Nair Vishnu S, Chandrasekaran Venkatesh, Jagadisan Barath, Biswal Niranjan
Department of Paediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry 605006, India.
J Trop Pediatr. 2017 Aug 1;63(4):321-323. doi: 10.1093/tropej/fmw084.
An 18-month-old male infant was referred to us with pruritus from 6 months of age. He was being managed as atopic eczema with emollients and antihistamines without any response to treatment. On examination, he was found to have extensive scratch marks, mild hepatomegaly and no icterus. Blood investigations were suggestive of anicteric cholestatic liver disease. Liver biopsy was suggestive of progressive familial intrahepatic cholestasis type 3. He showed symptomatic improvement after starting ursodeoxycholic acid and fat-soluble vitamins and is under follow up.
一名18个月大的男婴自6个月大起就因瘙痒前来我院就诊。他一直被当作特应性皮炎进行治疗，使用了润肤剂和抗组胺药，但治疗毫无效果。检查发现，他身上有广泛的抓痕，肝脏轻度肿大，无黄疸。血液检查提示为无黄疸型胆汁淤积性肝病。肝活检提示为3型进行性家族性肝内胆汁淤积症。在开始使用熊去氧胆酸和脂溶性维生素后，他的症状有所改善，目前正在接受随访。
