pulmonary veno-occlusive disease

An extremely rare idiopathic condition that may affect children and young adults, which causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension.

Aetiology
Uncertain, possibly linked to bone marrow transplants, chemotherapy (e.g., carmustine), malignancy (especially lymphoproliferative—e.g., lymphoma), leukaemia, viral infection, systemic lupus erythematosus.

Clinical findings
Dyspnoea, exertional fatigue, syncope, haemoptysis.
Imaging
Prominent interstitial markings, Kerley B lines, pulmonary arterial dilatation.
Management
Temporary response with vasodilators, nifedipine, alpha-adrenergic blocker, prazosin, and immunosuppressants; definitive therapy requires lung transplantation.

Prognosis
Poor—most die in 2 years.

pulmonary veno-occlusive disease

An idiopathic condition of children/young adults that causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension Etiology Uncertain, possibly linked to BM transplantation, chemotherapy–eg, carmustine, malignancy, viral infection Clinical Nodular zones of congestion, edema, hemorrhage, hemosiderosis Radiology CXR reveal prominent interstitial markings, Kerley B lines, pulmonary artery dilatation Management Temporary response may occur with vasodilators, nifedipine, α-adrenergic blocker, prazosin Prognosis Poor, most die in 2 yrs

pulmonary veno-occlusive disease

A condition that may complicate organ transplantation rejection. It is marked by extensive occlusion of the small and medium-sized veins of the lung by loose, sparsely cellular, fibrous tissue. Some larger veins may be involved. This disease produces severe pulmonary venous hypertension.

单词	pulmonary veno-occlusive disease
释义	pulmonary veno-occlusive disease pulmonary veno-occlusive disease An extremely rare idiopathic condition that may affect children and young adults, which causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension. Aetiology Uncertain, possibly linked to bone marrow transplants, chemotherapy (e.g., carmustine), malignancy (especially lymphoproliferative—e.g., lymphoma), leukaemia, viral infection, systemic lupus erythematosus. Clinical findings Dyspnoea, exertional fatigue, syncope, haemoptysis. Imaging Prominent interstitial markings, Kerley B lines, pulmonary arterial dilatation. Management Temporary response with vasodilators, nifedipine, alpha-adrenergic blocker, prazosin, and immunosuppressants; definitive therapy requires lung transplantation. Prognosis Poor—most die in 2 years. pulmonary veno-occlusive disease An idiopathic condition of children/young adults that causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension Etiology Uncertain, possibly linked to BM transplantation, chemotherapy–eg, carmustine, malignancy, viral infection Clinical Nodular zones of congestion, edema, hemorrhage, hemosiderosis Radiology CXR reveal prominent interstitial markings, Kerley B lines, pulmonary artery dilatation Management Temporary response may occur with vasodilators, nifedipine, α-adrenergic blocker, prazosin Prognosis Poor, most die in 2 yrs pulmonary veno-occlusive disease A condition that may complicate organ transplantation rejection. It is marked by extensive occlusion of the small and medium-sized veins of the lung by loose, sparsely cellular, fibrous tissue. Some larger veins may be involved. This disease produces severe pulmonary venous hypertension. See PVOD See PVOD
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