Biliary Atresia in 2021: Epidemiology, Screening and Public Policy

被引:28
作者
Schreiber, Richard A. [1 ]
Harpavat, Sanjiv [2 ,3 ]
Hulscher, Jan B. F. [4 ]
Wildhaber, Barbara E. [5 ]
机构
[1] Univ British Columbia, Dept Pediat, Fac Med, Div Gastroenterol Hepatol & Nutr, Vancouver, BC V6T 1Z3, Canada
[2] Baylor Coll Med, Dept Pediat, Div Gastroenterol Hepatol & Nutr, Houston, TX 77030 USA
[3] Texas Childrens Hosp, Houston, TX 77030 USA
[4] Univ Groningen, Univ Med Ctr Groningen, Div Pediat Surg, Dept Surg, NL-9713 GZ Groningen, Netherlands
[5] Univ Geneva, Dept Pediat Gynecol & Obstet, Div Pediat Surg, Swiss Pediat Liver Ctr, CH-1205 Geneva, Switzerland
关键词
biliary atresia; pediatric liver disease; newborn screening; public health; epidemiology; STOOL COLOR CARD; NEONATAL LIVER-DISEASE; KASAI OPERATION; HEPATIC PORTOENTEROSTOMY; TERM OUTCOMES; INFANT; BILIRUBIN; ETIOLOGY; IMPACT; COHORT;
D O I
10.3390/jcm11040999
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Biliary atresia (BA) is a rare newborn liver disease with significant morbidity and mortality, especially if not recognized and treated early in life. It is the most common cause of liver-related death in children and the leading indication for liver transplantation in the pediatric population. Timely intervention with a Kasai portoenterostomy (KPE) can significantly improve prognosis. Delayed disease recognition, late patient referral, and untimely surgery remains a worldwide problem. This article will focus on biliary atresia from a global public health perspective, including disease epidemiology, current national screening programs, and their impact on outcome, as well as new and novel BA screening initiatives. Policy challenges for the implementation of BA screening programs will also be discussed, highlighting examples from the North American, European, and Asian experience.
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页数:15
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