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Pediatr Endocrinol Rev., 2008; 6 Suppl 1: 127-31, PMID: 19360988

Study of bone density by pQCT analysis in healthy adults and patients with B-thalassemia major and intermedia

Jahr: 2008

Ladis V, Raptou P, Rigatou E, Chouliaras G, Galanos A, Korres D, Kattamis C
Thalassemia Unit, First Department of Pediatrics, University of Athens, Aghia Sophia Children"s Hospital, Athens, Greece.


BACKGROUND: Bone changes are a prominent symptom of beta-thalassemias, related to expansion of bone marrow and reduction of bone density. Conventional treatment ameliorates bone changes and improves survival, thus increasing the morbidity of bone diseases in adulthood. Peripheral quantitative computer tomography (pQCT) was used recently to assess the changes in volumetric bone mineral density (vBMD) in various bone compartments. OBJECTIVES: Assessment of indices of bone density and structure in patients with thalassemia major (thal-major) and intermedia (thal-interm) on conventional therapy and in healthy adults. MATERIAL AND METHODS: 45 patients with thal-major, 27 with thal-interm and 32 healthy individuals aged 21-42 years were studied by pQCT analysis. The vBMD total (tot), trabecular (trab) and cortical (cort), the bone mineral content (BMC), the cross sectional area (CSA), the cortical thickness (CRTHK) and the stress strain index (SSI) were assessed at the 4% site of the distal radius. RESULTS: Tot, trab, and cort vBMD, BMC, and cortical thickness showed statistically significant differences among the three groups with significant reduction in thalassemics. No significant differences were found in the three groups with CSA and SSI. Impairment of bone density and structure in Greek thalassemics on proper treatment was not as severe as expected. A significant proportion of patients had bone density indices within the normal range and above the 10th percentile of normal. CONCLUSIONS: Peripheral QCT analysis is a convenient method to study the regional changes of bone density in patients with thalassemia. These changes affect mainly the cortical compartment and are more pronounced in thalassemia intermedia.

GID: 1804; Letzte Änderung: 16.04.2009