Contributor: Gordon K. Klintworth
Juvenile nephronophthisis is a heterogeneous group of inherited disorders of the kidney with an autosomal recessive mode of inheritance. It is a cystic kidney disease [cyst -kidney] and is apparently the most frequent cause of renak failure due to a defect in a single gene. Several types of  juvenile nephronophthisis are recognized: nephronophthisis - juvenile type 1, nephronophthisis - juvenile type 2, nephronophthisis - juvenile type 3,  and nephronophthisis - juvenile type 4.