Bone mineral density in children with fanconi anemia after hematopoietic cell transplantation.
Bone mineral density in children with fanconi anemia after hematopoietic cell transplantation.
复制标题
范可尼贫血儿童造血细胞移植后的骨密度。
DOI：
10.1016/j.bbmt.2015.01.002
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
MacMillan,MargaretL
中科院分区：
文献类型：
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作者：
Petryk,Anna;Polgreen,LyndaE;Barnum,JessieL;Zhang,Lei;Hodges,JamesS;Baker,KScott;Wagner,JohnE;Steinberger,Julia;MacMillan,MargaretL
Fanconi贫血(FA)是一种遗传性DNA修复障碍，与身材矮小和骨髓衰竭有关，通常需要造血细胞移植(HCT)。虽然白血病患者HCT后出现低骨密度(BMD)的报道，但对FA儿童HCT后的BMD(FA HCT)知之甚少。这项研究的目的是比较FA HCT的骨密度与健康对照组和接受HCT治疗的恶性血液病(癌症)儿童的BMD，并测试BMD与骨丢失危险因素之间的关系。这项横断面研究包括20名FA Hct、13名癌症Hct和90名健康对照，年龄匹配，评估时为18岁。用双能X线骨密度仪测量全身(TBMD)和腰椎(LBMD)的BMD Z评分，并调整年龄身高Z评分(HAZ)。FA Hct的TBMDHAZZ评分低于健康对照组(42%)，≤和−1的Z评分高于健康对照组(42%比11%)。未检测到LBMD缺失。FA Hct组和癌症Hct组在TTBMD或LBMD Z-Score上没有显著差异。在FA HCT患者中，较低的体重指数和较低的脂肪百分比与较低的BMD相关。这项研究强调了监测骨密度对优化FA患者骨健康的重要性。
Fanconi anemia (FA) is an inherited DNA repair disorder associated with short stature and bone marrow failure, usually requiring hematopoietic cell transplantation (HCT). Although low bone mineral density (BMD) has been reported in leukemia patients after HCT, little is known about BMD in FA children after HCT (FA HCT). This study's goals were to compare BMD in FA HCT to BMD in healthy controls and in children who received HCT for hematologic malignancy (cancer HCT), and to test for associations between BMD and risk factors for bone loss. This cross-sectional study included 20 FA HCT, 13 cancer HCT, and 90 healthy controls, age-matched and <18 years old at evaluation. BMD Z-scores for total body (TBMD) and lumbar spine (LBMD) were measured by dual energy x-ray absorptiometry and adjusted for height-for-age Z-score (HAZ). FA HCT had lower mean TBMDHAZZ-score (by .8 SD) and higher fraction with Z-score ≤ −1 than healthy controls (42% versus 11%). No LBMD deficits were detected. FA HCT and cancer HCT groups did not differ significantly in TBMD or LBMD Z-scores. In FA HCT patients, lower body mass index and lower percent fat were associated with lower BMD. This study highlights the importance of monitoring BMD to optimize bone health in FA patients.