Neurosecretory Dysfunction of Growth Hormone Secretion in Thalassemia Major |
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Authors: | NAIM SHEHADEH ANNA HAZANI MARY C. J. RUDOLF ISRAELA PELEG ABRAHAM BENDERLY ZEEV HOCHBERG |
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Affiliation: | Departments of Pediatrics, Hematology and Endocrinology, Ramban Medical Center, and Department of Pharmacology, Faculty of Medicine and Rappaport Family Institute for Research in the Medical Sciences, Technion-Israel Institute of Technology, Haifa, Israel |
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Abstract: | ABSTRACT. The growth retardation of children with thalassemia major is multifactorial. Along the endocrine axis of growth hormone (GH), serum somatomedin has been shown to be deficient and GH response to GH-relasing hormone impaired, while GH response to provocative stimuli is normal. We studied the spontaneous secretion of GH in seven patients with thalassemia major and growth retardation. Three of the patients were hypothyroid, and the other four were euthyroid. Spontaneous secretion of GH in all seven patients was subnormal: the number of pulses, the mean pulse amplitude, and the integrated concentration of GH were all lower than in 14 age- and sex-matched (10 pubertal and 4 prepubertal) control subjects. GH response to provocative stimuli was normal in the euthyroid patients. This pattern of response corresponds with the definition of neurosecretory dysfunction of GH secretion. It is concluded that the growth retardation of patients with thalassemia major is partly due to neurosecretory dysfunction of GH secretion. |
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Keywords: | thalassemia growth hormone growth |
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