Abstract：
Multiple causes of congenital neonatal cholestasis have been identified, and are classified as extrahepatic or intrahepatic. Biliary atresia (BA), Alagille syndrome (AGS), and progressive familial intrahepatic cholestasis (PFIC) are the most common of these. Many factors associated with cholestatic diseases are known to degrade the oral health of these children. What are the oral manifestations associated with these diseases in the pediatric population? The aim of this article was to evaluate the impact of congenital cholestasis on oral health in pediatric patients. A systematic review of case reports and case series was carried out in PubMed, the Cochrane Library, and the Web of Science to identify relevant articles in French and English published up to April 2022. The review included 19 studies, 16 case reports, and three case series. Only studies dealing with BA and AGS were found. These studies showed an impact on jaw morphology, dental structure, and periodontal health. The facial dysmorphism observed in AGS was specific. Exposure to high levels of bilirubin during the period of dental calcification led to particular coloration. Regarding periodontal status, gingival inflammation was common in these patients, probably resulting from the use of certain treatment-associated drugs and poor oral hygiene. Cohort studies are needed to confirm the classification of these children as being at high individual risk of caries. Many major oral manifestations are found in children with AGS and BA, confirming the need to include a dentist in the care team of patients with congenital cholestatic disease as early as possible. It appears necessary to carry out individual prospective studies of each phenotype in order to confirm and better describe the oral impact of these cholestatic diseases and provide adequate medical care.
摘要：
已经确定了先天性新生儿胆汁淤积的多种原因，分为肝外或肝内。胆道闭锁(BA),Alagille综合征（AGS），和进行性家族性肝内胆汁淤积症（PFIC）是最常见的。已知与胆汁淤积疾病相关的许多因素会降低这些儿童的口腔健康。在儿科人群中，与这些疾病相关的口腔表现是什么？本文的目的是评估先天性胆汁淤积对儿科患者口腔健康的影响。在PubMed中对病例报告和病例系列进行了系统审查，Cochrane图书馆,和WebofScience以识别截至2022年4月出版的法语和英语相关文章。该综述包括19项研究，16例病例报告，三个案例系列。仅发现涉及BA和AGS的研究。这些研究显示了对下颌形态的影响，牙科结构,和牙周健康。在AGS中观察到的面部畸形是特异性的。在牙齿钙化期间暴露于高水平的胆红素会导致特殊的着色。关于牙周状况，牙龈炎症在这些患者中很常见，可能是由于使用某些与治疗相关的药物和口腔卫生不良所致。需要进行队列研究，以确认这些儿童的龋齿风险较高的分类。在患有AGS和BA的儿童中发现了许多主要的口腔表现，确认有必要尽早将牙医纳入先天性胆汁淤积病患者的护理团队。似乎有必要对每种表型进行单独的前瞻性研究，以确认和更好地描述这些胆汁淤积性疾病的口腔影响并提供足够的医疗服务。
