"Clinical and histopathological characterization of children with autoimmune hepatitis at a referral center in bogotá, colombia"

"Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease. It is uncommon in children and adolescents, and is a diagnostic challenge for clinicians and pathologists. We describe the clinical, biochemical and histopathological characteristics of 21 pediatric patients with AIH diagn...

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Detalles Bibliográficos
Autores Principales: Suárez D., Andrade R., Vera J.F., López-Panqueva R.P.
Formato: Artículo (Article)
Lenguaje:Inglés (English)
Publicado: Asociacion Colombiana de Gastroenterologia 2019
Materias:
Acceso en línea:https://repository.urosario.edu.co/handle/10336/23032
https://doi.org/10.22516/25007440.346
Descripción
Sumario:"Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease. It is uncommon in children and adolescents, and is a diagnostic challenge for clinicians and pathologists. We describe the clinical, biochemical and histopathological characteristics of 21 pediatric patients with AIH diagnosed in the last 14 years. Liver biopsies were reassessed to analyze histopathological findings in detail. Of the 21 cases evaluated, 12 (57.1%) were girls and young women, the median age was 14 years old, and 17 (80.9%) had type 1 AIH. The most frequent clinical signs were jaundice (66.7%), choluria (44.4%), evidence of portal hypertension with esophageal varices (47.1%), and splenomegaly (41.2%). Histories of other autoimmune diseases were found in 11.8% of these patients. Elevated levels of aminotransferases were found in 89.5% of the patients, hyperbilirubinemia was found in 88.9%, and 60.0% of the cases had low levels of serum albumin. Reassessed biopsies showed portal lymphoplasmocytic infiltrate (94.4%), interface hepatitis (77.8%) and rosette formation (50.0%). Hyaline inclusions were found in Kupffer cells in 42.9% of the biopsies. About 33.5% of the cases showed cirrhosis at the initial biopsy. Despite immunosuppressive treatment, four patients required liver transplantation and two are on the waiting list. AIH in children can manifest with jaundice, choluria, signs of portal hypertension, elevated aminotransferases, hyperbilirubinemia and circulating antibodies. Hyaline inclusions in Kupffer cells may be a useful finding in the histopathological diagnosis of AIH in children. © 2019 Asociaciones Colombianas de Gastroenterología, Endoscopia digestiva, Coloproctología y Hepatología."