2025
Chapter 30 Wilson disease⊛
To U, Schilsky M. Chapter 30 Wilson disease⊛. 2025, 841-859. DOI: 10.1016/b978-0-443-26711-6.00030-5.Peer-Reviewed Original ResearchWilson's diseaseAutosomal recessive disorderATP7B proteinNegative copper balanceDiverse phenotypesATP7B geneUnexplained liver diseaseLong-term survivalCopper transportBiochemical testsGenetic testingRecessive disorderMetabolic correctionLiver transplantationMedical historyHepatic symptomsLiver diseaseATP7BCopper excretionNeurological assessmentDietary restrictionIncreased excretionCopper balancePsychiatric symptomsExcretion
2023
Examining the Impact of the COVID-19 Pandemic on Racial and Ethnic Disparities in Access to Liver Transplantation: A Retrospective Analysis
Deka A, Pegram D, Schilsky M, Zimbrean P. Examining the Impact of the COVID-19 Pandemic on Racial and Ethnic Disparities in Access to Liver Transplantation: A Retrospective Analysis. Journal Of The Academy Of Consultation-Liaison Psychiatry 2023, 64: s115. DOI: 10.1016/j.jaclp.2023.11.233.Peer-Reviewed Original Research
2021
Coagulopathy, Bleeding Events, and Outcome According to Rotational Thromboelastometry in Patients With Acute Liver Injury/Failure
Stravitz RT, Fontana RJ, Meinzer C, Durkalski‐Mauldin V, Hanje AJ, Olson J, Koch D, Hamid B, Schilsky M, McGuire B, Ganger D, Liou I, Karvellas CJ, Rule JA, Lisman T, Clasen K, Reuben A, Cripps M, Lee WM, Group A. Coagulopathy, Bleeding Events, and Outcome According to Rotational Thromboelastometry in Patients With Acute Liver Injury/Failure. Hepatology 2021, 74: 937-949. PMID: 33636020, PMCID: PMC10668528, DOI: 10.1002/hep.31767.Peer-Reviewed Original ResearchConceptsALI/ALFROTEM parametersRotational thromboelastometryDisease severityAcute liver injury/failureAbnormal hemostatic profileSevere systemic complicationsAcute liver injuryBleeding eventsBleeding riskLiver transplantationSystemic complicationsHemostatic failureLiver injuryIllness severityNative liverAbnormal hemostasisHemostatic profileStudy groupHigh incidencePatientsNormal rangeLaboratory evidenceClot formationClot stability
2020
Inherited diseases of copper metabolism: Wilson’s disease and Menkes’ disease
Schilsky M, Mistry P. Inherited diseases of copper metabolism: Wilson’s disease and Menkes’ disease. 2020, 2115-2120. DOI: 10.1093/med/9780198746690.003.0234.Peer-Reviewed Original ResearchLiver diseaseCopper excretionHigh urinary copper excretionWilson's diseaseDecompensated liver diseasePrevious affected siblingFulminant hepatic failureUrinary copper excretionKayser-Fleischer ringsConnective tissue disordersLow serum ceruloplasminAge 3 yearsFunction mutationsDefective copper transportAutosomal recessive lossLiver copper contentSingle biochemical testFlorid presentationLiver transplantationHepatic failureMedical therapyClinical presentationClinical findingsUncommon disorderTypical presentation
2019
Chapter 15 General Considerations and the Need for Liver Transplantation
Camarata M, Weiss K, Schilsky M. Chapter 15 General Considerations and the Need for Liver Transplantation. 2019, 173-182. DOI: 10.1016/b978-0-12-811077-5.00015-3.Peer-Reviewed Original ResearchAcute liver failureLiver transplantationWilson's diseaseLiver failureMedical therapyUse of LTEnd-stage liver diseaseLive donor transplantsOnly viable treatment optionViable treatment optionCombination of dietRare autosomal recessive disorderAlternative transplant strategyWilson's disease patientsAutosomal recessive disorderAuxiliary transplantInitial presentationLiver diseaseMedical managementNeurological symptomsTransplant strategiesTreatment optionsDisease patientsDonor poolDisease progression
2018
Liver Transplantation for Wilson Disease
Miloh T, Schilsky M. Liver Transplantation for Wilson Disease. Clinical Gastroenterology 2018, 183-202. DOI: 10.1007/978-3-319-91527-2_11.Peer-Reviewed Original ResearchAcute liver failureLiver transplantationLiver failureWilson's diseaseChronic end-stage liver diseaseEnd-stage liver disease (MELD) scoreNon-resectable liver cancerEnd-stage liver diseaseHeterotopic liver transplantationLiver Disease scoreRisk of morbidityStatus 1APortal hypertensionLiver donationLiver diseaseLiver functionCell transplantationLifelong immunosuppressionTransplant surgeryChronic diseasesDonor organsLiver cancerWait listDisease scorePatientsTreatment Options for Wilson Disease
