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Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis 被引量:2
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作者 Vratislav Smolka Eva Karaskova +5 位作者 Oksana Tkachyk Kvetoslava Aiglova Jiri Ehrmann Kamila Michalkova Michal Konecny Jana Volejnikova 《Hepatobiliary & Pancreatic Diseases International》 SCIE CAS CSCD 2016年第4期412-418,共7页
BACKGROUND: Sclerosing cholangitis(SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC acco... BACKGROUND: Sclerosing cholangitis(SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS: We retrospectively followed up 25 children with SC over a period of 4-17 years(median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis(ASC); other patients were included in a group of primary sclerosing cholangitis(PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS: Fourteen(56%) patients had PSC and 11(44%) had ASC. Patients with ASC were significantly younger at the time of diagnosis(12.3 vs 15.4 years, P=0.032) and had higher Ig G levels(22.7 vs 17.2 g/L, P=0.003). The mentioned complications occurred in 4(16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION: In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC. 展开更多
关键词 autoimmune sclerosing cholangitis childhood inflammatory bowel disease primary sclerosing cholangitis prognosis
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