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家族性腹泻后(志贺毒素介导)溶血尿毒综合征的聚集性。

Clustering of post-diarrheal (Shiga toxin-mediated) hemolytic uremic syndrome in families.

作者信息

Siegler R L, Sherbotie J R, Denkers N D, Pavia A T

机构信息

Division of Nephrology and Hypertension, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, USA.

出版信息

Clin Nephrol. 2003 Aug;60(2):74-9. doi: 10.5414/cnp60074.

Abstract

AIMS

  1. To study the epidemiological and clinical features of Shiga toxin (Stx)-mediated (post-diarrheal) hemolytic uremic syndrome (HUS) occurring in more than 1 family member. 2. To compare familial with non-familial episodes, and concurrent familial with non-concurrent familial cases. 3. To determine the likelihood of Stx HUS occurring in a second family member.

METHODS

A retrospective review from January 1970 through September 2001 of families in whom Stx HUS occurred in more than 1 family member was conducted using a computerized HUS registry. It contains information on 373 episodes that occurred in 356 families from Utah and neighboring states. Cases were categorized as being either concurrent (i.e., occurring within a month of one another) or non-concurrent, and the study was limited to those with typical (post-diarrheal) episodes.

RESULTS

HUS occurred in 2 or more family members in 17 (4.8%) of the families in our registry. In 12 (3.4%) of these families episodes occurred with days to weeks of each other; in 5 families (1.4%) episodes were separated by intervals of several years. There were no statistically significant differences in demographic, seasonal, laboratory, clinical, or outcome variables between familial subsets (concurrent versus non-concurrent) or between familial and non-familial cases.

CONCLUSIONS

When a child is diagnosed with D+ HUS, there is an increased risk that a second family member will also develop HUS; most often within days to weeks (i.e., within a month), but in some cases episodes may be separated by intervals of years. Non-concurrent cases suggest common environmental risk factors, or perhaps a genetic predisposition. Concurrent cases suggest a common source of infection or person-to-person transmission; a genetic predisposition cannot be excluded. These observations suggest that siblings of an index case who develop diarrhea should be kept under close surveillance.

摘要

目的

  1. 研究志贺毒素(Stx)介导的(腹泻后)溶血尿毒综合征(HUS)在一个以上家庭成员中发生的流行病学和临床特征。2. 比较家族性发作与非家族性发作,以及同时发生的家族性病例与非同时发生的家族性病例。3. 确定第二个家庭成员发生Stx HUS的可能性。

方法

使用计算机化的HUS登记系统,对1970年1月至2001年9月期间有一个以上家庭成员发生Stx HUS的家庭进行回顾性研究。该登记系统包含来自犹他州及周边州356个家庭发生的373次发作的信息。病例分为同时发生(即彼此在一个月内发生)或非同时发生,且该研究仅限于典型(腹泻后)发作的病例。

结果

在我们的登记系统中,17个(4.8%)家庭中有2个或更多家庭成员发生了HUS。其中12个(3.4%)家庭的发作彼此间隔数天至数周;5个家庭(1.4%)的发作间隔数年。家族性子集(同时发生与非同时发生)之间或家族性病例与非家族性病例之间在人口统计学、季节、实验室、临床或结局变量方面无统计学显著差异。

结论

当一名儿童被诊断为D + HUS时,第二个家庭成员发生HUS的风险增加;大多数情况下在数天至数周内(即一个月内),但在某些情况下发作可能间隔数年。非同时发生的病例提示存在共同的环境危险因素,或者可能存在遗传易感性。同时发生的病例提示存在共同的感染源或人传人传播;不能排除遗传易感性。这些观察结果表明,发生腹泻的索引病例的兄弟姐妹应密切监测。

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