Faculty

Anju Seth

Multitransfused Thalassemia

Endocrinopathies in multi-transfused thalassemia.

Beginning second decade of life, multi-transfused patients with thalassemia are vulnerable to develop multiple endocrinopathies. The onset and severity of endocrine involvement is primarily dependent on the iron overload. While all endocrine glands are susceptible, the commonest endocrine issues seen in these patients include short stature and pubertal delay/failure, followed by disturbances of glucose metabolism, Thus, management of a child with thalassemia requires regular growth monitoring, assessment for pubertal development and annual endocrine screening after 9 years of age. Early identification and prompt management of the endocrine complications reduces morbidity and improves quality of life in patients with thalassemia. The annual endocrine screening includes assessment of free
T4 and TSH, fasting glucose, oral glucose tolerance test (in case of impaired fasting glucose), serum calcium, phosphate and ALP, vitamin D and PTH (in suspected hypoparathyroidism), and bone age. The LH, FSH, and sex steroids are evaluated in the adolescents with delayed/arrested puberty. A baseline DXA scan followed by annual/once in 2 years screening of bone mineral density is also recommended. Other endocrine testing is undertaken on clinical suspicion.

Evidence indicates institution of intensive chelation may retard progression/ reverse early endocrine involvement. However, prevention continues to be the best approach. Thus, preventing anemia through a regular transfusion schedule, optimum chelation to keep serum ferritin level under control, maintaining an adequate nutritional status and regular physical exercise would help prevent/delay endocrine involvement.

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