Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism.

作者: N. Samuels , N. Berkman , E. Milgalter , J. Bar-Ziv , G. Amir

DOI: 10.1136/THX.54.9.858

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摘要: Neurofibromatosis has been known to involve blood vessels throughout the body. Pulmonary involvement with interstitial fibrosing alveolitis described but no case of pulmonary vascular reported date. A 51 year old patient cutaneous neurofibromatosis is who presented severe hypertension and radiographic, scintigraphic, angiographic evidence chronic thromboembolic hypertension. Severe intimal fibrosis consistent was found on endarterectomy thromboembolism. arteries should be considered as a possible cause

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