一名慢性肝病患者出现的严重低氧血症相关性肺内分流：经药物治疗后改善。

Severe hypoxemia-associated intrapulmonary shunt in a patient with chronic liver disease: improvement after medical treatment.

作者信息

Cadranel J L, Milleron B J, Cadranel J F, Fermand J P, Andrivet P, Brouet J C, Adnot S, Akoun G M

机构信息

Service de Pneumologie et de Réanimation Respiratoire, Hôpital Tenon, Paris, France.

出版信息

Am Rev Respir Dis. 1992 Aug;146(2):526-7. doi: 10.1164/ajrccm/146.2.526.

DOI:10.1164/ajrccm/146.2.526

PMID:1489152

Abstract

A 20-yr-old woman with chronic liver disease and angioimmunoblastic lymphadenopathy presented with marked hypoxemia caused by intrapulmonary shunt. Her respiratory tract showed her to be free of angioimmunoblastic lymphadenopathy manifestations. After 12 months of treatment with cyclophosphamide and corticosteroid, the immunologic disease disappeared. Unexpectedly, hypoxia-associated intrapulmonary shunt was no longer present either. To the best of our knowledge, this is the first case of dramatic improvement, with medical therapy, of severe hypoxemia related to noncirrhotic liver disease. However, the mechanism by which this treatment caused the regression of intrapulmonary shunt is unknown.

摘要

一名患有慢性肝病和血管免疫母细胞性淋巴结病的20岁女性，因肺内分流导致明显低氧血症。她的呼吸道未出现血管免疫母细胞性淋巴结病的表现。在用环磷酰胺和皮质类固醇治疗12个月后，免疫性疾病消失。出乎意料的是，与缺氧相关的肺内分流也不再存在。据我们所知，这是第一例通过药物治疗使与非肝硬化性肝病相关的严重低氧血症得到显著改善的病例。然而，这种治疗导致肺内分流消退的机制尚不清楚。