Suppr超能文献

[QT综合征。四代人的家族研究]

[The QT syndrome. A family study of 4 generations].

作者信息

Franek A, Böcker H, Busam A

机构信息

Kinderklinik des Marienhospitals, Bottrop.

出版信息

Monatsschr Kinderheilkd. 1989 Jul;137(7):411-4.

PMID:2797010
Abstract

Starting from two related children diagnosed with a syndrome of QT prolongation (Romano-Ward) a nearly complete family history was revealed in that 8 further patients with a QT-time over 115% of normal were found. Four additional family members had died of a sudden syncope at a young age, 2 of them in early childhood. No less than 6 sudden infant or early childhood deaths without clear cause were found in one generation of 12 members. This demonstrates the urgent necessity to examine the whole family with this syndrome in as much detail as possible. Quite often these syncopes are wrongly diagnosed as epileptic seizure. The treatment with beta-blocking medication very much improves the bad prognosis. This treatment should also include family members with QT-prolongation sofar without any symptoms.

摘要

从两名被诊断患有QT间期延长综合征(罗曼诺-沃德综合征)的相关儿童入手,发现了一份近乎完整的家族病史,其中还发现另外8名患者的QT时间超过正常时间的115%。另外有4名家庭成员在年轻时死于突然晕厥,其中2人在幼儿期。在一个12名成员的家族一代中,发现不少于6例原因不明的婴儿或幼儿期猝死。这表明迫切需要尽可能详细地对患有该综合征的整个家族进行检查。这些晕厥常常被误诊为癫痫发作。使用β受体阻滞剂药物治疗可大大改善不良预后。这种治疗还应包括目前尚无任何症状的QT间期延长的家庭成员。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验