Minimal change disease 85
• Mesengial proliferation 5
• Focal segmental glomerulosclerosis 10

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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

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Pathophysiology
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Pathophysiology
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IDIOPATHIC NEPHROTIC SYNDROME
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SECONDARY NEPHROTIC SYNDROME
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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

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Clinical Features

• EXAMINAITON
• Vital bp
• Height weight for age
• Anemia
• Periorbital puffiness
• Lymphadenopathy
• Pleural effusion, ascites
• Ankle, sacral, genital edema

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Clinical Features
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Diagnosis

• URINE ANALYSIS
• PROTEINURIA 3 Or 4
• 24HRS URINARY PROTEIN EXCRETION Children
  gt40mg/m2/hr
• URINARY PROTEIN TO CREATININE RATIO gt2.0
• MICROSCOPIC HEMATURIA 20
• PUS CELLS underlying UTI
• CELLULAR CASTS not in minimal change disease,
  common in other forms

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Diagnosis

• SERUM
• S. CREATININE
• Normal
• S. CHOLESTROL
• Elevated
• S. ALBUMIN
• lt2.5g/dl
• C3 C4
• Normal
• TOTAL CALCIUM
• Decreased

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Diagnosis

• OTHERS
• VITRAL SEROLOGY
• 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

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Diagnosis

• MANTOUX TEST
• R/O Tb before starting steroids
• RENAL BIOPSY
• ANA R/O SLE

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SCHEME FOR MANAGEMENT OF CHILDREN WITH NEPHROTIC
SYNDROME
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SCHEME FOR MANAGEMENT OF CHILDREN WITH NEPHROTIC
SYNDROME
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SCHEME FOR MANAGEMENT OF CHILDREN WITH NEPHROTIC
SYNDROME
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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

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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

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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

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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

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Management of Nephrotic Syndrome

• REPONSE TO STEROID
• 10 respond by first week
• 70 by second week
• 85 by third week
• 92 by forth week

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Management of Nephrotic Syndrome

• CORTICOSTEROID THERAPY
• RESPONSE TO STEROIDS
• STEROID RESPONSIVE PATIENTS
• 70-90 pts . Responsive
• gt75 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

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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
• Alternative therapy

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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

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Management of Nephrotic Syndrome

• METHYLPREDNISOLONE
• DOSE30mg/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

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Complications

• INFECTIONS
• SBP, pneumonia, cellulitis, UTI, disseminated
  varicella
• THROMBOEMBOLISM
• Renal vein thrombosis, pulmonary embolism,
  saggital sinus thrombosis of arterial venous
  catheters

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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

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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

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Follow-up

• Blood CP
• Urine RE
• Growth parameters
• General examination
• Blood Pressure
• Eye examination
• RFTs
• Serum electrolytes
• BSR

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Follow-up

• Serum calcium
• X-Ray wrist
• X-Ray spine
• Chest X-Ray
• PT/APTT

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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

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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

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Congenital Nephrotic Syndrome

• CLINICAL FEATURES
• Massive proteinuria ( alpha fetoprotein)
• Large placenta
• marked edema
• prematurity
• respiratory distress
• separation of cranial
• sutures
• Recurrent infections

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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

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Thank You