nephrotic syndrome presented by dr. huma daniel. characteristic features heavy proteinuria >...
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Nephrotic SyndromeNephrotic Syndrome
Presented byPresented byDr. Huma DanielDr. Huma Daniel
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Characteristic Features
• Heavy proteinuria > 40mg/m2/hr
• Hypoalbuminemia <2.5g/dl
• Edema
• Hyperlipidema >250mg/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
Permeability of glom.cap.memb. Proteinuria
Intravascular vol
ADH Renal perfusionpressure
WaterReabsorptnInCollectingducts
Actv. reininAng. ald. sys
Tubular reabsorp.Of Na
Hypoalbuminemia
Hepatic protein synthesis Plasma oncoticpressure
Hyperlipidemia Transudation of fluidfrom intravascularcomp. To interstialspace
Edema
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Pathophysiology
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IDIOPATHIC NEPHROTIC SYNDROME
MINIMAL CHANGE DISEASE FOCALSEGMENTALSCLEROSIS
AGE 2-6yrs 2-10yrsSEX 2:1 male 1:3:1 maleHEMATURIA 10-20% 60-80%HYPERTENSION 10% 20%RENAL FAILURE No progression 10yrsASSOCIATIONS Allergy & Hodgkin NoneSERUM CREATININ Inc. in 15-30% Inc. in 20-40%IMMUNOGENETIC HLA-B8, B12 NoneLIGHT MICROSCOPE Normal Focal sclerosisIMMUNOFLOUR Negative IgM & C3 in lesionsELECTRON MICRO Foot process fusion Foot process fusionSTEROID RESPONSE 90% 15-20%
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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 : >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
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Diagnosis
• SERUM:• S. CREATININE:
Normal• S. CHOLESTROL:
Elevated• S. ALBUMIN:
<2.5g/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
– >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
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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:– 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
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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