variants

Last reviewed 01/2018

Four variants are described:

  • sporadic - 20%
  • familial juvenile nephronophthisis - autosomal recessive inheritance - 50%
  • renal-retinal dysplasia - autosomal recessive inheritance, associated with retinitis pigmentosa - 15%
  • adult-onset medullary cystic disease - autosomal dominant inheritance - 15%