NEPHROTIC SYNDROME BY Dr ALPANA SOMALE DR ALPANA
NEPHROTIC SYNDROME BY : Dr ALPANA SOMALE DR ALPANA KONDEKAR www. peditips. com
COMMONEST TYPE OF RENAL LESION IN CHILDREN 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 2
TWO MAIN TYPES NEPHROTIC SYNDROME • PRIMARY (IDIOPATHIC) 90% ü MCNS 75% ü FSGS 10% ü Mn <5% ü MPGN 10 -15% 9/15/2020 • SECONDARY TO (10%) ü INFECTIONS ü MALIGNANCY ü DRUGS ü CONNECTIVE TISSUE DISEASES DR ALPANA KONDEKAR www. peditips. com 3
SECONDARY NS MALIGNANCY LUNG Ca GI TUMORS HODGKINS INFECTIONS TB MALARIA HEP VIRUSES FILARIA LEPROSY HIV 9/15/2020 COLLAGEN VASCULAR SLE HSP AND DRUG INDUCED NS DR ALPANA KONDEKAR www. peditips. com 4
DRUG INDUCED NS MPGN PROCAINAMIDE CHORPROPAMIDE PHENYTOIN TRIMETHADIONE PARAMETHADIONE 9/15/2020 MEMBRANOUS PENICILLAMINE CAPTOPRILL GOLD MERCURY NSAIDS DR ALPANA KONDEKAR www. peditips. com MCNS PROBENESIDS ETHOSUXIMIDE METHIMAZOLE LITHIUM 5
NEPHROTIC SYNDROME HEAVY PROTEINURIA HYPOALBUMINEMIA EDEMA HYPERLIPIDEMIA 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 6
CHARACTERISTICS • ü ü ü ü PRIMARY 1 -8 YR NO HT HEMATURIA 20% N RENAL FUNCN NO EXTRARENAL NORMAL C 3 STEROID RESPONSE 9/15/2020 • ü ü ü ü SECONDARY >8 YR HT HEMATURIA RENAL DYSFUNCN RASH/ARTHRLGIA LOW C 3 STEROID RESISTANT DR ALPANA KONDEKAR www. peditips. com 7
CONGENITAL NS • FINNISH TYPE SCANDINAVIAN DESCENT 1: 8000 A-RECESSIVE NPHN 1 Gene mutn(CR 19) PATHO: DILTN PROX TUBULES + MESANGIAL HYPERCELLULARITY + GLOMERULAR SCLEROSIS+ AETIO: GENETIC: SYPH; TORCH; HIV/HBV AT BIRTH / < 3 Mo 9/15/2020 • DENYS DRASH SYNDROME CR 11; WILMS TUMOR GENE MUTN ü^AFP IN AMNIOTIC FLUID üLARGE PLACENTA üPREMATURITY üMARKED EDEMA üMASSIVE PROTEINURIA üRESP DISTRESS üCR SUTURE SEPARATION üRECURRENT INFECTION üPERSISTANT EDEMA üPROGRESSIVE R FAILURE DR ALPANA KONDEKARüDEATH BY 5 YR 8 www. peditips. com
TREATMENT CONG NS • • ACE INHIBITORS INDOMETHACIN UNILAT NEPHRECTOMY B/L NEPHRECTOMY CHRONIC DIALYSIS AGGRESSIVE NUTRITIONALSUP RENAL TRANSPLANT 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 9
T CELL DYSFUNCTION OGENESIS ↓↓ alteration of cytokines IL 2/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 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 10
COMPLICATIONS NS • INFECTIONS – STREPT PNEUMO, TB, E COLI, H INFLU, VARICELLA – SKIN, GI, PERITONEUM, SEPSIS; UTI, RS • THROMBOEMBOLIC • • – DUE TO LOSS OF AT 3, 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 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 11
INVESTIGATIONS NS • PROTEINURIA • • 40 MG/M 2/HR 1 GM/M 2/DAY 50 MG/KG/DAY 4+ MASSIVE ALBUMINURIA • TG/CHOLESTEROL >220 • SERUM ALB <2. 5 • SERUM PROTEIN EPP − low albumin, increased alpha-2 (macroglobulin), reduced gamma • NORMAL C 3 • URINE ALB/CR >3; Sr Ca LOW, 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 12
