the aim of this study was to evaluate the clinical, laboratory and histopathological characteristics and the response to immunosuppression in children and adolescents with autoimmune hepatitis (AIH).
Methodsthe present research is a descriptive study consisting of 39 children and adolescents with AIH who receive care at the Department of Pediatric Gastroenterology of Hospital das Clínicas (UFMG) from 1986 to 1998.
Resultschildren's age ranged from 1.6 to 17 years (mean 8.7 ± 3.49), most of them were females (87.2%). There were three types of clinical presentations: chronic (53.9%), acute (41%), and serious hepatic failure (5.1%). The most relevant laboratory parameters were the aminotransferases and g-globulin increase. Antinuclear antibodies were positive in 66.7% of the patients, while smooth muscle antibodies were positive in 52.8% and anti-LKM1 in 3% of the patients. In the histopathology the most important findings were the piecemeal necrosis (93.7%), moderate to severe portal inflammation (78.1%), definitive or incomplete cirrhosis (76.9%), absence of lesion of biliary ducts (93.7%) and presence of rosettes (90.6%). During the treatment, 77.8% obtained complete resolution, associated to side effects in 27.8% of them. Seven patients died (17.9%). During the treatment there was significant z score reduction (p< 0.05) for height/age.
Conclusionsafter carrying out this study, we observed that the typical characteristics of AIH were: female sex, several clinical presentations, increased aminotransferase, and hypergammaglobulinemia. Histopathology showed a predominance of incipient and/or definitive cirrhosis associated with moderate to severe portal inflammation and piecemeal necrosis. Treatment using corticosteroids and azathioprine, turned out to be effective. However, the reduction in the height/age z score probably represents an adverse effect of corticoid treatment.
avaliar o quadro clínico, laboratorial, histopatológico e a resposta ao tratamento imunossupressor em crianças e adolescentes com hepatite auto-imune.
Métodosestudo descritivo de trinta e nove crianças e adolescentes, atendidos no setor de gastroenterologia pediátrica do Hospital das Clínicas da UFMG, no período de 1986 a 1998.
Resultadosa idade variou de 1,6 a 17 anos (média 8,7±3,49), com predomínio do sexo feminino (87,2%). Ocorreram três formas de apresentação clínica: crônica (53,9%), aguda (41%) e falência hepática grave (5,1%). A alteração laboratorial mais relevante foi a elevação das aminotransferases e gama-globulina. O fator antinuclear foi positivo em 66,7% dos pacientes, antimúsculo liso em 52,8%, e o antimicrossomal de fígado e rim em 3%. Na histopatologia, destacaram-se a necrose em saca-bocados (93,7%), inflamação portal moderada a acentuada (78,1%), cirrose definitiva e/ou incompleta (76,9%), ausência de lesões dos ductos biliares (93,7%) e presença de rosetas (90,6%). No tratamento, 77,8% obtiveram resposta completa, com efeitos colaterais em 27,8% dos pacientes. Ocorreram sete (17,9%) óbitos. No decurso do tratamento, ocorreu redução com significância estatítica do escore z para estatura/idade.
Conclusõesa hepatite auto-imune predomina no sexo feminino, com formas de apresentação clínica variada, presença de auto-anticorpos, elevação das aminotransferases e hipergamaglobulinemia. Predomínio histopatológico de cirrose incipiente e/ou definitiva, com inflamação portal de moderada a acentuada e necrose em saca-bocados. O tratamento com corticosteróide e azatioprina em doses diárias revelou-se efetivo, todavia, a diminuição do escore z para estatura/idade representa, provavelmente, um efeito indesejável do corticóide.