Growth hormone secretion in adult patients with thalassaemia.
OBJECTIVE
Growth retardation and short stature are frequent clinical features of patients with beta-thalassaemia major. Dysfunction of the GH-IGF-1 axis has been described in many thalassaemic children and adolescents with short stature and reduced growth velocity. Several studies have demonstrated that recombinant GH treatment improves growth velocity in these patients, although response to the treatment is variable and not predictable. A reassessment of the GH-IGF-1 axis must be performed in young adults with childhood-onset GH deficiency (GHD), after attainment of final height, to select those who are candidates for replacement therapy as adults. To our knowledge there are no data available on retesting the GH-IGF-1 axis in adult thalassaemic patients with childhood-onset GHD. The aim of our study was to investigate GH secretion in adult thalassaemic patients with childhood-onset GHD.


METHODS
We reassessed GH secretion in a group of adult thalassaemic patients in whom partial GHD had been diagnosed during childhood.


METHODS
We performed an arginine plus GH-releasing hormone (GHRH) stimulation test in 16 thalassaemic patients (10 males, six females) with a mean age of 24.8 +/- 3.6 years. The cut-off level for GH response was set at 9 microg/l, according to the literature. Ferritin, IGF-1, liver enzymes and lipid levels were also determined.


RESULTS
We found persisting GHD in three patients, one patient had borderline values (GH peak = 10.4 microg/l), whereas the others had a normal response. These results are in accordance with the data on GH retesting in adult patients with idiopathic partial childhood-onset GHD.


CONCLUSIONS
We conclude that GH status should be retested in adult thalassaemic patients with childhood-onset GHD. If the diagnosis of adult GHD is established, GH treatment may be considered as it could contribute to improve heart function and bone mineral density, which are frequently impaired in adult thalassaemic patients.
La Rosa
Clementina
C
Department of Paediatrics, Azienda Policlinico-Università di Catania, Catania, Italy.
De Sanctis
Vincenzo
V
Mangiagli
Antonino
A
Mancuso
Michele
M
Guardabasso
Vincenzo
V
Galati
Maria Concetta
MC
Caruso-Nicoletti
Manuela
M
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001120:Arginine; D005260:Female; D005293:Ferritins / Q000097:blood; D013006:Growth Hormone / Q000097:blood / Q000172:deficiency*; D013007:Growth Hormone-Releasing Hormone; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008055:Lipids / Q000097:blood; D008297:Male; D013789:Thalassemia / Q000097:blood*
D008055:Lipids; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone; D005293:Ferritins; D013007:Growth Hormone-Releasing Hormone; D001120:Arginine