Nephrotic Syndrome Department of Prdiatrics, Tongji Hospital Tel:83662684
Nephrotic Syndrome Department of Prdiatrics, Tongji Hospital Tel: 83662684
Nephrotic Syndrome Definition Etiology Pathology Pathophysiology Clinical manifestation Laboratory Data Diagnosis Therapy and prognosis
Nephrotic Syndrome • Definition • Etiology • Pathology • Pathophysiology • Clinical Manifestation • Laboratory Data • Diagnosis • Therapy and Prognosis
lale 4 years and 6 months old Complaint of edema and oliguria
Male 4 years and 6 months old Complaint of edema and oliguria
Definition: Nephrotic Criteria Massive proteinuria ISKDC:>40mg/m2/hr CAN: >++ trice/2w or >50-100mg/kg/24hr Mendoza: Urine Protein/Cr22.0 Hypoalbuminemia: 30g/L Hyperlipidemia: Cholesterol> 5.72mmol/L o Edema
Definition: Nephrotic Criteria • Massive proteinuria • ISKDC: > 40mg/m2 /hr • CAN: >+++ trice/2w or >50-100mg/kg/24hr • Mendoza: Urine Protein/Cr ≥2.0 • Hypoalbuminemia: 5.72mmol/L • Edema
Definition: Nephritic Criteria Hematuria: RBC+(>10/HP), trice/2w Hypertension: >130/90 mmHg in children over 7y >120/80 mm Hg in 3-6y children >110/70 mm Hg in 3y children Azotemia: bun>6. 4mmolL cr>133umolL Hypocomplementemia: C3<0.8/L
Definition: Nephritic Criteria • Hematuria: RBC ++( >10 /HP), trice/2w • Hypertension: • >130/90 mmHg in children over 7y • > 120/80 mm Hg in 3-6y children • > 110/70 mm Hg in 6.4mmol/L, Cr >133umol/L • Hypocomplementemia: C3<0.8/L
Definition Nephritic Nephrosis nephrotic criteria with at least one nephritic criteria Simple nephrosis nephrotic criteria without nephritic criteria
Definition • Nephritic Nephrosis: nephrotic criteria with at least one nephritic criteria • Simple nephrosis: nephrotic criteria without nephritic criteria
Etiology Congenital Ns: rare Idiopathic Ns: majority Secondary ns: many causes especially in y or >13y patients causes-DIAMOND
Etiology • Congenital NS: rare • Idiopathic NS: majority • Secondary NS: many causes especially in 13y patients causes―DIAMOND
Secondary ns: DIAMOND e Infection: APSGN, HBV, HIV, shunt nephropathy, reflux nephropathy, leprosy, syphilis, schistosomiasis, hydatid disease Drug,Toxic, Allegy mercury, snake venom, vaccine pellicillamine, Heroin, gold, NSAID, captopril, probenecid, volatile hydrocarbons Neoplasma: Hodgkin' s disease, carcinoma renal cell, lung neuroblastoma, breast, and etc) Autoimmune or collagen-vascular diseases SLE. Hashimoto's thyroiditis EMc. HSP Vasculitis Genetic Disease Alport syn. Fabry syn, Nail-patella syn Sickle cell disease, Amyloidosis, Congenital nephropathy Metabolic disease. Diabetes mellitus Others Chronic transplant rejection. congenital nephroscler
Secondary NS : DIAMOND • Infection: APSGN, HBV, HIV,shunt nephropathy, reflux nephropathy, leprosy, syphilis,schistosomiasis, hydatid disease • Drug,Toxic,Allegy: mercury, snake venom, vaccine, pellicillamine, Heroin,gold, NSAID, captopril, probenecid, volatile hydrocarbons • Neoplasma: Hodgkin’s disease, carcinoma ( renal cell, lung, neuroblastoma, breast, and etc) • Autoimmune or collagen-vascular diseases: SLE, Hashimoto’sthyroiditis, EMC, HSP, Vasculitis • Genetic Disease: Alport syn., Fabry syn., Nail-patella syn., Sickle cell disease, Amyloidosis, Congenital nephropathy • Metabolic disease: Diabetes mellitus • Others: Chronic transplantrejection, congenital nephrosclerosis
Pathology Primary NS: Minimal change Nephropathy (MCN):80% Mesangial proliferative glomerulonephritis MsPGn: 10-129 Focal segmental glomerulosclerosis(FSGS): 5% Others. Membranous Nephropathy(Mn)most common in adults Membrane proliferative glomerulonephritis MPGN): 2-5% Cresent glomerulonephritis: rare, Crescent glomeruli>50% Secondary Ns: dependent on causes, APSGN, MN FSGS MsPgN or MPgn
Pathology • Primary NS: – Minimal Change Nephropathy (MCN): 80% – Mesangial proliferative glomerulonephritis (MsPGN): 10-12% – Focal segmental glomerulosclerosis (FSGS): 5% – Others: Membranous Nephropathy (MN) ―most common in adults Membrane proliferative glomerulonephritis (MPGN): 2-5% Cresent glomerulonephritis: rare, Crescent glomeruli > 50% • Secondary NS: dependent on causes, APSGN, MN, FSGS, MsPGN or MPGN
Minimal Change Nephropathy ( MCN Little or no microscopic abnormality Absence of immune complexes Effacement of epithelial foot process Occasionally mesangial hypercellurity
Minimal Change Nephropathy (MCN ) • Little or no microscopic abnormality • Absence of immune complexes • Effacement of epithelial foot process • Occasionally mesangial hypercellurity