Senior-Loken syndrome (familial renal-retinal dystrophy) and Coats' disease

Am J Ophthalmol. 1985 Dec 15;100(6):822-7. doi: 10.1016/s0002-9394(14)73374-4.


Senior-Loken syndrome is a rare hereditary disease that combines a disorder resembling familial juvenile nephronophthisis with retinitis pigmentosa. Retinitis pigmentosa is even less frequently associated with exudative retinopathy. The patient, a 15-year-old boy, had hereditary renal-retinal dystrophy combined with an exudative vasculopathy of the Coats' type. The patient is on thrice-weekly hemodialysis after two kidney transplants failed. One eye became painful and blind and was eventually enucleated.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Humans
  • Kidney Diseases / complications
  • Kidney Diseases / genetics*
  • Kidney Diseases / pathology
  • Male
  • Microscopy, Electron
  • Retina / pathology
  • Retinal Degeneration / complications
  • Retinal Degeneration / genetics*
  • Retinal Degeneration / pathology
  • Retinal Diseases / complications*
  • Retinal Diseases / pathology
  • Retinal Vessels / ultrastructure
  • Syndrome