Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 1 de 1
Filtrar
Más filtros











Base de datos
Intervalo de año de publicación
1.
J Pediatr ; 218: 121-129.e3, 2020 03.
Artículo en Inglés | MEDLINE | ID: mdl-31955873

RESUMEN

OBJECTIVES: To report baseline features and long-term medical/social outcomes of juvenile autoimmune liver disease, including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. STUDY DESIGN: Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. RESULTS: Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. CONCLUSIONS: Seamless management of juvenile autoimmune liver disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile autoimmune liver disease.


Asunto(s)
Colangitis Esclerosante/terapia , Hepatitis Autoinmune/terapia , Adolescente , Autoanticuerpos/inmunología , Niño , Colangitis Esclerosante/epidemiología , Continuidad de la Atención al Paciente , Empleo , Femenino , Estudios de Seguimiento , Hepatitis Autoinmune/epidemiología , Humanos , Terapia de Inmunosupresión , Inmunosupresores/uso terapéutico , Estimación de Kaplan-Meier , Trasplante de Hígado , Masculino , Grupo de Atención al Paciente , Periodo Posoperatorio , Recurrencia , Estudios Retrospectivos , Centros de Atención Terciaria , Factores de Tiempo , Resultado del Tratamiento
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA