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溶血性尿毒症综合征的家族性发病情况。

Familial occurrence of the haemolytic uraemic syndrome.

作者信息

Hogewind B L, de la Rivière G B, van Es L A, Veltkamp J J

出版信息

Acta Med Scand. 1980;207(1-2):73-7. doi: 10.1111/j.0954-6820.1980.tb09679.x.

DOI:10.1111/j.0954-6820.1980.tb09679.x
PMID:7368978
Abstract

A family is described in which the haemolytic uraemic syndrome (HUS) occurred in two generations. Both juvenile and adult onset of this syndrome were observed in this family. Those affected were all women, three developed HUS in the postpartum period, one towards the end of pregnancy and one as a five-year old child. Because five cases were observed over a period of 16 years, exposure to the same infectious agent is highly unlikely. Although the transmission of a "dormant" virus cannot be excluded, the occurrence of HUS in two generations of one and the same family seems compatible with the hypothesis that susceptibility to the disease is transmitted as an autosomal dominant characteristic. This observation suggests a genetic influence on the development of HUS, possibly in conjunction with other factors, such as infectious agents, pregnancy and/or delivery.

摘要

本文描述了一个家族,其中两代人都出现了溶血性尿毒症综合征(HUS)。在这个家族中观察到了该综合征的青少年期和成年期发病情况。所有受影响的都是女性,其中三人在产后出现HUS,一人在妊娠末期发病,一人在五岁时发病。由于在16年的时间里观察到了5例病例,因此极不可能是接触了相同的感染源。虽然不能排除“潜伏”病毒的传播,但同一家庭两代人都出现HUS的情况似乎与疾病易感性作为常染色体显性特征遗传的假设相符。这一观察结果表明,遗传因素可能对HUS的发生发展有影响,可能与其他因素如感染源、妊娠和/或分娩共同作用。

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