/nephrotic syndrome, type 4

nephrotic syndrome, type 4

Rare Disease

MONDO:0009733

Also Known As

WT1 nephrotic syndromecongenital nephrotic syndrome - diffuse mesangial sclerosisnephrotic syndrome caused by mutation in WT1nephrotic syndrome, type 4DMSNPHS4diffuse isolated mesangial sclerosisdiffuse mesangial sclerosisfamilial mesangial sclerosisisolated diffuse mesangial sclerosismesangial sclerosis, diffusenephrotic syndrome, early onset with diffuse mesangial sclerosis

Definition

Nephrotic syndrome within the first three motnhs of life, characterized initially by increased mesangial matrix, with or without hypertrophy and hyperplasia of podocytes, and eventual glomerular sclerosis.