De Vries T W, Weening J J, Roorda R J
Dept of Paediatrics, University Hospital Groningen, The Netherlands.
Eur Respir J. 1991 Sep;4(8):1029-32.
We describe the observation of a 12 yr old girl who died of pulmonary veno-occlusive disease (PVOD). Diagnosis was based on histological examination of an open lung biopsy. The differential diagnosis, pathogenesis and possible therapies are discussed. Although medical therapy can sometimes give some temporary relief, lung transplantation might offer these patients a better chance of survival and a better quality of life.
我们描述了一名12岁死于肺静脉闭塞性疾病(PVOD)女孩的观察情况。诊断基于开放性肺活检的组织学检查。讨论了鉴别诊断、发病机制和可能的治疗方法。尽管药物治疗有时能带来一些暂时缓解,但肺移植可能为这些患者提供更好的生存机会和更高的生活质量。