Oculocutaneous albinism among schoolchildren in Harare, Zimbabwe.

The term oculocutaneous albinism (OCA) covers a range of autosomal recessive genetic conditions involving hypopigmentation of the skin, hair, and eyes. In southern African black ethnic groups the tyrosinase positive form, OCA2, is predominant, with few cases of tyrosinase negative OCA1. The prevalence of OCA2 in schoolchildren in Harare, the capital city of Zimbabwe, was found to be 1 in 2833. The gene frequency for OCA2 was 0.0188, with a carrier frequency of 1 in 27. Most of the pupils with albinism belong to the majority Shona ethnic group. As consanguineous marriages are discouraged in the Shona culture this high rate is likely to be a result of genetic drift in a relatively small population showing limited mobility. OCA pupils were found in more than a third of the secondary schools in Harare, emphasising the importance of distributing information on albinism and its management widely throughout the school system.