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环氧化酶2和肝静脉血流在遗传性出血性毛细血管扩张症或肝肺综合征患者中的作用。

Roles of cyclooxygenase 2 and hepatic venous flow in patients with HHT or hepatopulmonary syndrome.

作者信息

Lacout Alexis, Marcy Pierre Yves, Thariat Juliette, Sellier Jacques, El Hajjam Mostafa, Lacombe Pascal

机构信息

Pluridisciplinary HHT team, Ambroise Paré Hospital, Groupement des Hôpitaux Ile-de-France Ouest, Assistance Publique Hôpitaux de Paris, Université de Versailles Saint Quentin en Yvelines, 9, Avenue Charles de GAULLE, 92100 Boulogne Billancourt, France.

Pluridisciplinary HHT team, Ambroise Paré Hospital, Groupement des Hôpitaux Ile-de-France Ouest, Assistance Publique Hôpitaux de Paris, Université de Versailles Saint Quentin en Yvelines, 9, Avenue Charles de GAULLE, 92100 Boulogne Billancourt, France.

出版信息

Med Hypotheses. 2014 Sep;83(3):302-5. doi: 10.1016/j.mehy.2014.06.001. Epub 2014 Jun 14.

DOI:10.1016/j.mehy.2014.06.001
PMID:24986705
Abstract

BACKGROUND

Hereditary hemorrhagic telangiectasia (HHT) and hepatopulmonary syndrome are disorders characterized by the development of multiple pulmonary arteriovenous malformations (PAVM).

PRESENTATION OF THE HYPOTHESIS

COX2 may be at the origin of a cascade of pro inflammatory events to favour angiogenesis and PAVM development.

TESTING THE HYPOTHESIS

HHT and hepatopulmonary syndrome mouse models may be used to show its effects on PAVM formation. Anti COX-2 therapy could also be tested in human individuals, particularly in patients presenting a hepatopulmonary syndrome or HHT with small PAVM.

IMPLICATION OF THE HYPOTHESIS

PAVMs are one of the main causes of morbidity in patients presenting with HHT disease, owing to the risks of rupture as well as paradoxical embolism exposing to stroke and/or cerebral abscess. Percutaneous embolization has become the treatment of choice of PAVM. Anti COX2 may prevent from PAVM development and subsequent related complications and avoid either surgery and/or percutaneous embolization and thus subsequent related complication.

摘要

背景

遗传性出血性毛细血管扩张症(HHT)和肝肺综合征是以多发性肺动静脉畸形(PAVM)形成为特征的疾病。

假说提出

COX2可能是一系列促炎事件的起源,有利于血管生成和PAVM的发展。

假说验证

HHT和肝肺综合征小鼠模型可用于显示其对PAVM形成的影响。抗COX-2疗法也可在人类个体中进行测试,特别是在患有肝肺综合征或伴有小PAVM的HHT患者中。

假说意义

由于破裂风险以及反常栓塞导致中风和/或脑脓肿的风险,PAVM是HHT疾病患者发病的主要原因之一。经皮栓塞已成为PAVM的首选治疗方法。抗COX2可能预防PAVM的发展及随后的相关并发症,避免手术和/或经皮栓塞以及随后的相关并发症。

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