Hemoglobin H Disease in Guangxi Province, Southern China: Clinical Review of 357 Patients

被引：20

作者：

Yin, Xiao-Llin ^{[1
]}

Zhang, Xin-Hua ^{[1
]}

Zhou, Tian-Hong ^{[1
]}

Zhang, Tian-Lang ^{[1
]}

Luo, Rui-Gui ^{[1
]}

Wang, Li ^{[1
]}

Zhou, Ya-Li ^{[1
]}

Chen, Yong-Sheng ^{[1
]}

Kong, Xiang-Jin ^{[1
]}

Liang, Bo ^{[1
]}

He, Yuan-Yuan ^{[1
]}

Peng, Lin ^{[1
]}

Lu, Li-Biao ^{[1
]}

Fang, Su-Ping ^{[2
]}

Wu, Zhi-Kui ^{[2
]}

机构：

[1] 303rd Hosp Peoples Liberat Army, Dept Hematol, Nanning 530021, Guangxi, Peoples R China

[2] China Acad Tradit Chinese Med, GuangAnMen Hosp, Dept Mol Biol, Beijing, Peoples R China

来源：

ACTA HAEMATOLOGICA | 2010年 / 124卷 / 02期

关键词：

Hemoglobin H disease; Human alpha-globin genes; Anemia; Guangxi province; ERYTHROID MARROW ACTIVITY; BETA-THALASSEMIA; ALPHA-THALASSEMIA; GLOBIN GENE; WESTMEAD <ALPHA-122(H5)HIS->GLN>; KB DELETION; DIAGNOSIS; HYPERBILIRUBINEMIA; POPULATION; GENOTYPES;

D O I：

10.1159/000314058

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The clinical characteristics of 357 patients with hemoglobin H (HbH) disease from the Guangxi province of Southern China were studied. One hundred and ninety-one (53.3%) patients were diagnosed with HbH-Constant Spring, 19 were diagnosed with HbH Westmead. Ten patients were shown to have coinherited HbH-Constant Spring/QS with a beta-thalassemia mutation. Coinheritance of the beta-thalassemia gene does not alleviate anemia (8.2 +/- 2.3 vs. 7.6 +/- 1.7 g/dl, p = 0.276), or influence age at diagnosis (20.2 +/- 19.6 vs. 12.9 +/- 11.0 years, p = 0.276). Ferritin levels were significantly higher in the group of patients with the nondeletional form of the disease (475 +/- 719 vs. 249 +/- 264 ng/ml, p = 0.005). Copyright (c) 2010 S. Karger AG, Basel

引用

页码：86 / 91

页数：6

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