Department of Pediatrics, Dr Balabhai Nanavati hospital Mumbai, 501, Rangmahal 5th floor, 2 Mount Mary road, Bandra (West), Mumbai 400050, India.
Indian J Pediatr. 2010 Sep;77(9):987-91. doi: 10.1007/s12098-010-0158-2. Epub 2010 Aug 25.
To evaluate osteopathy in thalassemia by bone mineral densitometry (BMD) and biochemical indices.
Prospective review analysis with no follow up from 2006 to 2007 of 42 regularly transfused thalassemics aged 10-25 years (27 boys, 15 girls) was done. Anthropometry, pubertal stage and symptomatology were noted. Urinary C-terminal cross-linked telopeptide of type-1 collagen (Crosslaps) by ELISA; serum 25-OH vitamin D and osteocalcin by RIA; parathyroid hormone (PTH) and ferritin by chemiluminescence and IGF-1 by Enzyme immunoassay were evaluated. Dual Energy X-ray Absorptiometry (DEXA) of lumbar spine and femur was done on Lunar prodigy system. Data was entered and analyzed using the SPSS for Windows software. Mean comparisons were done by ANOVA 1 and data was compared using Chi-square test and p value < 0.05 was taken as significant.
Of 42 patients, 81% had osteoporosis by Z-score of DEXA. Urinary crosslaps was high in 55%; 36% had increased osteocalcin; 62% had low vitamin D levels; 38% had high parathyroid levels and IGF-1 was low in 52%. Mean serum ferritin level was 5344 ± 2855 ng/dl. There was statistical significance (p = 0.046) between chronological age and BMD. All 42 cases were divided into two groups: Group-1 (Normal DEXA), Group-2 (Abnormal DEXA) and analysis of biochemical indices between two groups showed no significant difference in any of the biochemical parameters.
This study revealed majority of thalassemics with inadequate chelation have bone resorption with advancing chronological age and BMD should be evaluated regularly for early diagnosis to prevent morbidity.
通过骨矿物质密度（BMD）和生化指标评估地中海贫血症患者的骨密度。
2006 年至 2007 年，对 42 名年龄在 10-25 岁（27 名男孩，15 名女孩）的定期输血地中海贫血症患者进行了前瞻性回顾性分析，无随访。记录人体测量学、青春期阶段和症状。通过 ELISA 检测尿 1 型胶原 C 端交联肽（Crosslaps）；用 RIA 检测血清 25-羟维生素 D 和骨钙素；用化学发光法检测甲状旁腺激素（PTH）和铁蛋白；用酶联免疫吸附试验检测 IGF-1。在 Lunar prodigy 系统上进行腰椎和股骨的双能 X 射线吸收法（DEXA）。使用 Windows 软件的 SPSS 软件输入和分析数据。通过方差分析 1 进行均值比较，并通过卡方检验比较数据，p 值<0.05 视为显著。
42 例患者中，81%的患者通过 DEXA 的 Z 评分患有骨质疏松症。55%的患者尿 Crosslaps 升高；36%的患者骨钙素升高；62%的患者维生素 D 水平低；38%的患者甲状旁腺水平高，52%的患者 IGF-1 水平低。血清铁蛋白平均水平为 5344±2855ng/dl。年龄与 BMD 之间存在统计学意义（p=0.046）。将 42 例患者分为两组：组 1（DEXA 正常）和组 2（DEXA 异常），对两组生化指标进行分析，两组之间的生化参数均无显著差异。
本研究显示，大多数铁螯合不足的地中海贫血症患者有骨吸收，随着年龄的增长，BMD 应定期评估，以便早期诊断，预防发病。
