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肺毛细血管血管瘤病合并 CREST 综合征 1 例报告并文献复习

Pulmonary capillary hemangiomatosis associated with CREST syndrome: a case report and review of the literature.

机构信息

University of South Carolina School of Medicine, 8 Medical Park, Columbia, SC 29203, USA.

出版信息

Respiration. 2010;80(5):435-8. doi: 10.1159/000314587. Epub 2010 May 6.

DOI:10.1159/000314587
PMID:20453487
Abstract

This is a report of fatality immediately after administration of epoprostenol. The patient was previously diagnosed with CREST syndrome and associated interstitial lung disease. She developed worsening pulmonary hypertension and was clinically diagnosed with pulmonary veno-occlusive disease. The patient developed flash pulmonary edema and arrested after administration of low-dose epoprostenol in the intensive care unit. An autopsy revealed the patient suffered from pulmonary capillary hemangiomatosis. We review our case and what is known about this rare disease.

摘要

这是一例伊洛前列素给药后即刻死亡的报告。患者此前被诊断为 CREST 综合征及相关间质性肺病。她的肺动脉高压恶化,并被临床诊断为肺静脉闭塞性疾病。患者在重症监护病房接受低剂量伊洛前列素治疗后出现暴发性肺水肿并心脏骤停。尸检显示患者患有肺毛细血管血管瘤病。我们回顾了我们的病例,并讨论了这种罕见疾病。

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