Fibrosis, biomarkers and liver biopsy in AAT deficiency and relation to liver Z protein polymer accumulation.

Background And Aims: The course of adults with ZZ alpha-1-antitrypsin deficiency (AATD) liver disease is unpredictable. The utility of markers, including liver biopsy, is undefined.

Methods: A prospective cohort, including protocol liver biopsies, was enrolled to address these questions.

Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease.

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.

Settler Witnessing at the Truth and Reconciliation Commission of Canada.

This article offers an account of settler witnessing of residential school survivor testimony that avoids the politics of recognition and the pitfalls of colonial empathy. It knits together the concepts of bearing witness, Indigenous storytelling, and affective reckoning. Following the work of Kelly Oliver, it argues that witnessing involves a reaching beyond ourselves and responsiveness to the agency and self-determination of the other.

Prolonged neonatal jaundice and the diagnosis of biliary atresia: a single-center analysis of trends in age at diagnosis and outcomes.

Age at diagnosis is a modifiable risk factor in outcomes after hepatoportoenterostomy in biliary atresia; however, distinguishing biliary atresia from other more common causes of prolonged neonatal jaundice can be difficult. To focus attention on diagnosis of biliary atresia, we analyzed secular trends in the age at diagnosis, and other factors that might influence outcome. We performed a retrospective analysis of 55 consecutive infants with biliary atresia presenting to a single academic pediatric center over 15-year period from 1990 to 2004.