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Title: Prevalence of low bone mass among adolescents with nontransfusion-dependent hemoglobin E/β-thalassemia and its relationship with anemia severity
Authors: Pairunyar Nakavachara
Jaturat Petchkul
Krittha Jeerawongpanich
Pornpimol Kiattisakthavee
Teerarat Manpayak
Parichat Netsakulnee
Katharee Chaichanwattanakul
Julaporn Pooliam
Somdet Srichairatanakool
Vip Viprakasit
Faculty of Medicine, Siriraj Hospital, Mahidol University
Chiang Mai University
Keywords: Medicine
Issue Date: 1-Jan-2018
Citation: Pediatric Blood and Cancer. Vol.65, No.1 (2018)
Abstract: © 2017 Wiley Periodicals, Inc. Background: Low bone mass is common among adolescents with transfusion-dependent β-thalassemia despite adequate transfusion and iron chelation. However, there are few reports regarding bone mineral density (BMD) among adolescents with nontransfusion-dependent thalassemia (NTDT). Indeed, only BMD data in patients with nontransfusion-dependent (NTD) β-thalassemia intermedia have been reported. No previous study has investigated BMD among adolescents with NTD hemoglobin (Hb) E/β-thalassemia. Objective: To determine the prevalence of low bone mass among adolescents with NTD Hb E/β-thalassemia and factors relating to low bone mass. Methods: We investigated BMD of lumbar spine (L2–L4; BMDLS) and total body (BMDTB), as measured by dual-energy X-ray absorptiometry, in 22 adolescents (aged 13.2–20 years) with NTD Hb E/β-thalassemia. Results: Low bone mass was found to be 18.2% and 22.7% at the lumbar spine (BMDLS Z-score adjusted for bone age and height age) and 13.6% and 9.1% at the total body (BMDTB Z-score adjusted for bone age and height age). Patients with mean Hb level <8 g/dl were more likely to have low bone mass (BMDLS and BMDTB Z-scores adjusted for bone age) compared to those with Hb level ≥ 8 g/dl. Mean Hb level correlated with BMDLS and BMDTB Z-scores adjusted for bone age. Conclusion: We demonstrated that a low Hb level was associated with low bone mass among adolescents with NTD Hb E/β-thalassemia. A significant proportion of low bone mass among these patients highlights the importance of appropriate management, including red cell transfusion, vitamin D and calcium supplementation for improved long-term bone health.
ISSN: 15455017
Appears in Collections:Scopus 2018

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