Nakavachara Pairunyar, Petchkul Jaturat, Jeerawongpanich Krittha, Kiattisakthavee Pornpimol, Manpayak Teerarat, Netsakulnee Parichat, Chaichanwattanakul Katharee, Pooliam Julaporn, Srichairatanakool Somdet, Viprakasit Vip
Division of Pediatric Endocrinology, Faculty of Medicine Siriraj Hospital, Department of Pediatrics, Mahidol University, Bangkok, Thailand.
Clinical Epidemiology Unit, Office for Research and Development, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Pediatr Blood Cancer. 2018 Jan;65(1). doi: 10.1002/pbc.26744. Epub 2017 Aug 12.
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.
To determine the prevalence of low bone mass among adolescents with NTD Hb E/β-thalassemia and factors relating to low bone mass.
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.
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.
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.
尽管进行了充分的输血和铁螯合治疗，但输血依赖型β地中海贫血青少年中低骨量很常见。然而，关于非输血依赖型地中海贫血（NTDT）青少年的骨矿物质密度（BMD）的报道很少。实际上，仅报道了中间型非输血依赖型（NTD）β地中海贫血患者的BMD数据。以前没有研究调查过非输血依赖型血红蛋白（Hb）E/β地中海贫血青少年的BMD。
确定非输血依赖型Hb E/β地中海贫血青少年中低骨量的患病率以及与低骨量相关的因素。
我们通过双能X线吸收法测量了22名非输血依赖型Hb E/β地中海贫血青少年（年龄13.2 - 20岁）的腰椎（L2 - L4；BMDLS）和全身（BMDTB）的BMD。
在腰椎（根据骨龄和身高年龄调整的BMDLS Z评分）低骨量的发生率分别为18.2%和22.7%，在全身（根据骨龄和身高年龄调整的BMDTB Z评分）分别为13.6%和9.1%。与血红蛋白水平≥8 g/dl的患者相比，平均血红蛋白水平<8 g/dl的患者更有可能出现低骨量（根据骨龄调整的BMDLS和BMDTB Z评分）。平均血红蛋白水平与根据骨龄调整的BMDLS和BMDTB Z评分相关。
我们证明，在非输血依赖型Hb E/β地中海贫血青少年中，低血红蛋白水平与低骨量有关。这些患者中相当一部分存在低骨量，这突出了适当管理的重要性，包括红细胞输血、补充维生素D和钙，以改善长期骨骼健康。
