Document Detail


Reduced insulin growth factor I concentrations in iron-overloaded beta thalassaemic patients with normal growth hormone secretion and liver function.
MedLine Citation:
PMID:  16462706     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We selected 92 subjects (46 females and 46 males), aged 10-15 years, from the Haematology and Endocrine Clinic of Shiraz University, Iran. Forty-six were beta thalassaemia patients (beta-Th) with short stature, 23 had idiopathic short stature (ISS) and 23 were healthy children with a standing height between the 10th and 95th percentile. Growth hormone (GH) secretion was normal in 23 beta-Th patients and reduced in the remaining 23 patients. A low insulin growth factor I (IGF-I) was found in 73.9% of beta-Th patients with GH deficiency, 56.5% of beta-Th patients with normal GH secretion to stimulation test and 8.7% of children with ISS. The reduced IGF-I concentration in beta-Th patients with normal GH secretion may be explained by partial insensitivity to GH (GHIS), neurosecretory dysfunction, low bioactive GH or increased proportion of circulating, non-22-kDa GH isoform. The possibility of GHIS in beta-Th patients with short stature indicates that higher doses of rechGH may be required to obtain an improvement in growth velocity in beta-Th patients.
Authors:
Hamdollah Karamifar; Mehran Karimi; Gholamhossein Amirhakimi; Mohammadreza Sharbatialaei; Vincenzo De Sanctis
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric endocrinology reviews : PER     Volume:  2 Suppl 2     ISSN:  1565-4753     ISO Abbreviation:  Pediatr Endocrinol Rev     Publication Date:  2004 Dec 
Date Detail:
Created Date:  2006-02-07     Completed Date:  2006-03-09     Revised Date:  2008-04-09    
Medline Journal Info:
Nlm Unique ID:  101202124     Medline TA:  Pediatr Endocrinol Rev     Country:  Israel    
Other Details:
Languages:  eng     Pagination:  256-8     Citation Subset:  IM    
Affiliation:
Endocrine Department, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran. karimim@sums.ac.ir
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Alanine Transaminase / blood
Body Height
Child
Child, Preschool
Deferoxamine / pharmacology
Female
Ferritins / blood
Human Growth Hormone / metabolism*,  secretion
Humans
Infant
Insulin-Like Growth Factor I / metabolism*
Iron Overload / enzymology,  metabolism*
Liver / enzymology,  metabolism
Male
beta-Thalassemia / blood,  enzymology,  metabolism*
Chemical
Reg. No./Substance:
12629-01-5/Human Growth Hormone; 67763-96-6/Insulin-Like Growth Factor I; 70-51-9/Deferoxamine; 9007-73-2/Ferritins; EC 2.6.1.2/Alanine Transaminase

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