Olin James, Abman Steven, Grady R, Ivy D, Lovell Mark, Partrick David, Balasubramaniam Vivek
Department of Pediatrics, University of Colorado, Aurora, Colorado, USA.
BMJ Case Rep. 2011;2011:bcr0220113843. doi: 10.1136/bcr.02.2011.3843. Epub 2011 Apr 20.
The authors discuss the case of a 7-year-old female who presented with exertional cyanosis and was found to have pulmonary arterial hypertension. Despite normal left-sided heart function, the patient developed pulmonary oedema in response to pulmonary vasodilator therapy, increasing suspicion for pathology in the pulmonary capillaries and veins. Lung biopsy confirmed a diagnosis of pulmonary capillary haemangiomatosis (PCH), a rare cause of pulmonary hypertension in both children and adults. The diagnosis requires lung biopsy and is often made postmortem. She was treated with interferon α-2a and doxycycline for their antiangiogenic properties and reports of disease regression. Although she initially demonstrated improvement in her pulmonary hypertension in response to these medications, she succumbed to the disease within the time frame previously reported for PCH.
作者讨论了一名7岁女性的病例，该患者出现劳力性发绀，被发现患有肺动脉高压。尽管左侧心功能正常，但患者在接受肺血管扩张剂治疗后出现肺水肿，这增加了对肺毛细血管和静脉病变的怀疑。肺活检确诊为肺毛细血管瘤病（PCH），这是儿童和成人肺动脉高压的一种罕见病因。该诊断需要肺活检，且通常在尸检后才能做出。她接受了具有抗血管生成特性的干扰素α-2a和强力霉素治疗，且有疾病消退的报道。尽管她最初在使用这些药物后肺动脉高压有所改善，但她仍在先前报道的PCH病程时间内死于该病。
