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GENATLAS PHENOTYPE
last update : 18-12-2013
Symbol NPHS4
Location 11p13
Name childhood nephrotic syndrome 4
Corresponding gene WT1
Main clinical features
  • onset in infancy of asymptomatic proteinuria with subsequent development of the nephrotic syndrome and progression to renal failure and death before the age of 3 years
  • renal histopathology showing infantile mesangial sclerosis
  • Genetic determination autosomal recessive
    Function/system disorder kidney and urinary tract
    Type disease
    Remark(s)