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比利时的家族性克罗恩病:家系、病例间的时间关系及家族病史

Familial Crohn's disease in Belgium: pedigrees, temporal relationships among cases, and family histories.

作者信息

Van Kruiningen Herbert J, Joossens Marie, Vermeire Severine, Joossens Sofie, Debeugny Stéphane, Gower-Rousseau Corinne, Cortot Antoine, Colombel Jean-Frédéric, Rutgeerts Paul, Vlietinck Robert

机构信息

Department of Pathobiology and Veterinary Science, University of Connecticut, Storrs, CT 06269, USA.

出版信息

J Clin Gastroenterol. 2007 Jul;41(6):583-90. doi: 10.1097/MCG.0b013e3180644d3c.

Abstract

BACKGROUND

Recently we published an analysis of environmental factors in familial Crohn's disease (CD) in Belgium. The aim of the current study was to assess pedigrees and sibships, temporal relationships among cases, and family circumstances relevant to the frequency or onset of CD.

STUDY

Twenty-one families with 3 or more affected first-degree relatives were studied. Seventy-four patients with CD and 84 unaffected family members were interviewed together at the parental home, with the aid of a 176 item questionnaire. Pedigrees were constructed establishing which family members had the disease and their relationships within sibships. Dates of onset of disease, validation of first symptoms and circumstances potentially relevant to the onset and distribution of disease within families were among the data documented during the interviews. Sequence of disease within families, consecutive versus nonconsecutive sequence of disease within sibships, and temporal relationships among cases were tabulated.

RESULTS

In 12 of the 21 families CD occurred in a parent before CD in any children. Five affected fathers preceded 9 affected children; 7 affected mothers preceded 10 affected children. First borns were affected more frequently. Within sibships there were 21 instances (36%) when an affected sibling was consecutive in birth order with an affected sibling. When a parent had CD before the birth of the first child the "exposure interval" to CD in the children was longer (mean 22.4 y) than when the parent developed CD after the child was born (mean 11.8 y).

CONCLUSIONS

The clusterings of CD within sibships and in time suggest that there is a contagious element in the etiology of CD.

摘要

背景

最近我们发表了一篇关于比利时家族性克罗恩病(CD)环境因素的分析。本研究的目的是评估家系和同胞关系、病例之间的时间关系以及与CD发生频率或发病相关的家庭情况。

研究

对21个有3名或更多受影响的一级亲属的家庭进行了研究。借助一份包含176个条目的问卷,在父母家中对74例CD患者和84名未受影响的家庭成员进行了访谈。构建家系图以确定哪些家庭成员患有该病以及他们在同胞关系中的亲属关系。访谈记录的数据包括疾病发病日期、首发症状的确认以及可能与家庭中疾病的发生和分布相关的情况。列出了家庭内疾病的顺序、同胞关系中疾病的连续与非连续顺序以及病例之间的时间关系。

结果

在21个家庭中的12个家庭中,CD在任何孩子发病之前先在父母一方出现。5名患病父亲先于9名患病孩子;7名患病母亲先于10名患病孩子。头胎孩子受影响的频率更高。在同胞关系中,有21例(36%)患病同胞在出生顺序上是连续的。当父母在第一个孩子出生前患有CD时,孩子接触CD的“暴露间隔”(平均22.4年)比父母在孩子出生后患CD时更长(平均11.8年)。

结论

CD在同胞关系中和时间上的聚集表明CD的病因中存在传染性因素。

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