Endocrine disorders in beta thalassemia major in Indian children
Sachdeva, Anupam; Khanna, Virendra Kumar; Subhash, Chander Arya; Dayal, Archana Araya and Yaddanapudi, Ravindranath (2002) Endocrine disorders in beta thalassemia major in Indian children. In: The 44th American Society of Hematology Annual Meeting, 2002, Detroit, USA.
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The association of endocrine disorders with Thalassemia was evaluated in a large cohort of Indian children with Beta Thalassemia Major and compared to available data on children from developed countries, employing measurements of standing and sitting height, onset of puberty and of development of Diabetes Mellitus and, or hypoparathyroidism. Age at onset of blood transfusions, average pretransfusion Hemoglobin levels, serum Ferritin, ALT, Random blood sugar, serum calcium, phosphorus and alkaline phosphatase were measured in all subjects and Parathormone levels were measured in those with suspected hypoparathyroidism. 33% of the study population were found to have short stature(SS), compared with 18% reported from developed countries. The prevalence of SS increased with advancing age. Most of the subjects with SS were over 10 years of age. 90% of the subjects in our study exhibited short trunks (ST). None of the children under 5 years of age were found to have SS, although 80% exhibited ST. These figures are comparable to the data reported from developed countries. Since iron overload is rare in this age group, it seems that ST is unique to Beta Thalassemia major and presents early in life. 36% of our study population demonstrated shortened subischial leg length (SLL) although it has been reported to be normal in most patients from developed countries. Almost half of the study population manifested delayed puberty and all such subjects had serum Ferritin levels ranging from 3000mg/l or more. Finally about 5-6% of the study population manifested Diabetes Mellitus or hypoparathyroidism. Based on these observations it is proposed that Indian children with Beta Thalassemia Major have a higher prevalence of development of short stature and hypoparathyroidism. These effects may be related to suboptimal iron chelation and other differences in management of Beta Thalassemia Major in the Indian subcontinent.
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