罕见“三重综合征”1例并文献复习
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摘要
<正>患者,女,49岁,身高151 cm,体质量42.3 kg,既往无乙肝病史。父亲曾有乙肝、黄疸病史,因肝癌去世,其弟弟亦有乙肝及黄疸病史。2017年7月无明显诱因下逐渐身体消瘦(体质量降低2.5 kg),后出现身目黄染,伴有小便黄,呈浓茶样,陶土样大便,量少,纳差,上腹部胀闷不适,自行服用胃药(具体不详)后无缓解。2018年2月10日身目黄染加重,门诊筛查肝炎分型均阴性,进一步行腹部CT增强扫描示:肝S4段小囊肿;肝右叶小斑点状钙化灶;肝门区淋巴管轻度扩张;胆囊结石。进一步行上腹部MR示:(1)早期肝硬化,脾大,胃底静脉曲张;肝内
引文
[1] Kia L,Beattie A,Green RM. Autoimmune hepatitis in patients with human immunodeficiency virus(HIV):Case reports of a rare, but important diagnosis with therapeutic implications[J]. Medicine,2017.(7):e6011. DOI:10.1097/MD. 0000000000006011.
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[7] Ehlken H,Zenouzi R, Schramm C. Risk of cholangiocarcinoma in patients with primary sclerosing cholangitis:diagnosis and surveillance[J]. Current Opinion in Gastroenterology,2017,(2):78-84. DOI:10.1097/MOG. 0000000000000335.
[8] Fattahi MR,Malek-Hosseini SA. Clinical course of ulcerative colitis after liver transplantation in patients with concomitant primary sclerosing cholangitis and ulcerative colitis[J]. Inflammatory Bowel Diseases,2017,(7):1160-1167. DOI:10.1097/MIB.0000000000001105.
[9] Cardile S,Candusso M,Papadat B. Lack of efficacy of infliximab in the treatment of primary sclerosing cholangitis in inflammatory bowel diseases in childhood[J]. European Journal of Gastroenterology&Hepatology,2017,(6):736-736. DOI:10.1097/MEG. 0000000000000847.
[10] Hov JR, Kummen M. Intestinal microbiota in primary sclerosing cholangitis[J]. Current Opinion in Gastroenterology,20.17,(2):85-92. DOI:10.1097/MOG.0000000000000334.
[11] Assis DN,Abdelghany O,Cai SY, et al. Combination therapy of-all-trans retinoic acid with ursodeoxycholic acid in patients with primary sclerosing cholangitis:A human pilot study[J]. Journal of Clinical Gastroenterology,2017,(2):e11-e16. DOI:10.1097/MCG.0000000000000591.
[12] Zheng HH,Jiang XL. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease:a meta-analysis of 16 observational studies[J]. European Journal of Castroenterology&Hepatology,2016,(4):383-390. DOI:10. 1097/MEG. 0000000000000576.
[13] Rossi RE,Sara DC. Massironi. Primary sclerosing cholangitis associated with inflammatory bowel disease:an update[J]. European Journal of Gastroenterology&Hepatology, 2016,(2):123-131. DOI:10.1097/MEG. 0000000000000532.
[14] Jochen M,Johannes H. Impact of microbes on the pathogenesis of pri-mary biliary cirrhosis(PBC)and primary sclerosing cholangitis(PSC)[J]. International Journal of Molecular Sciences,2016,(11):DOI:10. 3390/ijms17111864.
[15] Fraga M, Fournier N,Safron E. Primary sclerosing cholangitis in the Swiss Inflammatory Bowel Disease Cohort Study:prevalence, risk factors,and long-term follow-up[J]. European Journal of Gastroenterology&Hepatology,2017,(1):91-97. DOI:10. 1097/MEG.0000000000000747.
[16] Gauss A,Sauer P,Stiehl A, et al. Evaluation of biliary calprotectin as a biomarker in primary sclerosing cholangitis[J]. Medicine,2016,(17):e3510-e3510. DOI:10.1097/MD. 0000000000003510.
[17] Tenca A.Forkkila M.Jalanko H. et al. Environmental risk factors of ped-iatric-onset primary sclerosing cholangitis and autoimmune hepatitis[J].Journal of Pediatric Gastroenterology and Nutrition,2016,(3):437-442.D01:10.1097/MPG. 0000000000000995.
[18]Jegadeesan R, Navaneethan U,Shishi. Impact of concurrent non-IBD tImmunological diseases on the outcome of primary sclerosing cholangitis[J]. Inflammatory Bowel Diseases.2016.(4):948-954. DOI:10. 1097/MIB. 0000000000000690.
[19] Li YN,Zhou L,Zhao X, et al.The importance of IgG4 screening in patients diagnosed with primary sclerosing cholangitis in the past:A case rediagnosed as IgG4-SC after 10 years[J]. Medicine,2016,(50):e5628-e5628. DOI:10.1097/MD. 0000000000005628.
