Prevalence of growth hormone deficiency in adult polytransfused β-thalassemia patients and correlation with transfusional and chelation parameters.
BACKGROUND
Dysfunction of GH-IGF-I axis has been described in many patients affected by β-thalassemia major (TM), especially in children and in adolescents. Recent studies have demonstrated the necessity to evaluate adult patients affected by TM to establish the presence of this alteration which could be relevant in the pathogenesis of cardiac and bone disease, frequently present in this hematological condition. The pathogenesis of this alteration, correlated in the past with iron overload, is not yet completely understood.


OBJECTIVE
The aim of this paper is to evaluate GH-IGF-I axis in a group of adult polytransfused β-thalassemic patients (TM) and to correlate the results with transfusional and chelation parameters.


METHODS
We performed an arginine plus GHRH stimulation test in 28 adult TM patients. Ferritin, IGF-I, liver enzymes, and liver iron concentration, assessed by a superconducting quantum interference device (SQUID) susceptometer were also determined. Moreover, in each patient we evaluated the bone status by a dual-energy X-ray absorptiometry study.


RESULTS
We found the presence of GH deficit in 9 patients (32.1%). There were no significant differences between the two groups regarding the value of ferritin, liver enzymes, and liver iron concentration, assessed by SQUID. The group affected by GH deficit showed a worse bone profile.


CONCLUSIONS
This study confirms the necessity to screen the status of GH/IGF-I axis in this group of patients, even in adult age. The presence of GH deficiency does not seem to be correlated with the efficacy parameters of transfusional and chelation therapy. Other mechanisms, additional to iron overload, could therefore play a role in the pathogenesis of this clinical condition. The presence of GH deficit seems to be very important on clinical aspects, like bone disease, that are crucial for quality of life in these patients.
Poggi
M
M
Chair of Endocrinology, 2nd Faculty of Medicine, University of Rome La Sapienza, via di Grottarossa 1035, 00189 Rome, Italy. poggimaurizio@yahoo.it
Pascucci
C
C
Monti
S
S
Pugliese
P
P
Lauri
C
C
Amodeo
G
G
Girelli
G
G
Toscano
V
V
D015502:Absorptiometry, Photon; D000293:Adolescent; D000328:Adult; D001120:Arginine; D001803:Blood Transfusion; D015913:Chelation Therapy; D005260:Female; D013006:Growth Hormone / Q000172:deficiency*; D013007:Growth Hormone-Releasing Hormone; D006801:Humans; D008297:Male; D017086:beta-Thalassemia / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy
D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone; D001120:Arginine