Department of Pathology, St. John's Medical College, Bangalore, India.
Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, India.
Fetal Pediatr Pathol. 2020 Aug;39(4):317-333. doi: 10.1080/15513815.2019.1652376. Epub 2019 Aug 22.
Non syndromic paucity of interlobular bile ducts (NS-PILBD) constitutes a miniscule of infantile cholestasis. Clinical details, investigations, surgical findings, management and outcome of cases of NS-PILBD at liver biopsy were analyzed. Specific histopathological features including bile duct to portal tract ratio were studied. Eighteen cases (1993-2013) are detailed. Clinical presentation and investigations were similar to biliary atresia. Hepatic scintigraphy showed no gut excretion in 13/18 and operative cholangiogram was normal in all. Liver biopsy showed a median Scheuer fibrosis stage of 2, the mean bile duct/portal tract ratio was 0.29. The average age at last follow up of twelve cases was 54.9 months . Ten were asymptomatic and anicteric, the liver function tests had normalized over 3-15 months. Histopathology differentiated NS-PILBD from other causes of infantile cholestasis .The idiopathic form generally had a favorable long term outcome with medical management.
非综合征性肝内胆管缺如（NS-PILBD）构成了婴儿期胆汁淤积症的一小部分。对肝活检中 NS-PILBD 病例的临床详细信息、检查、手术发现、治疗和结果进行了分析。研究了包括胆管与门脉束比在内的特定组织病理学特征。详细描述了 18 例（1993-2013 年）病例。临床表现和检查与胆道闭锁相似。肝脏闪烁显像显示 13/18 例无肠道排泄，所有病例的手术胆管造影均正常。肝活检显示中位数 Scheuer 纤维化分期为 2，平均胆管/门脉束比为 0.29。12 例的最后随访平均年龄为 54.9 个月。10 例无症状且无黄疸，肝功能检查在 3-15 个月内恢复正常。组织病理学将 NS-PILBD 与婴儿期胆汁淤积症的其他原因区分开来。特发性形式通常通过药物治疗获得良好的长期预后。
