RESUMEN
BACKGROUND: Autoimmune hepatitis (AIH) is an auto-inflammatory liver disease of children and adults, affecting patients of any age, sex, race or ethnicity, with more prevalence in females. OBJECTIVE: The aim of this study was to evaluate clinical manifestation, laboratory findings, and outcome of children with autoimmune hepatitis. MATERIALS AND METHODS: We evaluated 86 patients treated and followed with final diagnosis of AIH between years 2010 to 2018. Physical findings (including jaundice, hepatomegaly, splenomegaly and encephalopathy), liver enzymes, liver histology and autoantibodies (including ANA, Anti LKM-1 and ASMA) were extracted from medical files. Then the patients were followed for their final outcome (including response to medical treatment or successful treatment withdrawal, liver transplantation or death). RESULTS: Among 86 patients with AIH with mean age 9.10±4.36 years old, 66.27% were females. Jaundice (75.6%) and hepatomegaly (46.5%) were the most frequent physical findings, followed by splenomegaly (32.6%) and encephalopathy (17.4%). Aminotransferases including AST and ALT were elevated at least 3 times more than upper limit of normal in most of the patients (61.6% and 55.81%, respectively). Autoantibodies were available in 53 of 86 patients, 24.5% had AIH-1, 3.8% had AIH-II and 67.9% were seronegative. Medical treatment including prednisolone and azathioprine was started for patients, 53 of 86 cases (61.6%) had remission and 11 of 86 (13.7%) tolerated medication withdrawal successfully. Among all cases, 26 (30.2%) patients needed liver transplantation. Mortality rate was 9 among 86 cases (10.5%). CONCLUSION: Jaundice and hepatomegaly was the most frequent clinical findings. Mortality rate was 10.5.
Asunto(s)
Hepatitis Autoinmune/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Hepatitis Autoinmune/terapia , Humanos , Lactante , Irán , Masculino , PronósticoRESUMEN
ABSTARCT Background : Autoimmune hepatitis (AIH) is an auto-inflammatory liver disease of children and adults, affecting patients of any age, sex, race or ethnicity, with more prevalence in females. Objective : The aim of this study was to evaluate clinical manifestation, laboratory findings, and outcome of children with autoimmune hepatitis. Materials and methods : We evaluated 86 patients treated and followed with final diagnosis of AIH between years 2010 to 2018. Physical findings (including jaundice, hepatomegaly, splenomegaly and encephalopathy), liver enzymes, liver histology and autoantibodies (including ANA, Anti LKM-1 and ASMA) were extracted from medical files. Then the patients were followed for their final outcome (including response to medical treatment or successful treatment withdrawal, liver transplantation or death). Results : Among 86 patients with AIH with mean age 9.10±4.36 years old, 66.27% were females. Jaundice (75.6%) and hepatomegaly (46.5%) were the most frequent physical findings, followed by splenomegaly (32.6%) and encephalopathy (17.4%). Aminotransferases including AST and ALT were elevated at least 3 times more than upper limit of normal in most of the patients (61.6% and 55.81%, respectively). Autoantibodies were available in 53 of 86 patients, 24.5% had AIH-1, 3.8% had AIH-II and 67.9% were seronegative. Medical treatment including prednisolone and azathioprine was started for patients, 53 of 86 cases (61.6%) had remission and 11 of 86 (13.7%) tolerated medication withdrawal successfully. Among all cases, 26 (30.2%) patients needed liver transplantation. Mortality rate was 9 among 86 cases (10.5%). Conclusion : Jaundice and hepatomegaly was the most frequent clinical findings. Mortality rate was 10.5%.
RESUMEN Antecedentes : La hepatitis autoinmune (AIH) es una enfermedad hepática autoinflamatoria de niños y adultos, que afecta a pacientes de cualquier edad, sexo, raza u origen étnico, con mayor prevalencia en las mujeres. Objetivo : El objetivo de este estudio fue evaluar la manifestación clínica, los hallazgos de laboratorio y el resultado de los niños con hepatitis autoinmune. Materiales y métodos : Evaluamos 86 pacientes tratados y seguidos con el diagnóstico final de AIH entre los años 2010 a 2018. Los hallazgos físicos (incluyendo ictericia, hepatomegalia, esplenomegalia y encefalopatía), enzimas hepáticas, histología hepática y autoanticuerpos (incluidos ANA, Anti LKM-1 y ASMA) se extrajeron de las historias médicas. Luego, los pacientes fueron seguidos para su resultado final (incluida la respuesta al tratamiento médico o la retirada exitosa del tratamiento, el trasplante de hígado o la muerte). Resultados : Entre 86 pacientes con AIH con una edad media de 9,10 ± 4,36 años, el 66,27% eran mujeres. La ictericia (75,6%) y la hepatomegalia (46,5%) fueron los hallazgos físicos más frecuentes, seguidos de esplenomegalia (32,6%) y encefalopatía (17,4%). Las aminotransferasas que incluyen AST y ALT se elevaron al menos 3 veces más que el límite superior de la normalidad en la mayoría de los pacientes (61,6% y 55,81%, respectivamente). Los autoanticuerpos estaban disponibles en 53 de 86 pacientes, 24,5% tenían AIH-1, 3,8% tenían AIH-II y 67,9% eran seronegativos. Se inició tratamiento médico que incluyó prednisolona y azatioprina, 53 de 86 casos (61,6%) tuvieron remisión y 11 de 86 (13,7%) toleraron el retiro de medicamentos con éxito. Entre todos los casos, 26 (30,2%) pacientes necesitaron un trasplante de hígado. La tasa de mortalidad fue de 9 entre 86 casos (10,5%). Conclusión : la ictericia y la hepatomegalia fueron los hallazgos clínicos más frecuentes. La tasa de mortalidad fue del 10,5%.