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复发性黄疸解析:一名印度患者的良性复发性肝内胆汁淤积症(BRIC)病例

Recurrent Jaundice Unraveled: A Case of Benign Recurrent Intrahepatic Cholestasis (BRIC) in an Indian Patient.

作者信息

Bansal Rachit, Patel Abhay, Singh Chinmaya, N C Kiran, Saxena Sonakshi

机构信息

General Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, IND.

General Medicine, Felix Hospital, Noida, IND.

出版信息

Cureus. 2024 Nov 29;16(11):e74736. doi: 10.7759/cureus.74736. eCollection 2024 Nov.

DOI:10.7759/cureus.74736
PMID:39735080
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11682693/
Abstract

Benign recurrent intrahepatic cholestasis (BRIC) is a rare, autosomal recessive liver disorder characterized by intermittent episodes of cholestasis without progression to chronic liver disease or cirrhosis. Patients experience recurrent jaundice and severe pruritus, significantly impacting their quality of life. This case report presents a 15-year-old boy with a history of recurrent jaundice and pruritus. The patient was successfully managed with ursodeoxycholic acid (UDCA), leading to symptomatic relief and biochemical improvement. BRIC is a rare disorder and should be considered in the differential diagnosis of recurrent cholestasis in adolescents. Early recognition and appropriate treatment can improve patient outcomes and prevent unnecessary investigations.

摘要

良性复发性肝内胆汁淤积症(BRIC)是一种罕见的常染色体隐性遗传性肝脏疾病,其特征为胆汁淤积间歇性发作,且不会进展为慢性肝病或肝硬化。患者会反复出现黄疸和严重瘙痒,严重影响其生活质量。本病例报告介绍了一名有反复黄疸和瘙痒病史的15岁男孩。该患者使用熊去氧胆酸(UDCA)成功治疗,症状得到缓解,生化指标有所改善。BRIC是一种罕见疾病,在青少年复发性胆汁淤积的鉴别诊断中应予以考虑。早期识别和适当治疗可改善患者预后,避免不必要的检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/872e/11682693/74f35aafc11a/cureus-0016-00000074736-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/872e/11682693/657dba0640b7/cureus-0016-00000074736-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/872e/11682693/74f35aafc11a/cureus-0016-00000074736-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/872e/11682693/657dba0640b7/cureus-0016-00000074736-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/872e/11682693/74f35aafc11a/cureus-0016-00000074736-i02.jpg

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本文引用的文献

1
Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies.1型、2型和3型进行性家族性肝内胆汁淤积症的最新进展:预后及治疗策略
World J Hepatol. 2022 Jan 27;14(1):98-118. doi: 10.4254/wjh.v14.i1.98.
2
Successful clinical application of extracorporal albumin dialysis in a patient with benign recurrent intrahepatic cholestasis (BRIC).体外白蛋白透析在一名良性复发性肝内胆汁淤积症(BRIC)患者中的成功临床应用。
Z Gastroenterol. 2001 Jun;39 Suppl 2:13-4. doi: 10.1055/s-2001-919024.