Franchi-Abella Stéphanie, Fabre Monique, Mselati Emmanuel, De Marsillac Marise Elia, Bayari Mohamed, Pariente Danièle, Jacquemin Emmanuel, Bernard Olivier
Department of Pediatric Radiology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Department of Histopathology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
J Pediatr. 2014 Jul;165(1):190-193.e2. doi: 10.1016/j.jpeds.2014.03.025. Epub 2014 Apr 25.
Childhood obliterative portal venopathy presents at any age and may be genetic in origin. We report 48 children with obliterative portal venopathy, based on strict histologic criteria, investigated between 1972 and 2011. Diagnosis requires histology and is suggested by ultrasonography findings. Portal hypertension is the main complication but is absent in some cases. Prognosis is relatively good, but the detection of cardiopulmonary complications is essential.
儿童闭塞性门静脉病可在任何年龄出现，其病因可能为遗传性。我们报告了48例根据严格组织学标准诊断的闭塞性门静脉病患儿，这些患儿于1972年至2011年间接受了调查。诊断需要组织学检查，超声检查结果可提示诊断。门静脉高压是主要并发症，但在某些病例中并不存在。预后相对较好，但检测心肺并发症至关重要。
