Lengthy Diagnostic Challenge In A Rare Case Of Pulmonary Veno-Occlusive Disease: Case Report And Review Of The Literature
INTERNAL MEDICINE(2011)
摘要
Pulmonary veno-occlusive disease (PVOD) is a rare and usually survival poor disorder. We report a patient with a long history of progressive dyspnea of over 8 years, who with a diagnosis of chronic cor pulmonale confirmed elsewhere, was ultimately diagnosed as PVOD via histological analysis of a lung biopsy. After treatment with combined bosentan, diuretics and digoxin, his symptoms and function improved. This case highlights that PVOD is an under-recognised and often misdiagnosed disease, especially in its chronic form. Understanding its pathogenesis, its poor response to medical therapy and its dismal prognosis remain challenges for the treatment of PVOD.
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关键词
pulmonary hypertension, pulmonary veno-occlusive disease, lung biopsy, bosentan
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