In the Indian Subcontinent
Bull. Org. mond. Sante 1966, 35, 837-856 Bull. Wld Hlth Org. Haemoglobinopathies, Glucose-6-phosphate Dehydrogenase Deficiency and Allied Problems in the Indian Subcontinent J. B. CHATTERJEA1 The present world-wide interest in haemoglobinopathies and allied disorders has given rise to a very considerable literature over the past two decades. This communication reviews this literature in sofar as it refers to the Indian subcontinent. The most common abnormality is thalassaemia, which hasbeen discovered in all regions under consideration: India, Pakistan, Nepal, Bhutanand Ceylon. Haemoglobins S, D and Eare also quite common: Hb S has been found mostly in the aboriginal tribes, Hb D in Gujaratis and Punjabis and Hb E in Bengalis, Assamese and Nepalese. A few instances ofhaemoglobins F, H, J, K, L and Mhave also been reported. However, there remain many population groups to be investigated. Studies of the distribution of glucose-6-phosphate dehydrogenase deficiency are also reviewed, and the correlation between the various haem3globin disorders and various environ- mentalfactors is discussed, but it is pointed out that the relevant data are still insufficient to allow any definite conclusions to be drawn. I. INDIA THALASSAEMIA SYNDROMES tion with various abnormal haemoglobins (S, D, E, H, J and K). In most of these cases, the investigations The first instance of thalassaemia in India was started with a patient who was suffering from a recorded by Mukherji (1938) in a Bengali boy. More certain degree of haemolytic anaemia with jaundice precise identification of the disorder was not possible and hepatosplenomegaly. The patient proved to be then and it is not known whether this boy was either homozygous for thalassaemia or a double suffering from homozygous thalassaemia or Hb E heterozygote for the thalassaemia gene and one or thalassaemia.
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