The primary cause of 68 enucleations in the University Hospital, Kuala Lumpur, are reviewed and compared with those from Uganda and Jerusalem. Trauma 25% especially in the 20 - 29 age group was the most important cause, followed by corneal diseases 22% seen largely over the age of 50. Malignant tumours 16% consisting nearly all of retinoblastoma and a very low incidence of malignant melanoma when compared with the Caucasians. Glaucoma 12% was mainly of the narrow angle type. Males predominate nearly all age groups with an overall ratio of 2:1 and a peak of 5:1 in trauma.
Newborns were examined for the presence of slow-moving haemoglobin components, tentatively designated X components and previously found in a group of Hb H disease in which invariably one of the parents of each patient had the same slow-moving Hb X components also. Structural studies showed that the abnormal haemoglobin in Chinese was identical with Hb Constant Spring, an c-chain variant. Newborns with Hb Bart’s and slow-moving X components invariably had one parent with the X components also. When the child grew older Hb Bart’s disappeared while the Hb X components remained in the blood. The homozygous state for the X components was found in a Malay boy through his newborn brother who had the X components in addition to Hb Bart’s and had both parents with the X components. One other Malay baby had the X components and Hb A2 Indonesia inherited from the parents. The present study of newborns also showed that Hb Bart’s can accompany different abnormalities of haemoglobin production, involving alpha-chains, beta-chains as well as gamm-chains. Its presence in cord blood is, therefore, not specific for alpha-thalassaemia
Key Words: Haemoglobinopathies; Hb Bart’s; Slow-moving Hb X; Thalassaemia