尼曼匹克病肝移植术后成功妊娠分娩1例并文献复习

Successful pregnancy and delivery in a patient with Niemaoh-Pick disease after liver transplantation: report of one case and literature review

目的总结尼曼匹克病肝移植患者术后妊娠的临床经验。方法回顾性分析1例尼曼匹克病B型患者接受肝移植后成功妊娠分娩的临床资料。结果该例患者于2011年7月8日行肝移植术,同时行脾动脉结扎,手术及术后恢复顺利,术后常规抗排斥治疗,后逐渐改为单用他克莫司,剂量2.5 mg/d,血药浓度维持在2 ng/m L。2015年9月该患者成功妊娠,于2016年6月2日行剖宫产分娩一男婴。分娩后短期内少量母乳喂养,产妇及新生儿随访至投稿日,母亲正常,婴儿生长发育正常。结论尼曼匹克病患者肝移植术后妊娠分娩可取得良好结局。

Objective To summarize the clinical experience of patient diagnosed with Niemaoh-Pick disease being pregnant after liver transplantation. Methods Clinical data of one case of type B Niemaoh-Pick disease being pregnant after liver transplantation were retrospectively analyzed. Results The patient successfully underwent liver transplantation combined with splenic artery ligation on July 8, 2011. She was well recovered postoperatively. After surgery, she received conventional anti-rejection treatment, and gradually switched to use of tacrolimus at a dosage of 2.5 mg/d. The serum drug concentration was maintained at 2 ng/m L. In September 2015, she was successfully pregnant. On June 2, 2016, she delivered a male infant through cesarean section. She could breastfeed the infant in a low quantity early after delivery. Both the mother and infant were followed up until submission date. The mother was physically stable and the infant grew normally. Conclusions Patients diagnosed with Niemaoh-Pick disease can obtain favorable clinical outcomes of pregnancy and delivery after liver transplantation.