血红蛋白H病患者的骨密度评估。

Evaluation of bone mineral density in patients with hemoglobin H disease.

作者信息

Zarei Tahereh, Haghpanah Sezaneh, Parand Shirin, Moravej Hossein, Dabbaghmanesh Mohammad Hossein, Omrani Gholamhossein Ranjbar, Karimi Mehran

机构信息

Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran.

Department of Pediatrics, School of Medicine, Shiraz University of Medical Science, Shiraz, Iran.

出版信息

Ann Hematol. 2016 Aug;95(8):1329-32. doi: 10.1007/s00277-016-2708-9. Epub 2016 Jun 3.

DOI:10.1007/s00277-016-2708-9

PMID:27256348

Abstract

OBJECTIVES

This study was conducted to assess bone mineral density (BMD) and bone mineral content (BMC) of patients with hemoglobin H (HbH) disease.

METHODS

BMD and BMC were measured by dual energy X-ray absorptiometry of the lumbar spines and femur neck in 21 patients with Hb H disease over the age of 10 years. An association of BMD with sex, age, hemoglobin, calcium, phosphorus, and serum ferritin level was also evaluated.

RESULTS

Prevalence of BMD below the expected range for age in the lumbar spine and femur neck region in patients with HbH disease were 33.3 and 14.3 %, respectively. Lumbar BMD was significantly lower in the patients compared to healthy individuals (median (min-max) 0.725 (0.595-0.924) vs. 1.061 (0.645-1.238), P < 0.001)). There was no significant relationship between BMD in the lumbar and femur neck with any of the evaluated variables (P value >0.05).

CONCLUSION

Data regarding bone density in HbH disease is limited; osteoporosis as a common complication of β-thalassemia intermedia syndrome should be considered even in HbH which shows its prevalence is less than β-thalassemia intermedia.

摘要

目的

本研究旨在评估血红蛋白H（HbH）病患者的骨密度（BMD）和骨矿物质含量（BMC）。

方法

采用双能X线吸收法测量了21例年龄超过10岁的HbH病患者腰椎和股骨颈的BMD和BMC。还评估了BMD与性别、年龄、血红蛋白、钙、磷和血清铁蛋白水平之间的相关性。

结果

HbH病患者腰椎和股骨颈区域BMD低于预期年龄范围的患病率分别为33.3%和14.3%。与健康个体相比，患者的腰椎BMD显著降低（中位数（最小值 - 最大值）0.725（0.595 - 0.924）对1.061（0.645 - 1.238），P < 0.001））。腰椎和股骨颈的BMD与任何评估变量之间均无显著关系（P值>0.05）。