title: Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis.
abstract: BACKGROUND
  Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term 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 IgG 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, two other 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.
  
  
  CONCLUSIONS
  In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.
authors: Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University and University Hospital Olomouc, I. P. Pavlova 6, Olomouc 779 00, Czech Republic. vratislav.smolka@fnol.cz.
authors: Vratislav
authors: V
authors: Smolka
authors: Eva
authors: E
authors: Karaskova
authors: Oksana
authors: O
authors: Tkachyk
authors: Kvetoslava
authors: K
authors: Aiglova
authors: Jiri
authors: J
authors: Ehrmann
authors: Kamila
authors: K
authors: Michalkova
authors: Michal
authors: M
authors: Konecny
authors: Jana
authors: J
authors: Volejnikova
mesh_terms: D000293:Adolescent; D000367:Age Factors; D015415:Biomarkers / Q000097:blood; D002648:Child; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D018153:Czech Republic / Q000453:epidemiology; D018450:Disease Progression; D058625:End Stage Liver Disease / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D006801:Humans; D006975:Hypertension, Portal / Q000453:epidemiology; D007074:Immunoglobulin G / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D041781:Jaundice, Obstructive / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents