Unit:
Τομέας Υγείας - Μητέρας - ΠαιδιούLibrary of the School of Health Sciences
Author:
Tsartsalis Athanasios
Dissertation committee:
Γεώργιος Χρούσος, Καθηγητής, Ιατρική, ΕΚΠΑ
Χριστίνα Κανακά-Gantenbein, Καθηγήτρια, Ιατρική, ΕΚΠΑ
Αντώνης Καττάμης, Καθηγητής, Ιατρική, ΕΚΠΑ
Ευάγγελος Τέρπος, Αν. Καθηγητής, Ιατρική, ΕΚΠΑ
Σουλτάνα Σιαχανίδου, Αν. Καθηγήτρια, Ιατρική, ΕΚΠΑ
Φλώρα Μπακοπούλου, Επ. Καθηγήτρια, Ιατρική, ΕΚΠΑ
Λυδία Κόσσυβα, Επ. Καθηγήτρια, Ιατρική, ΕΚΠΑ
Original Title:
Εκτίμηση της οστικής πυκνότητας και του οστικού μεταβολισμού σε ασθενείς με ομόζυγη και ενδιάμεση β-Μεσογειακή Αναιμία
Translated title:
Bone mineral density and bone metabolism in patients with thalassemia major and intermedia
Summary:
Introduction
Thalassemia major (TM) is a hereditary disease caused by defective globin synthesis. During recent years, patients with TM have benefited from a significant increase in life expectancy, but eventually they face many new health conditions, including endocrinopathies and low bone mineral density, usually observed in the aging general population. The aim of the present study was to perform a meta-analysis of all available data on the role of bisphosphonates (BPs) in the therapy of thalassemia major-induced osteoporosis, to evaluate biomarkers of bone remodeling in TM patients and to compare them with both osteoporotic and healthy population, in order to investigate new therapeutic paths.
Methods
The PRISMA recommendations for reporting systematic reviews and meta-analyses were used to guide the present study. We searched PubMed and the Cochrane Central Register of Controlled Trials (CENTRAL) through March 31st 2017 for articles related to thalassemia and BPs. In the next step Sixty-four patients with TM ( 32 men and 32 women) participated in a cross-sectional study design. The patients were recruited from Aghia Sofia Children’s Hospital and evaluated with dual-energy X-ray absorptiometry (DXA) of the lumbar spine and femoral neck and with markers of bone remodelling including receptor activator of nuclear factor kappa-Β ligand (RANKL), osteoprotegerin (OPG), C-terminal telopeptide (CTX), and sclerostin. Results were compared with those from 12 postmenopausal women with osteoporosis and 12 postmenopausal women with normal bone mineral density.
Results
The BPs investigated included zoledronate, alendronate, pamidronate, clodronate and neridronate. Zoledronate and alendronate showed a tendency to perform best as compared to neridronate and the placebo effect with respect to femoral neck, lumbar spine, total hip, and total body in terms of Bone Mass Density (gr/cm2). The statistical
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analysis of the biochemical markers of bone metabolism revealed overall significant differences between the three groups only for RANKL and OPG/RANKL
Conclusions
BPs and in particular, zolendronate, were quite effective in the treatment of osteoporosis. These findings suggested that bisphosphonates are still a front-line treatment of osteoporosis in TM. Patients with TM do not have a higher probability of suffering from osteoporosis from the general population. However, some markers of osteoclast activity differ between patients with TM and osteoporosis, indicating possibly differences in terms of anti-osteoporotic treatment. The lack of significant differences among the three groups in regards to the levels of CTX and sclerostin may indicate the potential efficacy of the current osteoporotic treatment also for TM patients.
Main subject category:
Health Sciences
Keywords:
Osteoporosis, Bone Mineral Density, Bisphosphonates, β-thalassemia major, Zoledronic acid, Sclerostin, RANKL, OPG
Number of references:
141
