Document Detail


Bone demineralization in adult thalassaemic patients: contribution of GH and IGF-I at different skeletal sites.
MedLine Citation:
PMID:  18221395     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND AND OBJECTIVE: GH and IGF-I exert an important role in the control of bone formation, as shown by decreased bone mineral density and increased fracture risk in adult hypopituitary patients untreated for GH deficiency (GHD). Different degrees of bone demineralization are frequently reported in patients affected by beta-thalassaemia. Considering the high prevalence of GHD recently observed by our group among adult thalassaemic patients, we elected to study the possible role of GH-IGF-I abnormalities in the pathogenesis of the osteopenia/osteoporosis of this disease.
DESIGN: Sixty-four adult thalassaemic patients (49 with thalassaemia major and 15 with thalassaemia intermedia, 23 men and 41 women, aged 31.4 +/- 6.8 years) were studied.
METHODS: Bone mineral density was assessed by dual energy X-ray absorptiometry at lumbar spine in 62 patients and at proximal femur in 58. All patients underwent GHRH (1 microg/kg as an i.v. bolus) plus arginine (0.5 g/kg as a 30-min i.v. infusion) testing. Severe GHD was defined by GH peaks < 9 microg/l, whereas partial GHD was defined by GH peaks ranging from 9 to 16.5 microg/l. Blood samples for IGF-I measurement were collected.
RESULTS: Lumbar osteoporosis and osteopenia were demonstrated in 46/62 (74.1%) and 14/62 (22.5%) patients, respectively. Femoral osteoporosis and osteopenia were documented in 22/58 (37.9%) and 32/58 (55.1%) patients, respectively. Severe GHD was demonstrated in 16/64 patients (25%), while 11 additional patients (17.1%) displayed partial GHD. IGF-I standard deviation score (SDS) was low, that is, below -1.88, in the majority (54.6%) of patients. Lumbar T-score values were not correlated with either GH peaks or IGF-I SDS values. Femoral T-score values were positively correlated with GH peaks (r = 0.38, P < 0.005) and IGF-I SDS values (r = 0.39, P < 0.005). Multiple regression analysis pointed to both GH peak and IGF-I SDS as predictors of femoral T-score. Furthermore, mean femoral T-score was significantly lower in patients with severe GHD than in those with normal GH secretion (-2.94 +/- 0.25 vs.-2.15 +/- 0.12, P < 0.01).
CONCLUSION: This study, while confirming the high prevalence of both osteopenia/osteoporosis and somatotropin-somatomedin deficiency in adult thalassaemic patients, indicates that defective GH secretion and diminished serum IGF-I levels may contribute to femoral demineralization in these patients. Further studies are worth carrying out to evaluate the efficacy of biosynthetic GH administration on bone abnormalities of GH-deficient thalassaemic adults.
Authors:
Massimo Scacchi; Leila Danesi; Agnese Cattaneo; Elena Valassi; Francesca Pecori Giraldi; Crocetta Argento; Emanuela D'Angelo; Nadia Mirra; Vittorio Carnelli; Laura Zanaboni; Benedetta Tampieri; Maria Domenica Cappellini; Francesco Cavagnini
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-01-21
Journal Detail:
Title:  Clinical endocrinology     Volume:  69     ISSN:  1365-2265     ISO Abbreviation:  Clin. Endocrinol. (Oxf)     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-08-21     Completed Date:  2009-07-23     Revised Date:  2011-11-24    
Medline Journal Info:
Nlm Unique ID:  0346653     Medline TA:  Clin Endocrinol (Oxf)     Country:  England    
Other Details:
Languages:  eng     Pagination:  202-7     Citation Subset:  IM    
Affiliation:
University of Milan, Ospedale San Luca IRCCS, Istituto Auxologico Italiano, Milan, Italy.
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MeSH Terms
Descriptor/Qualifier:
Absorptiometry, Photon
Adolescent
Adult
Bone Demineralization, Pathologic / blood,  epidemiology,  etiology*,  radiography
Female
Femur / pathology,  radiography
Hand Bones / pathology,  radiography
Human Growth Hormone / blood,  deficiency,  physiology*
Humans
Insulin-Like Growth Factor I / analysis,  physiology*
Lumbosacral Region / pathology,  radiography
Male
Middle Aged
Prevalence
Young Adult
beta-Thalassemia / blood,  complications*,  epidemiology,  radiography
Grant Support
ID/Acronym/Agency:
GGP05285//Telethon
Chemical
Reg. No./Substance:
12629-01-5/Human Growth Hormone; 67763-96-6/Insulin-Like Growth Factor I

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