isolated diffuse mesangial sclerosis (IDMS)

Genetics

• autosomal recessive
• associated with defects in WT1

Clinical manifestations

• early-onset nephrotic syndrome in the absence of other abnormalities
• results in renal failure

Laboratory

• WT1 gene mutation

More general terms

• genetic disease of the kidney
• nephrotic syndrome

Database

• OMIM: https://mirror.omim.org/entry/256370