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Pediatr Endocrinol Rev ; 8 Suppl 2: 284-9, 2011 Mar.
Article in English | MEDLINE | ID: mdl-21705979

ABSTRACT

Endocrine complications in Β-thalassemia represent a prominent cause of morbidity. Above all, dysfunction of GH-IGF-1 axis is of a major concern because of its pathogenic role on cardiac and bone disease, frequently described in this clinical setting. The aim of this paper is to analyze GH-IGF-1 axis in a cohort of 25 adult patients affected by Β-thalassemia. We found that GH deficiency was present in only 8% of our patients if diagnosis was based on GH peak below 9µg/L to two GH provocative tests instead of only one, and was mainly related to iron overload. On the contrary, IGF-1 production was impaired in a higher percentage of patients (72%), without significant correlation with iron burden. Of note, patients with hepatitis C virus infection showed lower IGF-1 concentrations than uninfected subjects despite a normal GH reserve, suggesting that partial GH insensitivity at the post-receptor level may play a key role in IGF-1 deficiency described in thalassemic patients.


Subject(s)
Human Growth Hormone/deficiency , Insulin-Like Growth Factor I/deficiency , Laron Syndrome , beta-Thalassemia , Adult , Age Factors , Arginine , Calcification, Physiologic/physiology , Cohort Studies , Female , Growth Disorders/diagnosis , Growth Disorders/epidemiology , Growth Disorders/metabolism , Growth Hormone-Releasing Hormone , Hepatitis C/epidemiology , Hepatitis C/metabolism , Human Growth Hormone/blood , Humans , Insulin-Like Growth Factor I/metabolism , Iron Overload/metabolism , Laron Syndrome/diagnosis , Laron Syndrome/epidemiology , Laron Syndrome/metabolism , Male , Prevalence , Young Adult , beta-Thalassemia/diagnosis , beta-Thalassemia/epidemiology , beta-Thalassemia/metabolism
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