Haemoglobin 0 Disease in a Sudanese Family
نویسنده
چکیده
Besides sickle-cell anaemia, the finding of Hb S with other /3-chain variants of adult haemoglobin was first demonstrated by Itano and Neel (1950) in the well-known discovery of sicklecell Hb C disease. Since then Hb S has been observed with Hb D, E, G, J. and K (Itano, 1951; Aksoy and Lehmann, 1957; Schwartz and Spaet, 1955 ; Went and MacIver, 1959; O'Gorman et al., 1963). In such circumstances there is a sickle-cell disease which, while of varying severity, is milder than the homozygous sickle-cell anaemia. Hb S was first observed with Hb 0 in two children of an Arab family who were reported to be suffering from a sicklecell Hb 0 trait condition. In those cases some Hb A was also found in addition to Hb S and 0 (Ramot et al., 1960), and it was subsequently concluded that there must have been a previous blood transfusion (Baglioni and Lehmann, 1962). The clinical picture of sickle-cell Hb 0 disease has not yet been reported. The present report describes two cases which occurred in a Sudanese family.
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