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地中海地区儿童内脏静脉血栓形成。

Splanchnic vein thrombosis in the mediterranean area in children.

机构信息

The Department of Pediatrics, Cairo University, Egypt.

出版信息

Mediterr J Hematol Infect Dis. 2011;3(1):e2011027. doi: 10.4084/MJHID.2011.027. Epub 2011 Jul 8.

Abstract

Abdominal venous thrombosis may present as splanchnic venous thrombosis (SVT) (occlusion of portal, splenic, superior or inferior mesenteric veins) or Budd- Chiari Syndrome (BCS) (thrombosis of inferior vena cava and/or hepatic veins). The aim of this review is to report the scanty data available for SVT in the South Mediterranean area. In one Egyptian study, the possible circumstantial risk factors for portal vein thrombosis (PVT) were found in 30% of cases: 19% neonatal sepsis, 8.7% umbilical catheterization, 6% severe gastroenteritis and dehydration. Another Egyptian study concluded that hereditary thrombophilia was common in children with PVT (62.5%), the commonest being factor V Leiden mutation (FVL) (30%). Concurrence of more than one hereditary thrombophilia was not uncommon (12.5%). The first international publication on hepatic veno-occlusive disease (VOD) in Egypt was in 1965 in children who rapidly develop abdominal distention with ascites and hepatomegaly. This disease was more frequent in malnourished children coming from rural areas; infusions given at home may contain noxious substances that were hepatotoxic and infections might play a role. VOD of childhood is rarely seen nowadays. Data from South Mediterranean area are deficient and this may be attributable to reporting in local medical journals that are difficult to access. Medical societies concerned with this topic could help distribute this information.

摘要

腹部静脉血栓形成可能表现为内脏静脉血栓形成(SVT)(门静脉、脾静脉、肠系膜上静脉闭塞)或巴德-奇阿里综合征(BCS)(下腔静脉和/或肝静脉血栓形成)。本综述的目的是报告南地中海地区 SVT 可用的少量数据。在一项埃及研究中,门静脉血栓形成(PVT)的可能环境危险因素在 30%的病例中被发现:19%新生儿败血症、8.7%脐导管插入术、6%严重胃肠炎和脱水。另一项埃及研究得出结论,遗传性血栓形成倾向在患有 PVT 的儿童中很常见(62.5%),最常见的是因子 V 莱顿突变(FVL)(30%)。同时存在多种遗传性血栓形成倾向并不罕见(12.5%)。埃及关于肝静脉闭塞性疾病(VOD)的第一篇国际出版物发表于 1965 年,涉及迅速出现腹水和肝肿大的儿童。这种疾病在来自农村地区营养不良的儿童中更为常见;在家中给予的输液可能含有有毒物质,具有肝毒性,感染可能起作用。现在很少见到儿童 VOD。来自南地中海地区的数据不足,这可能归因于在难以获取的当地医学期刊上报告。关注该主题的医学协会可以帮助传播这些信息。

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