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新生儿急性肝衰竭

Neonatal acute liver failure.

作者信息

Taylor Sarah A, Whitington Peter F

机构信息

Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, IL.

出版信息

Liver Transpl. 2016 May;22(5):677-85. doi: 10.1002/lt.24433.

Abstract

Neonatal acute liver failure (NALF) is a rare disease about which there is little published data; however, NALF is an extremely important condition as it is distinct from acute liver failure seen in older children and adults. First, unlike acute liver failure in older patients, NALF can be diagnosed in an infant with cirrhosis. This is due to the fetal-neonatal continuum of liver disease, or the principle that neonatal liver failure may be the result of a liver disease that began in utero. Further differences exist in the mechanism of disease, diagnostic principles, and the common etiologies of NALF when compared with pediatric and adult acute liver failure. This review will address many of the distinguishing features of NALF and focus on the most common etiologies of NALF, including gestational alloimmune liver disease (GALD), the most common cause of NALF. Additionally, this review will provide insight into the pathogenesis, diagnosis, and treatment of this rare condition. Liver Transplantation 22 677-685 2016 AASLD.

摘要

新生儿急性肝衰竭(NALF)是一种罕见疾病,已发表的数据很少;然而,NALF是一种极其重要的病症,因为它与大龄儿童和成人的急性肝衰竭不同。首先,与大龄患者的急性肝衰竭不同,NALF可在患有肝硬化的婴儿中被诊断出来。这是由于肝病的胎儿 - 新生儿连续体,或者说新生儿肝衰竭可能是始于子宫内的肝病的结果这一原则。与儿童和成人急性肝衰竭相比,NALF在疾病机制、诊断原则和常见病因方面还存在其他差异。本综述将阐述NALF的许多显著特征,并聚焦于NALF最常见的病因,包括妊娠同种免疫性肝病(GALD),这是NALF最常见的病因。此外,本综述将深入探讨这种罕见病症的发病机制、诊断和治疗。《肝脏移植》2016年第22卷,第677 - 685页,美国肝病研究学会。

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