肺不发育及相关肺动脉高压：一例报告及关于变异性、治疗和结局的综述
Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome.
作者信息
Muensterer Oliver, Abellar Rosanna, Otterburn David, Mathew Rajamma
机构信息
Department of Pediatric Surgery, University Medicine Mainz, Johannes Gutenberg University, Mainz, Germany.
Department of Pathology, Columbia University, New York, New York, United States.
出版信息
European J Pediatr Surg Rep. 2015 Jun;3(1):33-9. doi: 10.1055/s-0034-1395986. Epub 2015 Jan 8.
Pulmonary agenesis is a rare congenital disorder with large variability in presentation and prognosis. We describe a full-term infant born with right-sided pulmonary agenesis who underwent thoracoscopic placement of a tissue expander. He ultimately died of pulmonary hypertension. Immunohistology showed intimal hyperplasia without the loss of endothelial caveolin-1 expression. A literature review revealed that while some of these patients have favorable outcome, many succumb despite therapy.
肺不发育是一种罕见的先天性疾病，其临床表现和预后差异很大。我们描述了一名足月出生的患有右侧肺不发育的婴儿，该婴儿接受了胸腔镜下组织扩张器置入术。他最终死于肺动脉高压。免疫组织学检查显示内膜增生，但内皮小窝蛋白-1表达未丧失。文献综述表明，虽然这些患者中有一些预后良好，但许多患者尽管接受了治疗仍会死亡。
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