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OA北大核心CSTPCD
Emphasis on pulmonary vascular complications in children with a long-term survival after hepatoportal jejunostomy for biliary atresia
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.
In children with a long-term survival after hepato-jejunostomy for biliary atresia(BA),chro-nic liver cirrhosis leads to hepatopulmonary syndrome(HPS)and portopulmonary hypertension(PoPH),cau-sing 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 di-agnosis,rational selection of medication and proper timing of liver transplantation require multidisciplinary col-laborations.
陈功;张杰
复旦大学附属儿科医院普外科,上海 201102
胆道闭锁外科手术手术后并发症肝肺综合征肺动脉高压
Biliary AtresiaSurgical Procedures,OperativePostoperative ComplicationsHepatopul-monary SyndromePulmonary Arterial Hypertension
《临床小儿外科杂志》 2024 (008)
706-710 / 5
上海市自然科学基金(19ZR1406600) Shanghai Municipal Natural Science Foundation(19ZR1406600)
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