Publication: Bone histomorphometry in children and adolescents with β-thalassemia disease: Iron-associated focal osteomalacia
Issued Date
2003-08-01
Resource Type
ISSN
0021972X
Other identifier(s)
2-s2.0-0042383381
Rights
Mahidol University
Rights Holder(s)
SCOPUS
Bibliographic Citation
Journal of Clinical Endocrinology and Metabolism. Vol.88, No.8 (2003), 3966-3972
Suggested Citation
Pat Mahachoklertwattana, Vorachai Sirikulchayanonta, Ampaiwan Chuansumrit, Patcharee Karnsombat, Lulin Choubtum, Arporn Sriphrapradang, Somnuek Domrongkitchaiporn, Rojana Sirisriro, Rajata Rajatanavin Bone histomorphometry in children and adolescents with β-thalassemia disease: Iron-associated focal osteomalacia. Journal of Clinical Endocrinology and Metabolism. Vol.88, No.8 (2003), 3966-3972. doi:10.1210/jc.2002-021548 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/20704
Research Projects
Organizational Units
Authors
Journal Issue
Thesis
Title
Bone histomorphometry in children and adolescents with β-thalassemia disease: Iron-associated focal osteomalacia
Other Contributor(s)
Abstract
Thalassemia/hemoglobinopathy is a hereditary disease that causes chronic anemia and increased erythropoiesis. Consequently, an expansion of bone marrow spaces may contribute to osteopenia/osteoporosis. However, the pathogenesis of bone changes is not yet known. We, therefore, carried out the study on bone histomorphometry and biochemical and hormonal profiles in children and adolescents with suboptimally treated β-thalassemia disease with the hope of gaining some new insight into the cellular and structural alterations of thalassemic bone. Seventeen patients underwent iliac crest bone biopsy for histomorphometric analyses. Bone mineral density (BMD) measurements were performed by dual energy x-ray absorptiometry. Most patients had growth retardation and delayed bone age. BMD was low especially at the lumbar spine. Serum IGF-I levels were almost always low. Bone histomorphometry revealed increased osteoid thickness, osteoid maturation time, and mineralization lag time, which indicate impaired bone matrix maturation and defective mineralization. In addition, iron deposits appeared along mineralization fronts and osteoid surfaces. Moreover, focal thickened osteoid seams were found together with focal iron deposits. Dynamic bone formation study revealed reduced bone formation rate. These findings indicate that delayed bone maturation and focal osteomalacia are the pathogenesis of bone disease in suboptimally blood-transfused thalassemics with iron overload. Iron deposits in bone and low circulating IGF-I levels may partly contribute to the above findings.