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威尔逊-米基蒂综合征患儿的长期随访

Long-term follow up of children with the Wilson-Mikity syndrome.

作者信息

Rivlin J, Pape K, Reilly B J, Smallhorn J F, Levison H

出版信息

Eur J Respir Dis. 1985 Oct;67(4):286-93.

PMID:4076367
Abstract

Nine children who survived the development of Wilson-Mikity (W-M) syndrome were studied at a mean age of 7.6 +/- 0.3 years. Persistent respiratory symptoms during the year preceding the study were present in one child. Pulmonary function studies demonstrated a significant degree of airway obstruction in 3 children. Positive methacholine challenge was observed in 6 children indicating bronchial hyperreactivity. Abnormal chest radiographs were present in 3 children. One showed the characteristic appearances of asthma with air trapping, bronchial wall thickening and redistribution of pulmonary vessels. The other child showed minimal residual peribronchial thickening and the third child had normal lungs, but a rather prominent pulmonary artery segment. Echocardiographic studies revealed enlarged right ventricular cavity in 2 children. However, the pre-ejection period and the systolic time intervals were all within normal limits. It appears that unlike patients with bronchopulmonary dysplasia survivors of W-M syndrome have a good prognosis.

摘要

对9名存活下来的患威尔逊-米基蒂(W-M)综合征的儿童进行了研究,他们的平均年龄为7.6±0.3岁。在研究前一年,有1名儿童存在持续性呼吸道症状。肺功能研究显示3名儿童有明显程度的气道阻塞。6名儿童乙酰甲胆碱激发试验呈阳性,表明存在支气管高反应性。3名儿童胸部X线片异常。1名儿童表现出哮喘的典型表现,有气体潴留、支气管壁增厚和肺血管重新分布。另一名儿童显示支气管周围残留轻度增厚,第三名儿童肺部正常,但肺动脉段较为突出。超声心动图研究显示2名儿童右心室腔扩大。然而,射血前期和收缩期时间间期均在正常范围内。看来,与支气管肺发育不良患者不同,W-M综合征幸存者预后良好。

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Long-term follow up of children with the Wilson-Mikity syndrome.威尔逊-米基蒂综合征患儿的长期随访
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