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.
| Original language | English |
|---|---|
| Pages (from-to) | 284-289 |
| Number of pages | 6 |
| Journal | Pediatric Endocrinology Reviews |
| Volume | 8 Suppl 2 |
| Publication status | Published - Mar 2011 |
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ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Endocrinology, Diabetes and Metabolism
Cite this
GH deficiency in adult B-thalassemia major patients and its relationship with IGF-1 production. / Pincelli, Angela Ida; Masera, Nicoletta; Tavecchia, Luisa; Perotti, Mario; Perra, Silvia; Mariani, Raffaela; Piperno, Alberto; Mancia, Giuseppe; Grassi, Guido; Masera, Giuseppe.
In: Pediatric Endocrinology Reviews, Vol. 8 Suppl 2, 03.2011, p. 284-289.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - GH deficiency in adult B-thalassemia major patients and its relationship with IGF-1 production.
AU - Pincelli, Angela Ida
AU - Masera, Nicoletta
AU - Tavecchia, Luisa
AU - Perotti, Mario
AU - Perra, Silvia
AU - Mariani, Raffaela
AU - Piperno, Alberto
AU - Mancia, Giuseppe
AU - Grassi, Guido
AU - Masera, Giuseppe
PY - 2011/3
Y1 - 2011/3
N2 - 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.
AB - 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.
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M3 - Article
C2 - 21705979
AN - SCOPUS:80052309328
VL - 8 Suppl 2
SP - 284
EP - 289
JO - Pediatric Endocrinology Reviews
JF - Pediatric Endocrinology Reviews
SN - 1565-4753
ER -