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一项关于热性惊厥的流行病学研究,特别涉及家族史和HLA连锁关系。

An epidemiological study of febrile seizures with special reference to family history and HLA linkage.

作者信息

Wadhwa N, Bharucha B, Chablani U, Contractor N

机构信息

Department of Pediatrics, K.E.M. Hospital, Parel, Bombay.

出版信息

Indian Pediatr. 1992 Dec;29(12):1479-85.

PMID:1291492
Abstract

One hundred and forty four cases of febrile seizures, 95 simple (typical) and 49 complex (atypical); were studied and compared for clinical and epidemiological data and family history of febrile and afebrile seizures. Major results were: maximum age of onset below three years (75%) in both simple and complex groups, male preponderance, respiratory infection as the commonest etiology (69.4%) and maximum seizure onset within 24 hours of fever (73%). The familial prevalence of all seizures was 29.1%, 23.2% in the simple and 40.8% in the complex group (p < 0.01). The familial prevalence of febrile seizures was 20%; similar in both groups. The familial prevalence of afebrile seizures was 13.9%; 6.3% in simple and 28.6% in complex group (p < 0.01). The commonest relative was a sibling (13.2%). The prevalence in parents was 4%. Families with two additional members with history of seizures revealed complex seizure patterns in two-thirds of index cases. There was no correlation between family history of seizures and age at onset or sex. No clear inheritance pattern emerged and polygenic inheritance is likely. One third of eighteen families had siblings with identical segregation of parental HLA-A and B haplotypes. Five families showed the presence of HLA All. This small though adequate sample size did not reveal an HLA marker for febrile seizures.

摘要

对144例热性惊厥患者进行了研究,其中95例为单纯(典型)热性惊厥,49例为复杂(非典型)热性惊厥;比较了其临床、流行病学数据以及热性和无热惊厥的家族史。主要结果如下:单纯组和复杂组的最大发病年龄均低于3岁(75%),男性居多,呼吸道感染是最常见的病因(69.4%),且大多数惊厥在发热24小时内发作(73%)。所有惊厥的家族患病率为29.1%,单纯组为23.2%,复杂组为40.8%(p<0.01)。热性惊厥的家族患病率为20%;两组相似。无热惊厥的家族患病率为13.9%;单纯组为6.3%,复杂组为28.6%(p<0.01)。最常见的亲属是兄弟姐妹(13.2%)。父母的患病率为4%。有另外两名成员有惊厥病史的家庭中,三分之二的索引病例表现为复杂惊厥模式。惊厥家族史与发病年龄或性别之间无相关性。未出现明确的遗传模式,可能为多基因遗传。18个家庭中有三分之一的家庭,其兄弟姐妹的父母HLA - A和B单倍型呈相同分离。5个家庭显示存在HLA All。尽管样本量小但足够,未发现热性惊厥的HLA标记物。

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