Hepatitis autoinmune en niños: diagnostico / Autoimmune hepatitis in children: diagnosis

El diagnóstico de la hepatitis autoinmune (HAI), se basa en una serie de criterios definidos por elGrupo Internacional de HAI (IAIHG) que permite clasificarla como HAI probable o definitiva. Un criterio clave para el diagnóstico de la HAI es la detección de ANA, SMA, y anti- LKM-1 por inmunofluorescencia indirecta. Otros anticuerpos menos frecuentes probados, pero de importancia diagnóstica en HAI pediátrica incluyen los anticuerpos tipo: citosol 1 hígado (LC-1), anti- citoplasma de los neutrófilos (ANCA) y el antígeno soluble hepático (SLA). La Ig G está usualmente elevada en ambos tipos de HAI, cerca del 15% de niños con HAI I y el 25% de niños con HAI tipo II tienen valores normales. La biopsia hepática es necesaria para establecer el diagnóstico de HAI.

The diagnosis of autoimmune hepatitis (HAI), is based on a set of criteria defined by the International HAI Group (IAIHG) that allows classified as a probable or definite HAI. A key criterion for the diagnosis of HAI is the detection of ANA, SMA, and anti-LKM-1 by indirectimmunofluorescence. Other less common antibodies tested, but important diagnostic tool in pediatric HAI include antibodies such as: liver cytosol 1 (LC-1), anti-neutrophil cytoplasmic (ANCA) and soluble liver antigen (SLA). The Ig G is usually high in both types of HAI, about 15% of children with HAI I and 25% of children with HAI type II are normal. Liver biopsy is necessary to establish the diagnosis of HAI.

Autoimmune hepatitis in children, clinical features and biochemical of Iranian children

There are only a very small number of reports which discuss subtype, sex distribution, clinical features and laboratory characteristics of autoimmune hepatitis in children. The aim of this study was both to define the clinical features, biochemical and histological findings and also to determine the age and sex related distribution of autoimmune hepatitis [AIH]. Data of 30 children presenting with AIH [20 girls and 10 boys] have been analyzed for their clinical, serological, and histological profile. The most common presenting signs or symptoms were jaundice [60%], abdominal mass [23.4%] and constitutional symptoms [weakness, anorexia and paleness] [6.7%]. About 10% of patients had an acute hepatitis like clinical presentation. Twenty two children [73.3%] [15 girls, 7 boys, 2.1:1] had AIH type 1 and 4 patients [13.3%] type 2 due to specific autoantibodies. Four children could not be classified. In liver biopsy, 100% of patients had interface hepatitis and fibrosis with or without cirrhosis were found in 60%. In our cohort the prevalence of AIH was 2:1 in girls. Type 1 was the most frequent diagnosis [73.3%] and was more prevalent in older children. Patients with type 2 were younger. The clinical presentation of AIH in children was unspecific and each type could only be differentiated by the determination of the specific autoantibodies