RETINITIS-PIGMENTOSA IN SOUTHERN AFRICA

Retinitis pigmentosa (RP) is a heterogeneous group of inherited retina l disorders which are a common cause of genetic blindness. The relativ e frequencies of the different forms of RP in South Africa, as determi ned from the register at the DNA banking centre for RP at the Departme nt of Human Genetics, University of Cape Town, are presented and discu ssed. Of the 125 families analysed, 29 (23%) showed autosomal dominant , 33 (27%) autosomal recessive and 3 (3%) X-linked inheritance. In 10 families the pedigree data were insufficient to allow accurate genetic subtyping and a further 50 patients were sporadic without a family hi story of RP or other syndromic features which would allow categorizati on.