2022
Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study
Vandriel S, Li L, She H, Wang J, Gilbert M, Jankowska I, Czubkowski P, Gliwicz‐Miedzińska D, Gonzales E, Jacquemin E, Bouligand J, Spinner N, Loomes K, Piccoli D, D'Antiga L, Nicastro E, Sokal É, Demaret T, Ebel N, Feinstein J, Fawaz R, Nastasio S, Lacaille F, Debray D, Arnell H, Fischler B, Siew S, Stormon M, Karpen S, Romero R, Kim K, Baek W, Hardikar W, Shankar S, Roberts A, Evans H, Jensen M, Kavan M, Sundaram S, Chaidez A, Karthikeyan P, Sanchez M, Cavalieri M, Verkade H, Lee W, Squires J, Hajinicolaou C, Lertudomphonwanit C, Fischer R, Larson‐Nath C, Mozer‐Glassberg Y, Arikan C, Lin H, Bernabeu J, Alam S, Kelly D, Carvalho E, Ferreira C, Indolfi G, Quiros‐Tejeira R, Bulut P, Calvo P, Önal Z, Valentino P, Desai D, Eshun J, Rogalidou M, Dezsőfi A, Wiecek S, Nebbia G, Pinto R, Wolters V, Tamara M, Zizzo A, Garcia J, Schwarz K, Beretta M, Sandahl T, Jimenez‐Rivera C, Kerkar N, Brecelj J, Mujawar Q, Rock N, Busoms C, Karnsakul W, Lurz E, Santos‐Silva E, Blondet N, Bujanda L, Shah U, Thompson R, Hansen B, Kamath B, Group T. Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study. Hepatology 2022, 77: 512-529. PMID: 36036223, PMCID: PMC9869940, DOI: 10.1002/hep.32761.Peer-Reviewed Original ResearchConceptsNative liver survivalLarge international cohortAlagille syndromeInternational cohortNLS rateNative liverLiver diseaseMedian total bilirubin levelsEvent-free survival rateNatural historyEarly biochemical predictorsEvident portal hypertensionLiver-related eventsRisks of transplantationMulticenter retrospective studyTotal bilirubin levelsEvaluation of therapyMonths of ageGALA studyHepatic outcomesLiver survivalPortal hypertensionTertiary centerBilirubin levelsRetrospective study
2021
Liver Pathology, Including MOC31 Immunohistochemistry, in Congenital Tufting Enteropathy
Chen S, Goldsmith J, Fawaz R, Al-Ibraheemi A, Perez-Atayde A, Vargas S. Liver Pathology, Including MOC31 Immunohistochemistry, in Congenital Tufting Enteropathy. The American Journal Of Surgical Pathology 2021, 45: 1091-1097. PMID: 33756496, DOI: 10.1097/pas.0000000000001710.Peer-Reviewed Original ResearchConceptsCTE patientsCongenital tufting enteropathyParenteral nutritionBiliary epitheliumDuctular reactionLiver pathologyChronic hepatitis CHepatitis C patientsEpithelial cell adhesion moleculeLiver core biopsyIntestinal villous atrophyNormal brush borderClinicopathologic materialEpCAM-deficientCore biopsyTufting enteropathyC patientsEpCAM mutationsCell adhesion moleculesHepatitis CLiver biopsyMOC31Affected patientsLobular inflammationPathological findings