[2] Cheung AC.Lazaridis KN,Nichola. Emerging pharmacologic therapies for primary sclerosing cholangitis[J]. Current Opinion in Gastroenterology,2017,(3):149-157. DOI:10.1097/MOG. 0000000000000352.
[3] Chung BK, Hirschfield GM. Immunogenetics in primary sclerosing cholangitis[J]. Current Opinion in Gastroenterology,2017,(2):93-98. DOI:10.1097/MOG.0000000000000336.
[4] Tabibian JH,Gossard A,El-Youssef M. Prospective clinical trial of rifaximin therapy for patients with primary sclerosing cholangitis[J].American Journal of Therapeutics, 2017,(1):e56-e63. DOI:10.1097/MJT. 0000000000000102.
[5] Housset C. Updates on primary sclerosing cholangitis[J]. Current Opinion in Gastroenterology,2017,(2):69-70. DOI:10. 1097/MOG.0000000000000342.
[6] Takakura WR,Tabibian JH,Christopher L.The evolution of natural history of primary sclerosing cholangitis[J]. Current Opinion in Gastroenterology,2017,(2):71-77. DOI:10.1097/MOG. 0000000000000333.
[7] Ehlken H,Zenouzi R, Schramm C. Risk of cholangiocarcinoma in patients with primary sclerosing cholangitis:diagnosis and surveillance[J]. Current Opinion in Gastroenterology,2017,(2):78-84. DOI:10.1097/MOG. 0000000000000335.
[8] Fattahi MR,Malek-Hosseini SA. Clinical course of ulcerative colitis after liver transplantation in patients with concomitant primary sclerosing cholangitis and ulcerative colitis[J]. Inflammatory Bowel Diseases,2017,(7):1160-1167. DOI:10.1097/MIB.0000000000001105.
[9] Cardile S,Candusso M,Papadat B. Lack of efficacy of infliximab in the treatment of primary sclerosing cholangitis in inflammatory bowel diseases in childhood[J]. European Journal of Gastroenterology&Hepatology,2017,(6):736-736. DOI:10.1097/MEG. 0000000000000847.
[10] Hov JR, Kummen M. Intestinal microbiota in primary sclerosing cholangitis[J]. Current Opinion in Gastroenterology,20.17,(2):85-92. DOI:10.1097/MOG.0000000000000334.
[11] Assis DN,Abdelghany O,Cai SY, et al. Combination therapy of-all-trans retinoic acid with ursodeoxycholic acid in patients with primary sclerosing cholangitis:A human pilot study[J]. Journal of Clinical Gastroenterology,2017,(2):e11-e16. DOI:10.1097/MCG.0000000000000591.
[12] Zheng HH,Jiang XL. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease:a meta-analysis of 16 observational studies[J]. European Journal of Castroenterology&Hepatology,2016,(4):383-390. DOI:10. 1097/MEG. 0000000000000576.
[13] Rossi RE,Sara DC. Massironi. Primary sclerosing cholangitis associated with inflammatory bowel disease:an update[J]. European Journal of Gastroenterology&Hepatology, 2016,(2):123-131. DOI:10.1097/MEG. 0000000000000532.
[14] Jochen M,Johannes H. Impact of microbes on the pathogenesis of pri-mary biliary cirrhosis(PBC)and primary sclerosing cholangitis(PSC)[J]. International Journal of Molecular Sciences,2016,(11):DOI:10. 3390/ijms17111864.
[15] Fraga M, Fournier N,Safron E. Primary sclerosing cholangitis in the Swiss Inflammatory Bowel Disease Cohort Study:prevalence, risk factors,and long-term follow-up[J]. European Journal of Gastroenterology&Hepatology,2017,(1):91-97. DOI:10. 1097/MEG.0000000000000747.
[16] Gauss A,Sauer P,Stiehl A, et al. Evaluation of biliary calprotectin as a biomarker in primary sclerosing cholangitis[J]. Medicine,2016,(17):e3510-e3510. DOI:10.1097/MD. 0000000000003510.
[17] Tenca A.Forkkila M.Jalanko H. et al. Environmental risk factors of ped-iatric-onset primary sclerosing cholangitis and autoimmune hepatitis[J].Journal of Pediatric Gastroenterology and Nutrition,2016,(3):437-442.D01:10.1097/MPG. 0000000000000995.
[18]Jegadeesan R, Navaneethan U,Shishi. Impact of concurrent non-IBD tImmunological diseases on the outcome of primary sclerosing cholangitis[J]. Inflammatory Bowel Diseases.2016.(4):948-954. DOI:10. 1097/MIB. 0000000000000690.
[19] Li YN,Zhou L,Zhao X, et al.The importance of IgG4 screening in patients diagnosed with primary sclerosing cholangitis in the past:A case rediagnosed as IgG4-SC after 10 years[J]. Medicine,2016,(50):e5628-e5628. DOI:10.1097/MD. 0000000000005628.