Relationship of endocrinopathy to iron chelation status in young patients with thalassaemia major.
نویسندگان
چکیده
منابع مشابه
Relationship of endocrinopathy to iron chelation status in young patients with thalassaemia major.
Disturbances of growth and development in patients with thalassaemia receiving hypertransfusion programmes are well recognised and are most likely to be due to iron overload. The extent of endocrine dysfunction was investigated in a group of 18 patients thought to have been treated by acceptable modern standards, 11 of whom could be considered as well chelated. Assessment of growth and puberty ...
متن کاملEndocrinopathy in thalassaemia major.
Pituitary, adrenal, and pancreatic functions were investigated in 9 patients with thalassaemia major. 9 a.m. plasma ACTH values were 148-480 pg/ml (normal range 15-70 pg/ml). Cortisol and growth hormone response to insulin-induced hypoglycaemia was normal in all. 24-hour urinary excretions of 17-ketosteroids and 17-hydroxycorticosteroids were normal. There was normal cortisol response to intram...
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Thalassaemics in Malaysia are poorly chelated because desferrioxamine is too expensive and cumbersome for long term compliance. The efficacy and tolerability of the oral chelator deferiprone, and the effects of using a combination therapy in our patients were studied. Ten patients completed the study and the mean serum ferritin reduced from 7066.11 ug/L (2577-12,896 ug/L) to 3242.24 ug/L (955-6...
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To the Editor: Improvements in managing b-thalassaemia major have allowed many patients to survive beyond puberty. Fertility can, however, be impaired as a result of iron overload-related hypogonadism (1), although assisted reproductive techniques and advances in treating iron overload have increased the number of successful pregnancies in such patients (2–9). During pregnancy, patients may req...
متن کاملEarly iron overload in beta-thalassaemia major: when to start chelation therapy?
Twenty-eight children with beta-thalassaemia major aged between 11 and 48 months were given intensive transfusions. Serum iron, transferrin saturation, serum ferritin, non-transferrin iron, and subcutaneous desferrioxamine-induced urinary iron excretion were measured. The results showed that even children with a limited number of transfusions had severe iron overload as indicated, in particular...
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ژورنال
عنوان ژورنال: Archives of Disease in Childhood
سال: 1994
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.71.2.128