Purpose GH deficit (GHD) could represent an endocrine issue in ss-Thalassemia Major(ssTM) patients. GH/IGF-1 axis has not been extensively explored in ssTM adults, so far. We aim to assess GHD and IGF-1 deficiency prevalence in ssTM adult population, focusing on the relationship with liver disease. Methods Cross-sectional multi-centre studyconducted on 81 adult ssTM patients (44 males, mean age 41 +/- 8 years) on transfusion and chelation therapy. GHD was investigated by GHRH + arginine test. IGF-1 levels, routine biochemical exams, Fibroscan, Hepatic Magnetic Resonance Imaging (MRI) and pituitary MRI were collected. Results Eighteen patients were affected by GHD and 63 were not (nGHD) according to GHRH + arginine test, while basal GH levels did not differ. GHD was associated with a higher BMI and a worse lipid profile (p < 0.05). No significant differences were observed regarding liver function between the two groups. Pituitary MRI scan was normal except for one case of empty sella. The 94.4% and 93.6% of GHD and nGHD, respectively, presented lower IGF-1 levels than the reference range, and mean IGF-1 SDS was significantly lower in GHD patients. Conclusion GHD is frequent in adult ssTM patients and is associated with higher BMI and worse lipid profile. nGHD patients present lower IGF-1 levels as well. There was no relationship between IGF-1 levels and liver disease. Further, multicentric studies with larger cohorts and standardized diagnostic protocols are needed.

GH/IGF-1 axis in a large cohort of ss-thalassemia major adult patients: a cross-sectional study

I. Gagliardi;R. Mungari;L. Aliberti;M. Bondanelli;Maria Chiara Zatelli;M. R. Ambrosio
2022

Abstract

Purpose GH deficit (GHD) could represent an endocrine issue in ss-Thalassemia Major(ssTM) patients. GH/IGF-1 axis has not been extensively explored in ssTM adults, so far. We aim to assess GHD and IGF-1 deficiency prevalence in ssTM adult population, focusing on the relationship with liver disease. Methods Cross-sectional multi-centre studyconducted on 81 adult ssTM patients (44 males, mean age 41 +/- 8 years) on transfusion and chelation therapy. GHD was investigated by GHRH + arginine test. IGF-1 levels, routine biochemical exams, Fibroscan, Hepatic Magnetic Resonance Imaging (MRI) and pituitary MRI were collected. Results Eighteen patients were affected by GHD and 63 were not (nGHD) according to GHRH + arginine test, while basal GH levels did not differ. GHD was associated with a higher BMI and a worse lipid profile (p < 0.05). No significant differences were observed regarding liver function between the two groups. Pituitary MRI scan was normal except for one case of empty sella. The 94.4% and 93.6% of GHD and nGHD, respectively, presented lower IGF-1 levels than the reference range, and mean IGF-1 SDS was significantly lower in GHD patients. Conclusion GHD is frequent in adult ssTM patients and is associated with higher BMI and worse lipid profile. nGHD patients present lower IGF-1 levels as well. There was no relationship between IGF-1 levels and liver disease. Further, multicentric studies with larger cohorts and standardized diagnostic protocols are needed.
2022
Gagliardi, I.; Mungari, R.; Gamberini, M. R.; Fortini, M.; Dassie, F.; Putti, M. C.; Maffei, P.; Aliberti, L.; Bondanelli, M.; Zatelli, Maria Chiara; Ambrosio, M. R.
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/2488837
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 4
  • ???jsp.display-item.citation.isi??? 2
social impact