Pediatric Chronic Liver Diseases: A Clinicopathological Study from a Tertiary Care Center
Pediatric Chronic Liver Diseases: A Clinicopathological Study from a Tertiary Care Center
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小儿慢性肝病：三级护理中心的临床病理学研究
DOI：
10.22038/ijp.2019.37294.3246
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发表时间：
2019
影响因子：
2.1
通讯作者：
A. El
中科院分区：
文献类型：
--
作者：
A. Abou;A. Ahmed;A. El
儿童慢性肝病（CLD）是一个日益严重的健康问题，发病率和死亡率都很高。本研究旨在确定上埃及Sohag大学医院儿科CLD的临床病理模式。材料与方法2014年6月至2018年5月，共有151名CLD儿童纳入了一项前瞻性医院研究。排除急性肝病或肝局灶性病变病例。所有患者均接受详细病史和全面体格检查。对所有儿童进行腹部超声检查、全血细胞计数、肝功能检查、病毒血清学检查、自身免疫性肝炎自身抗体评估和肝核心活检。结果儿科CLD占儿科住院总人数的1.6%。新生儿胆汁淤积障碍（NCD）和代谢性肝脏疾病（MLD）是导致CLD的主要原因（分别占41.05%和35.1%）。非传染性疾病包括新生儿肝炎（25.1%）、肝外胆道闭锁（13.2%）和小叶间胆管缺乏（2.7%）。MLD包括糖原储存病（26.5%）、未确定的先天性代谢错误（5.3%）、戈谢病（2.0%）和尼曼匹克病（1.3%）。CLD的其他病因包括自身免疫性肝炎（8.6%）、先天性肝纤维化（5.9%）、非酒精性脂肪性肝病（4.0%）、慢性丙型肝炎感染（2.7%）和布氏病（0.6%）。89例患者随访中，临床病程平稳的占52.8%，临床好转的占34.8%，临床恶化的占12.3%。结论上埃及地区CLD发病率呈上升趋势，主要由新生儿胆汁淤积和代谢性肝脏疾病引起。总的来说，儿童的结果是有利的，与其他国家相当。
Background Chronic liver diseases (CLD) in children represent a growing health problem with significant morbidity and mortality. This study aimed to define the clinicopathological pattern of pediatric CLD in Sohag University Hospital, Sohag,Upper Egypt. Materials and Methods A total of 151children with CLD were included in a prospective hospital-based study from June 2014 to May 2018. Cases of acute liver illness or hepatic focal lesions were excluded. All patients were subjected to detailed history and thorough physical examination. Abdominal ultrasonography, CBC, liver function tests, viral serology, evaluation of autoantibodies for autoimmune hepatitis, and liver core biopsies were performed for all children. Results Pediatric CLD comprised 1.6% of total admissions in pediatric department. Neonatal cholestasis disorders (NCD), and metabolic liver disorders (MLD) were the leading causes of CLD (41.05% and 35.1%, respectively). NCD comprised neonatal hepatitis (25.1%), extrahepatic biliary atresia (13.2%), and paucity of interlobular bile ducts (2.7%). MLD included glycogen storage disease (26.5%), undetermined inborn error of metabolism (5.3%), Gaucher's disease (2.0%), and Niemann Pick disease (1.3%). Other causes of CLD comprised autoimmune hepatitis (8.6%), congenital hepatic fibrosis (5.9%), non-alcoholic fatty liver disease (4.0%), chronic hepatitis C infection (2.7%), and Budd Chiari disease (0.6%). On follow-up of 89 cases, stationary clinical course, clinical improvement, and clinical deterioration were seen in 52.8%, 34.8%, and 12.3% of them, respectively. Conclusion The rate of CLD is growing in Upper Egypt and is mainly caused by neonatal cholestasis and metabolic liver disorders. In general, the outcome of children is favorable and comparable to other countries.