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NEPHROTIC SYNDROME

NEPHROTIC SYNDROME. BY : Dr ALPANA SOMALE. COMMONEST TYPE OF RENAL LESION IN CHILDREN. PRIMARY (IDIOPATHIC) 90% MCNS 75% FSGS 10% Mn <5% MPGN 10-15% . SECONDARY TO (10%) INFECTIONS MALIGNANCY DRUGS CONNECTIVE TISSUE DISEASES. TWO MAIN TYPES NEPHROTIC SYNDROME. AND DRUG INDUCED NS.

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NEPHROTIC SYNDROME

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  1. NEPHROTIC SYNDROME BY : Dr ALPANA SOMALE

  2. COMMONEST TYPE OF RENAL LESION IN CHILDREN DR ALPANA KONDEKAR www.peditips.com

  3. PRIMARY (IDIOPATHIC) 90% MCNS 75% FSGS 10% Mn <5% MPGN 10-15% SECONDARY TO (10%) INFECTIONS MALIGNANCY DRUGS CONNECTIVE TISSUE DISEASES TWO MAIN TYPESNEPHROTIC SYNDROME DR ALPANA KONDEKAR www.peditips.com

  4. AND DRUG INDUCED NS DR ALPANA KONDEKAR www.peditips.com

  5. DR ALPANA KONDEKAR www.peditips.com

  6. NEPHROTIC SYNDROME HEAVY PROTEINURIA DR ALPANA KONDEKAR www.peditips.com

  7. PRIMARY 1-8 YR NO HT HEMATURIA 20% N RENAL FUNCN NO EXTRARENAL NORMAL C3 STEROID RESPONSE SECONDARY >8 YR HT HEMATURIA RENAL DYSFUNCN RASH/ARTHRLGIA LOW C3 STEROID RESISTANT CHARACTERISTICS DR ALPANA KONDEKAR www.peditips.com

  8. FINNISH TYPE SCANDINAVIAN DESCENT 1:8000 A-RECESSIVE NPHN1 Gene mutn(CR19) PATHO: DILTN PROX TUBULES + MESANGIAL HYPERCELLULARITY + GLOMERULAR SCLEROSIS+ AETIO: GENETIC: SYPH;TORCH; HIV/HBV AT BIRTH / < 3 Mo DENYS DRASH SYNDROME CR11; WILMS TUMOR GENE MUTN CONGENITAL NS • ^AFP IN AMNIOTIC FLUID • LARGE PLACENTA • PREMATURITY • MARKED EDEMA • MASSIVE PROTEINURIA • RESP DISTRESS • CR SUTURE SEPARATION • RECURRENT INFECTION • PERSISTANT EDEMA • PROGRESSIVE R FAILURE • DEATH BY 5 YR DR ALPANA KONDEKAR www.peditips.com

  9. TREATMENT CONG NS • ACE INHIBITORS • INDOMETHACIN • UNILAT NEPHRECTOMY • B/L NEPHRECTOMY • CHRONIC DIALYSIS • AGGRESSIVE NUTRITIONALSUP • RENAL TRANSPLANT DR ALPANA KONDEKAR www.peditips.com

  10. T CELL DYSFUNCTION ↓↓ alteration of cytokines IL2/SIRS====PROTEINURIA ↓ ↓ ↓ ↓ INTRAVASC VOLUME LOSS OF SIALOPROTEINS ↓ (NEG CHARGED GLYOPROTEINS) RELEASE OF ADH ↓ ↓ MASSIVE PROTEINURIA ↓ ↓ HYOALBUMINEMIA ↓ ↓ ↓↓ PLASMA ONCOTIC PRESSURE---------------- ↓ ↓ TRANSUDATION OF FLUID -----ADH---- ↓↓ RENAL PERFUSION ↓ PRESSURE FROM IVC ---> INTERSTITIUM ↓ ↓ ↓ EDEMA ACTIVATION OF RENIN ANGIOTENSIN/ALDOSTERONE AXIS ↑ ↓ ---------------------------------TUBULAR REABSORPTION PATHOGENESIS DR ALPANA KONDEKAR www.peditips.com

