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.

Pincelli, A., Masera, N., Tavecchia, L., Perotti, M., Perra, S., Mariani, R., et al. (2011). GH deficiency in adult B-thalassemia major patients and its relationship with IGF-1 production. PEDIATRIC ENDOCRINOLOGY REVIEW, 8(S 2), 284-289.

GH deficiency in adult B-thalassemia major patients and its relationship with IGF-1 production

MARIANI, RAFFAELLA;PIPERNO, ALBERTO;MANCIA, GIUSEPPE;GRASSI, GUIDO;MASERA, GIUSEPPE
2011

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.
Articolo in rivista - Articolo scientifico
B-thalassemia major, GH, IGF-1
English
2011
8
S 2
284
289
none
Pincelli, A., Masera, N., Tavecchia, L., Perotti, M., Perra, S., Mariani, R., et al. (2011). GH deficiency in adult B-thalassemia major patients and its relationship with IGF-1 production. PEDIATRIC ENDOCRINOLOGY REVIEW, 8(S 2), 284-289.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/26720
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