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

Abstract

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.

• Authors: J S Schuman, K V Lieberman, A H Friedman, M Berger, M J Schoeneman
• Journal: American journal of ophthalmology (Am J Ophthalmol) Vol. 100 Issue 6 Pg. 822-7 (Dec 15 1985) ISSN: 0002-9394 [Print] United States
• PMID: 4073180 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Topics

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