ERCP联合健脾清胆汤治疗PH1儿童肝移植术后吻合口狭窄合并化脓性胆管炎伴胆汁瘤1例

ERCP combined with Jianpi Qingdan decoction was used in the treatment of anastomotic stenosis complicated with suppurative cholangitis with biloma in children with PH1 after liver transplantation

原发性1型高草酸尿症(primary hyperoxaluria type 1,PH1)是一种由于肝酶缺乏致草酸盐全身沉积的罕见遗传性疾病,目前一线治疗推荐肝肾联合移植。肝移植术后多并发吻合口狭窄、化脓性胆管炎等胆道并发症,严重影响患者的生存质量。内镜逆行胰胆管造影术是诊断化脓性胆管炎的“金标准”,同时能有效解除胆道梗阻,并根据胆汁药敏结果指导抗生素的应用。中医胆为六腑之一,“腑以通为用”,我科经验方健脾清胆汤能疏肝健脾、利湿退黄、清热通腑,有助于减轻胆汁淤积,恢复正常排泄,改善胆道炎症。现报道内镜逆行胰胆管造影术联合健脾清胆汤治疗PH1肝肾联合移植术后吻合口狭窄合并化脓性胆管炎伴胆汁瘤患儿1例,以期提高临床医师对该病的认识。

Primary hyperoxaluria type 1(PH1)is a rare genetic disorder caused by systemic oxalate deposition due to liver enzyme deficiency,and combined liver-kidney transplantation is currently recommended as first-line treatment.After liver transplantation,biliary complications such as anastomotic stenosis(AS)and suppurative cholangitis(SC)are often complicated,which significantly affects the quality of life of patients.Endoscopic retrograde cholangiopancreatography(ERCP)is the"gold standard"for the diagnosis of BC,and it is effective in relieving biliary obstruction and guiding the use of antibiotics based on bile susceptibility results.Gallbladder is one of the six Fu-organs in Chinese medicine,"the six hollow viscera keeping its unobstructed state",Jianpi Qingdan decoction can soothe the liver and fortify the spleen,disinhibit dampness and relieve jaundice,clear heat and remove obstruction of Fu-organs,help to reduce cholestasis,restore normal excretion,and improve biliary inflammation.In order to improve the understanding of clinicians about this disease,ERCP combined with Jianpi Qingdan decoction was reported in the treatment of a child with AS complicated with SC with biloma after PH1 liver-kidney transplantation.