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家族性溶血尿毒综合征——阿根廷的经验

Hemolytic uremic syndrome in families-an Argentinian experience.

作者信息

Voyer L E, Wainsztein R E, Quadri B E, Corti S E

机构信息

Nephrology Unit, Hospital General de Niños Pedro de Elizalde, Buenos Aires, Argentina.

出版信息

Pediatr Nephrol. 1996 Feb;10(1):70-2. doi: 10.1007/BF00863451.

Abstract

Six hundred and thirty-one patients with hemolytic uremic syndrome (HUS) were treated from 1960 to 1992; 19 (3%) were familial cases, of which 9 were classified as concomitant (including twins), 6 as non-concomitant, and 4 as recurrent. In the recurrent group there were 15 HUS episodes, 10 being concomitant in 2 patients. Prodromal diarrhea was present only in concomitant and non-concomitant cases. Patients with recurrences were sisters from a single family. Concomitant and non-concomitant cases had clinical features, course, and age similar to typical endemoepidemic forms of HUS, in which an association with verocytotoxin-producing Escherichia coli has been reported. There may be a genetic determinant in concomitant cases; these occurred outside the season during which endemoepidemic forms are typically detected. In patients with recurrent disease a genetic factor which may lead to the development of the disease when triggered by viral infections is likely.

摘要

1960年至1992年期间,共治疗了631例溶血性尿毒症综合征(HUS)患者;其中19例(3%)为家族性病例,其中9例被归类为伴发性(包括双胞胎),6例为非伴发性,4例为复发性。在复发性组中,有15次HUS发作,其中10次发生在2名患者身上,为伴发性发作。前驱性腹泻仅出现在伴发性和非伴发性病例中。复发患者为来自单个家庭的姐妹。伴发性和非伴发性病例的临床特征、病程和年龄与典型的地方性流行形式的HUS相似,据报道后者与产志贺毒素大肠杆菌有关。伴发性病例可能存在遗传决定因素;这些病例发生在通常检测到地方性流行形式的季节之外。在复发性疾病患者中,可能存在一种遗传因素,当受到病毒感染触发时,可能导致疾病的发生。

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