INDICATIONS FOR KIDNEY BIOPSY ü ATYPICAL NS; AGE <2 Y & >9 Y ü PERSISTANT GROSS HEMATURIA ü PERSISTANT HT / HYPOCOMPLEMENTEMIA ü PERSISTANT AZOTEMIA ü CONGENITAL NS ü SRNS ü FREQUENT RELAPSER ü SLE / FAMILY HISTORY NS 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 13
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 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 14
TREATMENT ü GENERAL CARE üDIET: SALT AND WATER CONTROL üDIURETICS: STEROID AS DIURETIC üALBUMIN THERAPY ü STEROID THERAPY ü TREATMENT OF COMPLICATIONS ü TREATMENT OF DIFFICULT NS 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 15
STEROID THERAPY • ISKDC REGIMEN • GERMAN REGIMEN • NELSON REGIMEN 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 16
ISKDC REGIMEN: • CONTINUOS Rx PHASE PREDNISOLONE − 60 MG/SQM/DAY 3 DIVIDED DOSES − OR 2 MG/KG/DAY 3 DIVIDED DOSES − FOR 4 WEEKS • TAPERING PHASE − − 9/15/2020 40 MG/SQM/DAY OR 1. 5 MG/KG/DAY; SINGLE DAILY DOSE ALTERNATE DAY 4 WEEKS DR ALPANA KONDEKAR www. peditips. com 17
TREATMENT OF RELAPSE ü DAILY ORAL PREDNISOLONE ü TILL PROTEINURIA REMISSION ü THEN ALTERNATE DAY 4 WEEKS • RELAPSES BECOME LESS FREQUENT AND CEASE BY PUBERTY IN STEROID RESPONSIVE NS 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 18
DIFFICULT NS • FRNS / SRNS ü PROLONGED STEROID THERAPY ü HIGH DOSE STEROID THERAPY ü 0. 5 MG/KG/DAY 6 -12 MONTHS STEROID TOXICITY GROWTH RETARDATION; INFECTION CATARACT; OSTEOPOROSIS; FEMORAL HEAD NECROSIS 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 19
ALTERNATIVE THERAPIES FOR DIFFICULT NS: STEPWISE FOR FRNS AND SDNS CYCLOPHOSPHAMIDE 2. 5 MG/KG/DAY; 8 -12 WKS MONITOR: CBC, URINE, SPERM CT üREVERSIBLE ALOPECIA üHEMORHEGIC CYSTITIS üBM SUPPRESSION üINFECTIONS üALSO SEE=> üGONADAL TOXICITY üSTERILITY üMALIGNANCY STEP ONE CHLORAMBUCIL 0. 15 -0. 2 MG/KG/DAY 8 -12 WKS SAME AS ABOVE (SAA) FOCAL SEIZURES MONITOR: SAA EEG STEP TWO CYCLOSPORIN 4 -5 MG/KG/DAY 6 -24 MONTHS NEPHROTOX, INFECTIONS MONITOR: HT, HIRSUITISM, GINGIVAL TROUGH HYPERPLASIA, HYPER K+ LEVELS OF DRUG IN SR; Cr STEP THREE LEVAMISOLE 2. 5 -3 MG/KG/DAY ALT DAY, 6 -24 MONTHS SKIN RASH, ABDO PAIN, VOMITTING, NEUTROPENIA 9/15/2020 ENALAPRIL DR ALPANA KONDEKAR www. peditips. com MONITOR: CBC STEP FOUR STEP FIVE 20
ALTERNATIVE THERAPIES FOR DIFFICULT NS: STEPWISE FOR SRNS FSGS • PULSE DEXA/ MPRED THERAPY WEEK DOSE OF IV MP ORAL PREDNISOLONE 1 -2 WEEK 30 MG/KG ALT DAY - 3 -10 WEEK 30 MG/KG/WEEK 2 MG/KG/ALT DAY 11 -18 WEEK 30 MG/KG/ALT WEEK + TAPER ORAL 19 -52 WEEK 30 MG/KG/MONTH SLOW TAPER 53 -78 WEEK 30 MG/KG/ALTMONTH - 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 21
THANK YOU 9/15/2020 DR ALPANA KONDEKAR www. peditips. com 22
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