Chen Gong,Zhang Jie.Emphasis on pulmonary vascular complications in children with a long-term survival after hepatoportal jejunostomy for biliary atresia[J].Journal of Clinical Pediatric Surgery,2024,(08):706-710.[doi:10.3760/cma.j.cn101785-202406012-002]
重视胆道闭锁肝门空肠吻合术后长期存活患儿肺血管并发症
- Title:
- Emphasis on pulmonary vascular complications in children with a long-term survival after hepatoportal jejunostomy for biliary atresia
- Keywords:
- Biliary Atresia; Surgical Procedures; Operative; Postoperative Complications; Hepatopulmonary Syndrome; Pulmonary Arterial Hypertension
- 摘要:
- 在胆道闭锁(biliary atresia,BA)肝门空肠吻合术后长期存活的患儿中,有相当一部分人可能存在肝纤维化相关肺血管并发症,即慢性肝硬化导致的肝肺综合征(hepatopulmonary syndrome,HPS)及门静脉性肺动脉高压(portopulmonary hypertension,PoPH),可引起呼吸、循环系统症状,此类并发症在BA患儿中的发生率较其他儿童慢性肝病更高,进展更快,严重时危及患儿生命。HPS和PoPH可共存于同一患儿。提高对于HPS及PoPH的认知,早期诊断,合理选择药物治疗及评估肝移植时机,需要多学科共同协作。
- Abstract:
- In children with a long-term survival after hepato-jejunostomy for biliary atresia (BA),chronic liver cirrhosis leads to hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH),causing diverse respiratory and circulatory system symptoms.The incidence of such complications in BA children is higher than that of other chronic liver diseases.With a faster progression,it endangers life in some severe cases.HPS and PoPH may coexist in the same child.Improving the understanding of HPS/PoPH,making an early diagnosis,rational selection of medication and proper timing of liver transplantation require multidisciplinary collaborations.
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备注/Memo
收稿日期:2024-6-6。
基金项目:上海市自然科学基金 (19ZR1406600)
通讯作者:陈功,Email:chengongzlp@hotmail.com