TY - JOUR AU - AT Solima, MM ElZalabany, Y Mazloum, SMA Bedair, MS Ragab, AD Rogol, BM Ansari AB - Growth retardation in children with thalassaemia major is multifactorial. We studied the growth hormone (GH) response to provocation by clonidine and glucagon, measured the circulating concentrations of insulin, insulin-like growth factor-I (IGF-I), IFG-binding protein-3 (IGFBP3), and ferritin, and evaluated the spontaneous nocturnal (12 h) GH secretion in prepubertal patients with thalassaemia and age-matched children with constitutional short stature (CSS) (height SDS < -2, but normal GH response to provocation). The anatomy of the hypothalamic pituitary area was studied in patients with abnormal GH secretion using MRI scanning. Children with thalassaemia had significantly lower peak GH response to provocation by clonidine and glucagon (8.8 ± 2.3 μg/l and 8.2 ± 3.1 μg/l respectively) than did controls (17.6 ± 2.7 μg/l and 15.7 ± 3.7 μg/l respectively). They had significantly decreased circulating concentrations of IGF-I and IGFBP3 (68.5 ± 19 ng/ml and 1.22 ± 0.27 mg/l respectively) compared to controls (153 ± 42 ng/ml and 2.16 ± 0.37 mg/l respectively). Seven of the thalassaemic children had a GH peak response of < 7 μg/l after provocation. Those with a normal GH response after provocation also had significantly lower IGF-I and IGFBP3 concentrations than controls. Analysis of their spontaneous nocturnal GH secretion revealed lower mean (2.9 ± 1.77 μg/l) and integrated (2.53 ± 1.6 μg/l) concentrations compared to controls (4.9 ± 0.29 μg/l and 5.6 ± 0.52 μg/l respectively). Five of them had mean nocturnal GH concentration < 2 μg/l and four had maximum nocturnal peak below 10 μg/l. These data denoted defective spontaneous GH secretion in some of these patients. MRI studies revealed complete empty sella ( n =2), marked diminution of the pituitary size ( n =4), thinning of the pituitary stalk ( n =3) with its posterior displacement ( n =2), and evidence of iron deposition in the pituitary gland and midbrain ( n =7) in those patients with defective GH secretion ( n =9). Serum ferritin concentration was correlated significantly with the circulating IGF-I ( r =-0.47, p <0.01) and IGFBP3 ( r =-0.43), p <0.01) concentrations. These data prove a high prevalence of defective GH secretion in thalassaemic children associated with structural abnormality of their pituitary gland. Copyright 1999 « Previous | Next Article » Table of Contents This Article J Trop Pediatr (1999) 45 (6): 327-337. doi: 10.1093/tropej/45.6.327 » Abstract Free Full Text (PDF) Free Classifications Article Services Article metrics Alert me when cited Alert me if corrected Alert me if commented Find similar articles Similar articles in Web of Science Similar articles in PubMed Add to my archive Download citation Request Permissions Responses Submit a response No responses published Citing Articles Load citing article information Citing articles via CrossRef Citing articles via Scopus Citing articles via Web of Science Citing articles via Google Scholar Google Scholar Articles by Solima, A. Articles by Ansari, B. Search for related content PubMed PubMed citation Articles by Solima, A. Articles by ElZalabany, M. Articles by Mazloum, Y. Articles by Bedair, S. Articles by Ragab, M. Articles by Rogol, A. Articles by Ansari, B. 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