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[唐氏综合征中的肺发育不全与肺动脉高压:采用肺活检的预后评估]

[Hypoplasia and pulmonary hypertension in Down syndrome: prognostic evaluation using pulmonary biopsy].

作者信息

Tarroch X, Rovirosa M, Toran N, Lozano C

出版信息

Rev Esp Cardiol. 1989 May;42(5):348-50.

PMID:2528192
Abstract

The pulmonary hypoplasia, in Down's syndrome with congenital cardiac malformation, probably explains the poor behaviour of these patients, leading to early vascular pulmonary lesions, which progress quickly. We present a case, in which the lung biopsy, done before cardiac surgery, showed a lung hypoplasia and also enabled us to establish the grading of the pulmonary vascular disease. The pathologic evaluation is necessary to decide upon the intracardiac repair and to predict the child's outcome.

摘要

在患有先天性心脏畸形的唐氏综合征患者中,肺发育不全可能解释了这些患者的不良表现,导致早期肺部血管病变,并迅速进展。我们报告一例,在心脏手术前进行的肺活检显示肺发育不全,同时也使我们能够确定肺血管疾病的分级。病理评估对于决定心脏内修复以及预测患儿的预后是必要的。

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