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尼日利亚西南部接受包皮环切术的存活男婴中因子FVIIIC缺乏症的发病率。

Incidence of factor FVIIIC deficiency in live male infants undergoing circumcision in South West, Nigeria.

作者信息

Fakunle E E, Shokunbi W A, Shittu O B

机构信息

Department of Haematology, University College Hospital, Ibadan, Oyo State, Nigeria.

出版信息

Haemophilia. 2007 Sep;13(5):567-9. doi: 10.1111/j.1365-2516.2007.01549.x.

Abstract

A prominent evidence of inherited bleeding disorder in newborn males is excessive post-circumcision bleeding. Male circumcision in Nigeria is the rule rather than the exception. Male siblings of some of the Nigerian haemophiliacs consequently died from severe post-circumcision bleeding. The aim was to determine the incidence of inherited factor VIIIc (FVIIIc) deficiencies in live male infants undergoing circumcision in South West, Nigeria. The study population was 244 male infants drawn from University College Hospital and Our Lady of Apostles Catholic Hospital, Oluyoro, Ibadan. Pre-circumcision prothrombin time, activated partial thromboplastin time and FVIIIc levels were determined. Clinical features of inherited bleeding disorder particularly family history of bleeding diathesis, history of cephalhaematoma and bleeding from the umbilical stump in neonatal life were determined with the aid of a questionnaire. Only one of the mothers (0.4%) gave a family history of bleeding disorder. A history of excessive bleeding from the umbilical stump post delivery was obtained in three (2%) of the patients. Five (2%) other subjects had cephalhaematoma post delivery. Two of the subjects (0.8%) had prolonged activated partial thromboplastin time. The factor VIIIc level was between 31% and 49% in 16.1%, while 1.6% of the neonates had levels between 20% and 26%. This study detected four of the 244 (1.64%) neonates with FVIIIc deficiency, suggestive of either mild haemophilia or von Willebrand's disease. A larger study (including family studies) will be required, so as to arrive at the exact incidence of both haemophilia A and vWD in live male infants in Nigeria.

摘要

新生儿男性遗传性出血性疾病的一个突出证据是包皮环切术后出血过多。在尼日利亚,男性包皮环切术是惯例而非例外。因此,一些尼日利亚血友病患者的男性同胞死于严重的包皮环切术后出血。目的是确定在尼日利亚西南部接受包皮环切术的存活男婴中遗传性凝血因子VIIIc(FVIIIc)缺乏症的发病率。研究人群为从伊巴丹奥卢约罗的大学学院医院和使徒圣母天主教医院抽取的244名男婴。测定了包皮环切术前的凝血酶原时间、活化部分凝血活酶时间和FVIIIc水平。借助问卷确定遗传性出血性疾病的临床特征,特别是出血素质的家族史、头颅血肿病史和新生儿期脐带残端出血情况。只有一位母亲(0.4%)有出血性疾病家族史。三名患者(2%)有产后脐带残端出血过多的病史。另外五名受试者(2%)产后有头颅血肿。两名受试者(0.8%)活化部分凝血活酶时间延长。16.1%的新生儿FVIIIc水平在31%至49%之间,而1.6%的新生儿水平在20%至26%之间。本研究在244名新生儿中检测到4例(1.64%)FVIIIc缺乏,提示可能为轻度血友病或血管性血友病。需要进行更大规模的研究(包括家族研究),以便得出尼日利亚存活男婴中甲型血友病和血管性血友病的确切发病率。

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