Abstract
Endocrine complications of haemochromatosis and heart failure mostly affect morbidity and mortality in polytransfused patients. This study analyzes endocrine dysfunctions and the impact of GH-IGF-1 axis alteration on cardiac performance in a population of 31 patients. A retrospective study on 31 Caucasian polytransfused outpatients, 27 adults and 4 pediatric, residing in Apulia, Italy, followed from 2005 to 2016, was conducted. Patients underwent basal and dynamic hormonal evaluation. GHRH plus arginine test was performed in 21 patients (19 adults and 2 children). Among them, 9 patients were affected by left ventricle diastolic dysfunction and/or atrial or ventricular dilatation (HD group) and 12 patients did not have cardiovascular disease (non-HD group). Twenty-nine out of 31 patients (94%) had at least one endocrinopathy. We found severe or mild GH deficit (GHD) in all HD patients versus 3 patients in the non-HD group (p=0.001). Mean IGF-1 levels were significantly lower in the HD group than in non-HD subjects (53±30 versus 122±91 μg/L, p=0.04). Our study confirms the need to perform a dynamic evaluation of the GH-IGF1 axis in polytransfused patients, especially when heart dysfunction emerges. An intervention study with GH replacement therapy in a larger randomized adult population will clarify the role of GH/IGF axis on cardiovascular outcomes in this patient population.
Highlights
Despite the availability of effective new iron-chelating drugs, secondary haemochromatosis due to iron deposition in endocrine glands remains the main factor contributing to the endocrine complications in patients treated with periodic transfusional regimen because of β-thalassaemia and other haemoglobinopathies
Studies regarding the function of growth hormone (GH) and insulin like growth factor 1 (IGF-1) axis have shown a GH defect (GHD) in about 30% of polytransfused patients [2, 4, 5] and reported that lower IGF-I levels are correlated with a worst bone mineral density (BMD) [2, 5,6,7]
A GHD was found in 12 adult patients (39%) which was severe in 9 (29%) and mild in 3 patients (9.6%)
Summary
Despite the availability of effective new iron-chelating drugs, secondary haemochromatosis due to iron deposition in endocrine glands remains the main factor contributing to the endocrine complications in patients treated with periodic transfusional regimen because of β-thalassaemia and other haemoglobinopathies. Among several possible causes, impaired GH/IGF-I axis may contribute to cardiovascular outcomes in both polytransfused [2, 10] and in nonpolytransfused patients [11], but in the literature, there is a lack of clinical data on this issue. The purpose of this observational study was to explore GH-IGF-1 axis function in a population of mainly adult polytransfused patients and correlate it with cardiovascular function
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
