2023
A quality improvement intervention to decrease the decline in renal function in pediatric liver transplant recipients
Batsis I, Elisofon S, Ferguson M, Jonas M, Kimball B, Lee C, Mitchell P, Fawaz R. A quality improvement intervention to decrease the decline in renal function in pediatric liver transplant recipients. Pediatric Transplantation 2023, 27: e14506. PMID: 36938904, DOI: 10.1111/petr.14506.Peer-Reviewed Original ResearchConceptsPrevalence of eGFRPost-intervention periodPediatric liver transplant recipientsYear post-LTLiver transplant recipientsChronic kidney diseasePost-LTTransplant recipientsSignificant non-modifiable risk factorNon-modifiable risk factorsChildren post-LTLong-term morbidityBlood pressure percentilesGlomerular filtration rateQuality improvement interventionsQuality improvement projectHospital dischargeRenal functionMonth 24Kidney diseaseFiltration rateRisk factorsInclusion criteriaQI interventionsMetabolic disorders
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 studyComparing Serum Matrix Metalloproteinase-7 in Parenteral Nutrition-Associated Liver Disease and Biliary Atresia
Salvi PS, Fawaz R, Cowles RA. Comparing Serum Matrix Metalloproteinase-7 in Parenteral Nutrition-Associated Liver Disease and Biliary Atresia. The Journal Of Pediatrics 2022, 249: 97-100. PMID: 35714967, DOI: 10.1016/j.jpeds.2022.06.014.Peer-Reviewed Original ResearchConceptsBiliary atresiaLiver diseaseParenteral nutrition-associated liver diseaseParenteral Nutrition–Associated Liver DiseaseMetalloproteinase-7Matrix metalloproteinase 7 levelsSerum matrix metalloproteinase-7Cross-sectional studyMatrix metalloproteinase-7Parenteral nutritionInvasive testingAtresiaInfantsDiseaseJaundice
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