目的 分析小儿血红蛋白H病（HbH病）血清可溶性转铁蛋白受体（sTfR）水平，探讨其与HbH病贫血程度的关系。方法 纳入55例HbH病患儿以及30例正常健康儿童（对照组）为研究对象，回顾性分析缺失型、非缺失型HbH病组以及对照组的血液学指标与血清sTfR水平。结果 55例HbH病中，缺失型39例，非缺失型16例。缺失型与非缺失型HbH组血红蛋白（Hb）、平均红细胞体积（MCV）、平均血红蛋白含量（MCH）均低于对照组（P < 0.05），血清sTfR水平均高于对照组（P < 0.05）。非缺失型HbH病组红细胞（RBC）、Hb水平低于缺失型HbH病组（P < 0.05），而MCV、MCH以及血清sTfR水平高于缺失型HbH病组（P < 0.05）。HbH病患儿血清sTfR水平与RBC、Hb水平呈负相关（r分别为-0.739、-0.667，均P < 0.05）；而与MCV、MCH呈正相关（r分别为0.750、0.434，均P < 0.05）。结论 血清sTfR水平与HbH病患儿贫血程度相关，可能是HbH病治疗的一个靶点。
Abstract
Objective To investigate the serum level of soluble transferrin receptor (sTfR) and its association with the degree of anemia in children with hemoglobin H (HbH) disease. Methods A total of 55 children with HbH disease were enrolled as the HbH group, and 30 healthy children were enrolled as the control group. The HbH group was further divided into a deletional HbH disease group and a non-deletional HbH disease group. A retrospective analysis was performed for hematological parameters and serum sTfR level in all groups. Results Of the 55 children with HbH disease, 39 had deletional HbH disease and 16 had non-deletional HbH disease. Compared with the control group, the deletional and non-deletional HbH disease groups had significantly lower hemoglobin (Hb), mean corpuscular volume (MCV), and mean corpuscular hemoglobin (MCH) and a significantly higher serum level of sTfR. Compared with the deletional HbH disease group, the non-deletional HbH disease group had significantly lower red blood cell count (RBC) and Hb level and significantly higher MCV, MCH, and serum sTfR level. In children with HbH disease, serum sTfR level was negatively correlated with RBC and Hb level (r=-0.739 and -0.667 respectively, P < 0.05) and positively correlated with MCV and MCH (r=0.750 and 0.434 respectively, P < 0.05). Conclusions Serum sTfR level is associated the degree of anemia in children with HbH disease, and sTfR may be a target for the treatment of HbH disease.
关键词
血红蛋白H病 /
可溶性转铁蛋白受体 /
贫血 /
儿童
Key words
Hemoglobin H disease /
Soluble transferrin receptor /
Anemia /
Child
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基金
深圳市卫生计生系统科研项目（SZFZ2017051）。
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