کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
8601210 1565458 2018 11 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
La maladie veino-occlusive pulmonaire
ترجمه فارسی عنوان
بیماری انسدادی ورید ریوی
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی کاردیولوژی و پزشکی قلب و عروق
چکیده انگلیسی
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding. A noninvasive diagnostic approach, including oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest, is used to support a diagnosis of PVOD. PVOD prognosis is worse than other forms of PAH. There is no evidence-based medical therapy for PVOD and life-threatening pulmonary edema may occur following PAH targeted therapy in PVOD. Lung transplantation remains the preferred definitive therapy for eligible patients.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Revue des Maladies Respiratoires - Volume 35, Issue 2, February 2018, Pages 160-170
نویسندگان
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