■胆道闭锁(BA)是一种病因不明的严重新生儿进行性胆管病变。及时的开赛门肠造口术(KPE)可改善BA患者的天然肝脏生存率,尽管肝移植仍然是大多数(60%-80%)患者的最终治疗方法。然而,肝移植术后的不良反应可能是显著的。此外,肝移植后患者需要终身免疫抑制治疗.
■这里,我们报告了1例患有先天性BA(出生时间:2018年3月10日)的新生女婴(出生后76天时确诊),KPE存活(85天时首次手术),并成功接受了活体相关肝移植(LRLT)(194天时第二次手术).此外,我们回顾了有关BA的现有文献。在KPE之后(在生命的85天),宝宝的肝功能没有改善,肝肾功能指标呈加重趋势,表明肝功能在KPE之前(在生命的85天)已经严重受损,证明了肝移植手术的迫切需要。女婴在父亲的部分肝脏成功移植到她的体内后存活了下来(生命的194天)。病人成功康复。在4年的随访中没有发现其他疾病,肝肾功能指标趋于正常。
■本案例重点介绍了以下内容。术后碱性磷酸酶始终高于正常范围,尽管原因尚不清楚;他克莫司和环孢素A都没有专门为婴儿设计的配方,不符合临床个体化用药的需要,这些抗排斥药物是未来的发展方向。到目前为止,合肥仅发现一例先天性BA,这个案例对预防具有极其重要的参考意义,治疗,合肥BA的诊断,安徽省。
UNASSIGNED: Biliary atresia (BA) is a severe neonatal progressive cholangiopathy of unknown etiology. A timely Kasai portoenterostomy (KPE) improves survival of the native liver in patients with BA, although liver transplantation remains the ultimate treatment for most (60%-80%) patients. However, postoperative adverse effects of liver transplantation may be significant. In addition, patients require lifelong immunosuppressive therapy after liver transplantation.
UNASSIGNED: Here, we report a
case of a newborn female baby (birthday: 10-03-2018) with congenital BA (confirmed at 76 days of life) who survived KPE (first surgery at 85 days of life) and underwent successful living-related liver transplantation (LRLT) (second surgery at 194 days of life). Additionally, we reviewed the existing literature on BA. After KPE (at 85 days of life), the liver function of the baby did not improve, and the indicators of liver and kidney function showed a trend of aggravation, indicating that the liver function had been seriously damaged before KPE (at 85 days of life), demonstrating the urgent need for liver transplantation surgery. The female baby survived after part of her father\'s liver was successfully transplanted into her body (at 194 days of life). The patient recovered successfully. No other diseases were found at the 4-year follow-up, and all indices of liver and kidney functions tended to be normal.
UNASSIGNED: This
case highlights the following. Postoperative alkaline phosphatase was consistently above the normal range, although the reason for this was unclear; neither tacrolimus nor cyclosporine A has formulations designed specifically for infants, which does not meet the needs of clinical individualized medication, suggesting that these anti-rejection drugs are future development directions. Only one
case of congenital BA has been found thus far in Hefei, and this
case has extremely important reference significance for the prevention, treatment, and diagnosis of BA in Hefei, Anhui province.