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Sarcoidosis Vasc Diffuse Lung Dis ; 33(2): 182-5, 2016 Aug 01.
Article in English | MEDLINE | ID: mdl-27537724

ABSTRACT

Pulmonary arterial hypertension is a progressive life-threatening disease characterized by vascular remodeling. There is evidence that varied immune mechanism play an important role in progression of pulmonary hypertension.  We describe a case of a 35-year-old woman with idiopathic pulmonary arterial hypertension (IPAH) and a novel BMPR2 mutation, who underwent a successful lung transplantation.  Extensive granulomatous inflammation was seen in the resected lungs. The granulomatous inflammation found in the histology supports  a sarcoid-like reaction due to pulmonary hypertension in the context of the BMPR2 mutation.


Subject(s)
Bone Morphogenetic Protein Receptors, Type II/genetics , Familial Primary Pulmonary Hypertension/genetics , Mutation , Sarcoidosis, Pulmonary/etiology , Adult , Biopsy , Familial Primary Pulmonary Hypertension/complications , Familial Primary Pulmonary Hypertension/diagnosis , Familial Primary Pulmonary Hypertension/surgery , Female , Genetic Predisposition to Disease , Humans , Lung Transplantation , Sarcoidosis, Pulmonary/diagnosis
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