  11. COMPLICATIONS NS • INFECTIONS • STREPT PNEUMO, TB, E COLI, H INFLU, VARICELLA • SKIN, GI, PERITONEUM,SEPSIS; UTI,RS • THROMBOEMBOLIC • DUE TO LOSS OF AT3, PROTEIN C • ANEMIA (LOSS OF TRANSFERRIN) • HYPO Ca Zn Cu Na • Ac RENAL SHUTDOWN ; • CIRCULATORY INSUFFICIENCY • (DUE TO HYOVOLEMIA) • RETARDATION OF GROWTH • DUE TO SOMATOMEDIN & NITROGEN LOSS DR ALPANA KONDEKAR www.peditips.com

  12. INVESTIGATIONS NS • PROTEINURIA • 40MG/M2/HR • 1GM/M2/DAY • 50MG/KG/DAY • 4+ MASSIVE ALBUMINURIA • TG/CHOLESTEROL >220 • SERUM ALB <2.5 • SERUM PROTEIN EPP • low albumin, increased alpha-2 (macroglobulin), reduced gamma • NORMAL C3 • URINE ALB/CR >3; Sr Ca LOW, DR ALPANA KONDEKAR www.peditips.com

  13. INDICATIONS FOR KIDNEY BIOPSY • ATYPICAL NS; AGE <2 Y & >9 Y • PERSISTANT GROSS HEMATURIA • PERSISTANT HT / HYPOCOMPLEMENTEMIA • PERSISTANT AZOTEMIA • CONGENITAL NS • SRNS • FREQUENT RELAPSER • SLE / FAMILY HISTORY NS DR ALPANA KONDEKAR www.peditips.com

  14. BASIC DEFINITIONS • REMISSION: URINE NEG/TRACE FOR PROTEIN FOR 3 CONSEQ DAYS • RELAPSE: 3+PROTEINURIA FOR 3 CONSEQUTIVE DAYS DURING REMISSION • FREQUENT RELAPSER: >2 RELAPSES IN 6 MONTH; >3 IN 1 YR • STEROID RESPONDER: REMISSION WITHIN 8 WEEKS OF STEROID Rx • STEROID RESISTANCE: NO REMISSION FOR 8 WEEKS • STEROID DEPENDENT: RELAPSE WITHIN 2 WK OF STEROID DISCONTINUATION DR ALPANA KONDEKAR www.peditips.com

  15. TREATMENT • GENERAL CARE • DIET: SALT AND WATER CONTROL • DIURETICS: STEROID AS DIURETIC • ALBUMIN THERAPY • STEROID THERAPY • TREATMENT OF COMPLICATIONS • TREATMENT OF DIFFICULT NS DR ALPANA KONDEKAR www.peditips.com

  16. STEROID THERAPY • ISKDC REGIMEN • GERMAN REGIMEN • NELSON REGIMEN DR ALPANA KONDEKAR www.peditips.com

  17. ISKDC REGIMEN: • CONTINUOS Rx PHASE PREDNISOLONE • 60MG/SQM/DAY 3 DIVIDED DOSES • OR 2MG/KG/DAY 3 DIVIDED DOSES • FOR 4 WEEKS • TAPERING PHASE • 40MG/SQM/DAY OR 1.5MG/KG/DAY; • SINGLE DAILY DOSE • ALTERNATE DAY • 4 WEEKS DR ALPANA KONDEKAR www.peditips.com

  18. TREATMENT OF RELAPSE • DAILY ORAL PREDNISOLONE • TILL PROTEINURIA REMISSION • THEN ALTERNATE DAY4 WEEKS • RELAPSES BECOME LESS FREQUENT AND CEASE BY PUBERTY IN STEROID RESPONSIVE NS DR ALPANA KONDEKAR www.peditips.com

  19. DIFFICULT NS • FRNS / SRNS • PROLONGED STEROID THERAPY • HIGH DOSE STEROID THERAPY • 0.5MG/KG/DAY 6-12 MONTHS STEROID TOXICITY GROWTH RETARDATION; INFECTION CATARACT; OSTEOPOROSIS; FEMORAL HEAD NECROSIS DR ALPANA KONDEKAR www.peditips.com

  20. ALTERNATIVE THERAPIES FOR DIFFICULT NS: STEPWISE FOR FRNS AND SDNS DR ALPANA KONDEKAR www.peditips.com

  21. PULSE DEXA/ MPRED THERAPY ALTERNATIVE THERAPIES FOR DIFFICULT NS: STEPWISE FOR SRNS FSGS DR ALPANA KONDEKAR www.peditips.com

  22. THANK YOU DR ALPANA KONDEKAR www.peditips.com

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