RESUMO
BACKGROUND: Children with autoimmune hepatitis (AIH) often exhibit particular features. Accordingly, seven pediatric-specific criteria have been proposed. AIM: To develop a prediction model based on them, transform it into a scoring system and study its accuracy. METHODS: A cohort of children under study for liver disease was consecutively selected. AIH diagnosis was based on classical criteria. Already proposed pediatric criteria were recorded. The best possible regression model was selected, and the beta coefficient of each criterion was translated into a whole number (points). Total scores were obtained following the points system and the best cut-off was calculated. Subsequently, accuracy of the diagnostic score was studied in the validation set. RESULTS: Among 212 included patients, 100 had AIH. The score included 5 criteria: autoantibodies (0-2 points), hypergammaglobulinemia, exclusion of viral hepatitis, exclusion of Wilson's disease (1 point each) and liver histology (3 points). In addition, a normal cholangiogram is mandatory. The validation set was formed of 70 patients (24 with AIH). In this subsample, a score of ≥6 renders a sensitivity/specificity of 95.8%/100%. The area under the receiver operating characteristic curve was 97.1%. CONCLUSION: Pediatric-specific criteria for the diagnosis of AIH can be reliably used as a scoring system.
Assuntos
Hepatite Autoimune/diagnóstico , Autoanticorpos/sangue , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Hipergamaglobulinemia/sangue , Masculino , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
PURPOSE: Classical criteria for diagnosis of autoimmune hepatitis (AIH) are intended as research tool and are difficult to apply at patient's bedside. We aimed to study the accuracy of simplified criteria and the concordance with the expert diagnosis based on the original criteria. METHODS: A cohort of children under study for liver disorder was selected through consecutive sampling to obtain the prevalence of AIH within the group of differential diagnoses. AIH was defined, based on classical criteria, through committee review of medical reports. Validity indicators of the simplified criteria were obtained in an intention to diagnose approach. Optimal cut-off and the area under the receiver operating characteristic (ROC) curve were calculated. RESULTS: Out of 212 cases reviewed, 47.2% were AIH. For the optimal cut-off (6 points), the simplified criteria showed a sensitivity of 72.0% and a specificity of 96.4%, with a 94.7% positive and a 79.4% negative predictive value. The area under the ROC curve was 94.3%. There was a good agreement in the pre-treatment concordance between the classical and the simplified criteria (kappa index, 0.775). CONCLUSION: Simplified criteria provide a moderate sensitivity for the diagnosis of AIH, but may help in indicating treatment in cases under suspicion with 6 or more points.
Assuntos
Circulação Extracorpórea/métodos , Falência Hepática Aguda/terapia , Transplante de Fígado , Desintoxicação por Sorção/métodos , Fatores Etários , Criança , Pré-Escolar , Circulação Extracorpórea/efeitos adversos , Feminino , Humanos , Lactente , Falência Hepática Aguda/sangue , Falência Hepática Aguda/diagnóstico , Masculino , Fatores de Risco , Índice de Gravidade de Doença , Desintoxicação por Sorção/efeitos adversos , Fatores de Tempo , Resultado do Tratamento , Listas de EsperaRESUMO
No disponible
Assuntos
Humanos , Masculino , Recém-Nascido , Adulto , Síndrome de Alagille/complicações , Atresia Intestinal/complicações , Doenças Genéticas Inatas/diagnósticoAssuntos
Síndrome de Alagille/complicações , Atresia Intestinal/complicações , Adulto , Síndrome de Alagille/diagnóstico , Síndrome de Alagille/genética , Síndrome de Alagille/cirurgia , Biópsia , Colestase/diagnóstico , Colestase/etiologia , Códon sem Sentido , Facies , Humanos , Recém-Nascido , Atresia Intestinal/cirurgia , Proteína Jagged-1/genética , Fígado/patologia , Transplante de Fígado , Masculino , Nutrição Parenteral , FenótipoRESUMO
No disponible
