Haemoglobin synthesis in thalassaemia

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Complete suppression of haemoglobin A synthesis in haemoglobin D Los Angeles--beta thalassaemia.

A family study is reported in which all three siblings were shown to be doubly heterozygous for haemoglobin D Los Angeles and beta thalassaemia, which resulted in a complete suppression of haemoglobin A synthesis. This demonstrates the effects of genetic interaction which occur when the genes for haemoglobin D Los Angeles and beta thalassaemia are both transmitted to the offspring. The importan...

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Haemoglobin E-beta Thalassaemia in Singapore.

Medical History Twenty patients (36%) had a splenectomy. Fourteen (25%) patients had developed gallstones and 7 had a cholecystectomy performed. Other medical complications of disease and iron overload are summarised in Table 1. In addition, 21 out of the 55 patients had a MRI T2* of the heart and liver performed. None had iron loading in the heart. Twenty (98%) patients had evidence of iron lo...

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Studies in haemoglobin E beta-thalassaemia.

Haemoglobin E beta-thalassaemia is the commonest form of severe thalassaemia in many Asian countries, but little is known about its natural history, the reasons for its clinical diversity, or its optimal management. Despite its frequency, haemoglobin E beta-thalassaemia is often managed in an ill-defined and haphazard way, usually by demand transfusion. We studied a cohort of Sri Lankan patient...

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ژورنال

عنوان ژورنال: Biochemical Journal

سال: 1970

ISSN: 0306-3283

DOI: 10.1042/bj1190068pb