Patofisiologi Dan Manifestasi Klinis SN

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PATOFISIOLOGI DANMANIFESTASI KLINISSINDROMA NEFROTIKEtiologiSindrom nefrotik (SN) pada anak merupakan penyakit ginjal anak yang paling sering ditemukan.Di Indonesia dilaporkan ada 6:100.000 pertahun anak filamen tidak terbentuk -> proteinuria masif

2. SINDROMA NEFROTIK PRIMERSoluble Antigen Antibody ComplexAntigen masuk sirkulasi -> terdapat Complemen C3 -> reaksi antara antigen dengan Complemen C3 larut dalam darah -> deposit yg terperangkap dibawa epitel capsula bowman -> granuler/noduler di sepanjang membran basalis glomerulus -> permeabilitas terganggu Perubahan elektrokemishilangnya fixed negatif ion di lapisan membrana protein glomerulus -> permeabilitas naik -> albumin keluar bersama urin 3. SINDROMA NEFROTIK SEKUNDERPenyakit lain (ex: malaria ) akan menyebabkan supresi dari sel T yang membuat abnormalnya fungsi sel T -> sitokin toksis terhadap mbg -> inflamasi pada dinding glomerulus -> permeabilitas naik -> kebocoran ( size selectivity )-> katabolisme proteoglikan heparan sulfat pd dinding kapiler glomerulus -> muatan (-) hilang -> protein yg bermuatan (-) bocor ( charge selectivity )

DEFINITIONNEPHROTIC SYNDROME is defined as- heavy proteinuria- hypoproteinaemia- oedema- hyperlipidaemiaHeavy proteinuria : > 50 mg/kg BW/day or >40mg/m2/hcreatinine/protein ratio > 2 mg/mgselective proteinuria

Hypoalbuminaemia : < 2.5 g/dl

Hyperlipidaemia :variable degreePROTEINURIA- Transferine - Glob.Thyroxin - Glob. Vit. D - Coagulation factors F VII, IX, XII IgG IgE IgA IgM Fibrinogen HYPOALBUMINAEMIAB-lipoprot hyperlipidaemiaONCOTIC PRESSURE OEDEMAHYPOVOLAEMIALipiduriaAldosteron Na and H2O retention Hb Packed cell vol Viscocity Vein thrombosis Peripheral circulation collaps Death

Renal perfusion renin plasma Ureum + K dr.Bayu Kusuma WardanaMANIFESTASI KLINISSINDROMA NEFROTIKCLINICAL MANIFESTATIONSOedema (uptill 40% BW), is the common symptomLocal edema: edema in face,around eyes( Periorbital swelling), in lower extremities. Generalized edema (anasarca), edema in penis and scrotum.

Secondary infections : skin, peritonitisAnaemiaGrowth disturbancesTetany (hypocalcaemia)Hypovolemic shockVein thrombosis

Nonspesifik :Fatigue and lethargy,loss of appetite, nausea and vomiting,abdominal pain , diarrhea, body weight increase,urine output decrease, pleural effusion(respiratorydistress)

Nephrotic syndrome

Generelised edema(anasarca)

Older child with nephrotic syndromePitting peripheraloedema

Nephrotic SyndromeAscites

Nephrotic syndrome

SCROTAL EDEMALABIAL EDEMA