genitourinary disorders
Congenital Nephrotic Syndromes
Finnish type nephrotic syndrome
This syndrome is an autosomal recessive disorder that affects 1/8200 Finnish neonates and is caused by a mutation in theNPHS1gene, which codes for a podocytic slit-diaphragm protein (nephrin). Finnish-type nephrotic syndrome is rapidly progressive and usually necessitates dialysis within 1 year. Most patients die within 1 year, but a few have been supported nutritionally until renal failure occurs and then managed with Overview of Renal Replacement Therapy or 