先天性毛细血管肺泡发育不良所致新生儿持续性肺动脉高压

Persistent pulmonary hypertension of newborn due to congenital capillary alveolar dysplasia.

作者信息

Singh Soraisham Amuchou, Ibrahim Thowfique, Clark Deborah Jane, Taylor Richard Scott, George David Hilles

机构信息

Division of Neonatology, Department of Pediatrics, Foothills Medical Centre, University of Calgary, Calgary, Alberta, Canada.

出版信息

Pediatr Pulmonol. 2005 Oct;40(4):349-53. doi: 10.1002/ppul.20244.

DOI:10.1002/ppul.20244

PMID:15957181

Abstract

Congenital alveolar capillary dysplasia is a rare and fatal cause of pulmonary hypertension in neonates. We report on a term baby with severe pulmonary hypertension unresponsive to high-frequency ventilation and nitric oxide. A diagnosis of alveolar capillary dysplasia was established on autopsy. We review the literature regarding pathophysiology, clinical presentations, associated malformations, and treatment trials.

摘要

先天性肺泡毛细血管发育不良是新生儿肺动脉高压的一种罕见且致命的病因。我们报告一例足月儿，患有严重肺动脉高压，对高频通气和一氧化氮治疗无反应。尸检确诊为肺泡毛细血管发育不良。我们回顾了有关病理生理学、临床表现、相关畸形及治疗试验的文献。