RESUMO
BACKGROUND AND AIMS: Detailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis. APPROACH AND RESULTS: A targeted enzyme-linked immunosorbent assay-based panel of nine biomarkers (lysyl oxidase, tissue inhibitor matrix metalloproteinase (MMP) 1, connective tissue growth factor [CTGF], IL-8, endoglin, periostin, Mac-2-binding protein, MMP-3, and MMP-7) was examined in children with biliary atresia (BA; n = 187), alpha-1 antitrypsin deficiency (A1AT; n = 78), and Alagille syndrome (ALGS; n = 65) and correlated with liver stiffness (LSM) and biochemical measures of liver disease. Median age and LSM were 9 years and 9.5 kPa. After adjusting for covariates, there were positive correlations among LSM and endoglin ( p = 0.04) and IL-8 ( p < 0.001) and MMP-7 ( p < 0.001) in participants with BA. The best prediction model for LSM in BA using clinical and lab measurements had an R2 = 0.437; adding IL-8 and MMP-7 improved R2 to 0.523 and 0.526 (both p < 0.0001). In participants with A1AT, CTGF and LSM were negatively correlated ( p = 0.004); adding CTGF to an LSM prediction model improved R2 from 0.524 to 0.577 ( p = 0.0033). Biomarkers did not correlate with LSM in ALGS. A significant number of biomarker/lab correlations were found in participants with BA but not those with A1AT or ALGS. CONCLUSIONS: Endoglin, IL-8, and MMP-7 significantly correlate with increased LSM in children with BA, whereas CTGF inversely correlates with LSM in participants with A1AT; these biomarkers appear to enhance prediction of LSM beyond clinical tests. Future disease-specific investigations of change in these biomarkers over time and as predictors of clinical outcomes will be important.
Assuntos
Síndrome de Alagille , Colestase , Técnicas de Imagem por Elasticidade , Hepatopatias , Humanos , Criança , Fígado/patologia , Metaloproteinase 7 da Matriz , Endoglina , Interleucina-8 , Colestase/patologia , Cirrose Hepática/diagnóstico , Cirrose Hepática/patologia , Hepatopatias/patologia , Biomarcadores , Síndrome de Alagille/patologiaRESUMO
Se presenta la historia clínica de una paciente portadora de un Divertículo de Duodeno, que consideramos corresponde a una patología bastante rara en nuestro medio, por el hecho de no haberse encontrado ninguna comunicación al respecto, en revisiones que se ha realizado de historias clínicas en el Hospital "Santa Bárbara". Se menciona en la sintomatología el dato importante de la pérdida de peso, dolor constante, con mayor intensidad después de los alimentos y sobre todo nocturno, acompañado de naúseas y vómitos alimenticios, biliares mal olientes, y a veces hematemesisi. se describen los estudios radiográficos que se realizaron para confirmar el diagnóstico clínico. se relata la intervención quirúrgica. Posteriormente se hacen consideraciones sobre la clasificación de los diovertículos del intestino delgado. Con respecto a la frecuencia, a decir varios autores consultados, todos coinciden en la rareza de su presentación. Finalmente se considera de mucha importancia de los procedimientos radiológicos utilizados para confirmar el diagnóstico