METHODS: A comprehensive review of the literature using PubMed, Scopus, Web of Science and Embase was performed. Two authors independently identified the most relevant studies published over the past 20 years up to February 2022, including original papers, narrative reviews, observational studies, clinical trials, systematic reviews and meta-analyses. The literature was screened, and preclinical studies about pathogenetic mechanisms were also included. Keywords searched for each fat-soluble vitamin (A, D, E and K), alone or in combination, were \"cholestasis\", \"chronic liver disease\", \"biliary atresia\", \"malnutrition\" and \"nutritional needs\". Studies published prior to the selected time range were searched manually and, when considered relevant, included within the list of references.
RESULTS: Eight hundred twenty-six articles were initially screened. From these, 48 studies were selected. A comparison of the recommended methods of supplementation for fat-soluble vitamins was then carried out. The causes of malabsorption were explained and current methods for defining deficiency and monitoring complications were summarized.
CONCLUSIONS: According to the literature, children with cholestasis are at a higher risk of fat-soluble vitamin deficiency. Although there are general recommendations, the treatment for vitamin deficiency is not uniformly validated.
方法:使用PubMed对文献进行全面回顾,Scopus,进行了WebofScience和Embase。两位作者独立确定了截至2022年2月的过去20年中发表的最相关的研究,包括原始论文。叙事评论,观察性研究,临床试验,系统评价和荟萃分析。对文献进行了筛选,和关于发病机制的临床前研究也包括在内。关键词搜索每种脂溶性维生素(A,D,E和K),单独或组合,是“胆汁淤积”,“慢性肝病”,“胆道闭锁”,“营养不良”和“营养需求”。手动搜索在选定时间范围之前发表的研究,当被认为相关时,包含在参考文献列表中。
结果:最初筛选了八百二十六篇文章。从这些,选择了48项研究。然后比较了推荐的脂溶性维生素补充方法。解释了吸收不良的原因,并总结了目前定义缺陷和监测并发症的方法。
结论:根据文献,胆汁淤积患儿发生脂溶性维生素缺乏症的风险较高.虽然有一般性建议,维生素缺乏症的治疗方法尚未得到统一验证。