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中国大陆地区血红蛋白Bart's病妊娠情况调查。

A survey of pregnancies with Hb Bart's disease in Mainland China.

作者信息

Yang Yu, Li Dong-Zhi

机构信息

Department of Obstetrics and Gynecology, Guangzhou Maternal & Neonatal Hospital, Guangzhou Women and Children's Medical Center, Guangzhou Medical College, Guangzhou, Guangdong, China.

出版信息

Hemoglobin. 2009;33(2):132-6. doi: 10.1080/03630260902817313.

Abstract

alpha-Thalassemia (alpha-thal) is a common inherited disease in southern China. The severest form is Hb Bart's disease, in which the affected individuals are lethal in utero or shortly after birth, and the pregnancies are at high risk for severe morbidity. A survey by us revealed a total of 68 cases of Hb Bart's disease during the past 7 years at a maternal hospital and none of these showed that a risk of thalassemia had been recognized prior to the development of hydrops fetalis. The number of cases of Hb Bart's disease did not vary significantly during the study period. This means that the prevention program for alpha-thal has not been efficient. More effort is needed to efficiently control this disease in our region.

摘要

α地中海贫血(α-地贫)是中国南方常见的遗传性疾病。最严重的形式是血红蛋白巴特氏病,受影响的个体在子宫内或出生后不久就会死亡,并且怀孕会面临严重发病的高风险。我们进行的一项调查显示,在过去7年中,一家妇产医院共有68例血红蛋白巴特氏病病例,而且这些病例中没有一例表明在胎儿水肿发生之前已认识到地中海贫血风险。在研究期间,血红蛋白巴特氏病的病例数没有显著变化。这意味着α-地贫的预防计划没有成效。在我们地区需要付出更多努力来有效控制这种疾病。

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