title: GH deficiency in adult B-thalassemia major patients and its relationship with IGF-1 production.
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.
authors.affiliation: Clinica Medica, Universitá Milano-Bicocca, Ospedale San Gerardo dei Tintori Via Pergolesi 33, Monza, Italy. angelaida.pincelli@noesim.it
authors.forename: Angela Ida
authors.initials: AI
authors.lastname: Pincelli
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Masera
authors.forename: Luisa
authors.initials: L
authors.lastname: Tavecchia
authors.forename: Mario
authors.initials: M
authors.lastname: Perotti
authors.forename: Silvia
authors.initials: S
authors.lastname: Perra
authors.forename: Raffaela
authors.initials: R
authors.lastname: Mariani
authors.forename: Alberto
authors.initials: A
authors.lastname: Piperno
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Mancia
authors.forename: Guido
authors.initials: G
authors.lastname: Grassi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Masera
mesh_terms: D000328:Adult; D000367:Age Factors; D001120:Arginine; D002113:Calcification, Physiologic / Q000502:physiology; D015331:Cohort Studies; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism; D013007:Growth Hormone-Releasing Hormone; D006526:Hepatitis C / Q000453:epidemiology / Q000378:metabolism; D019382:Human Growth Hormone / Q000097:blood / Q000172:deficiency*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000172:deficiency* / Q000378:metabolism; D019190:Iron Overload / Q000378:metabolism; D046150:Laron Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism; D008297:Male; D015995:Prevalence; D055815:Young Adult; D017086:beta-Thalassemia / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism
chemical_list: D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; D013007:Growth Hormone-Releasing Hormone; D001120:Arginine