Papers And Originals

Sickle-cell Anaemia, Sickle-cell Thalassaemia, Sickle-cell Haemoglobin C Disease, and Asymptomatic Haemoglobin C Thalassaemia in one Ghanaian Family

Br Med J 1969; 1 doi: (Published 08 March 1969) Cite this as: Br Med J 1969;1:607
  1. F. I. D. Konotey-Ahulu,
  2. Bela Ringelhann


    A Ghanaian family is described in which a sickle-cell haemoglobin C man married to a sickle-cell thalassaemia woman produced 12 children (eight alive). Four children have sickle-cell anaemia, two sickle-cell haemoglobin C disease, one has sickle-cell thalassaemia, and one is asymptomatic haemoglobin C thalassaemia.

    It is emphasized that the contribution that adult sickle-cell disease patients make, through procreation, to the persistence of the S gene may be greater than is normally supposed, and that this contribution may soon outstrip that made by balanced polymorphism through falciparum malaria. Widespread haemoglobin genotyping in schools leading to genetic counselling is advocated to decrease the incidence of sickle-cell disease.