Home Page
References OMIM Gene GeneReviews HGMD HGNC
last update : 10-11-2014
Symbol NPHS7
Location 17q22
Name nephrotic syndrome, type 7
Corresponding gene DGKE
Main clinical features
  • onset of nephrotic syndrome with proteinuria usually in the first decade of life; the disorder is progressive, and some patients develop end-stage renal disease within several years
  • renal biopsy typically shows membranoproliferative glomerulonephritis
  • Genetic determination autosomal recessive
    Related entries including atypical hemolytic uremic syndrome-7 (AHUS7), characterized by acute onset in the first year of life of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure
    Function/system disorder kidney and urinary tract
    Type disease