Kelly C, Pericleous M, Schilsky M. Treatment Options for Wilson Disease. Clinical Gastroenterology 2018, 45-61. DOI: 10.1007/978-3-319-91527-2_3.Peer-Reviewed Original ResearchWilson's diseaseMedical therapyTreatment of WDAsymptomatic patientsLiver transplantationSymptomatic patientsZinc therapyTreatment optionsPhysical therapyPhysician preferenceCopper intakeTherapyDietary changesPatientsMaintenance phaseTreatmentDiseaseCopper chelatorTransplantationDiagnosisIntakeSymptomatologyChapter 19 Wilson Disease and Related Disorders
Schilsky M. Chapter 19 Wilson Disease and Related Disorders. 2018, 253-268. DOI: 10.1016/b978-0-323-47874-8.00019-5.Peer-Reviewed Original ResearchMedical therapyWilson's diseaseRegression of diseaseCentral nervous systemLiver transplantationLiver failureLiver injuryDisease progressionNervous systemRelated disordersThird decadeDiseaseGenetic disordersCopper metabolismTherapyDisordersSecond decadeCopper accumulationCirrhosisTransplantationInjuryDiagnosisLiverProgression
2017
Wilson Disease
Schilsky M, Ala A. Wilson Disease. 2017, 799-819. DOI: 10.1002/9781119251316.ch29.Peer-Reviewed Original ResearchWilson's diseaseExcellent patient survivalAcute liver failureCentral nervous systemAsymptomatic patientsBiliary copper excretionLiver transplantationPharmacologic treatmentSymptomatic patientsHepatic insufficiencyLiver failureMedical therapyLiver diseasePatient survivalHepatic diseasePsychiatric symptomsNervous systemBiochemical findingsCopper excretionDiseaseInherited disorderDisease-specific mutationsPatientsMutation analysisTreatment
2015
Su1011 Should Liver Transplantation Be Reconsidered in Patients With Intrahepatic Cholangiocarcinoma? A Systematic Review and Meta-Regression Analysis of Epidemiologic Evidence
Njei B, Schilsky M, Ditah I, Taddei T. Su1011 Should Liver Transplantation Be Reconsidered in Patients With Intrahepatic Cholangiocarcinoma? A Systematic Review and Meta-Regression Analysis of Epidemiologic Evidence. Gastroenterology 2015, 148: s-1036. DOI: 10.1016/s0016-5085(15)33541-1.Peer-Reviewed Original Research
2014
Radiology in Transplant
Kim A, Schilsky M. Radiology in Transplant. Current Transplantation Reports 2014, 1: 238-245. DOI: 10.1007/s40472-014-0034-5.Peer-Reviewed Original ResearchMagnetic resonance imagingHepatocellular carcinomaLiver transplantationContrast-enhanced ultrasoundUse of CTNon-invasive diagnosisGraft fibrosisLocoregional treatmentTumor histologyNew imaging techniquesLesion characteristicsNetwork criteriaDonor candidatesLiver lesionsOrgan allocationOrgan procurementResonance imagingLiver imagingEarly detectionTransplantationFibrosisUltrasoundImaging techniquesImagingSteatosis
2013
Human Monoclonal Antibody MBL-HCV1 Delays HCV Viral Rebound Following Liver Transplantation: A Randomized Controlled Study
Chung R, Gordon F, Curry M, Schiano T, Emre S, Corey K, Markmann J, Hertl M, Pomposelli J, Pomfret E, Florman S, Schilsky M, Broering T, Finberg R, Szabo G, Zamore P, Khettry U, Babcock G, Ambrosino D, Leav B, Leney M, Smith H, Molrine D. Human Monoclonal Antibody MBL-HCV1 Delays HCV Viral Rebound Following Liver Transplantation: A Randomized Controlled Study. American Journal Of Transplantation 2013, 13: 1047-1054. PMID: 23356386, PMCID: PMC3618536, DOI: 10.1111/ajt.12083.Peer-Reviewed Original ResearchConceptsLiver transplantationHepatitis C virusViral reboundViral loadPlacebo-controlled pilot studyAntibody-treated groupDays of transplantResistance-associated variantsHCV genotype 1aHuman monoclonal antibodyAllograft infectionRibavirin therapyPlacebo groupActing antiviralsPlacebo treatmentMedian timeViral clearanceSingle infusionC virusHCV E2Median changeGenotype 1aLimited efficacyDay 1Day 3
2011
Wilson Disease
Schilsky M, Tavill A. Wilson Disease. 2011, 803-824. DOI: 10.1002/9781119950509.ch29.Peer-Reviewed Original ResearchWilson's diseaseExcellent patient survivalSevere hepatic insufficiencyAcute liver failureUrine copper excretionKayser-Fleischer ringsHepatic copper concentrationAsymptomatic patientsLiver transplantationPharmacologic treatmentSymptomatic patientsHepatic insufficiencyLiver failureMedical therapyPatient survivalDisease progressionBiliary excretionBiochemical findingsToxic copper accumulationCopper excretionPatientsDiseaseCopper-transporting adenosine triphosphataseDisease-specific mutationsGenetic disordersWilson Disease: Pathogenesis and Clinical Considerations in Diagnosis and Treatment
Rosencrantz R, Schilsky M. Wilson Disease: Pathogenesis and Clinical Considerations in Diagnosis and Treatment. Seminars In Liver Disease 2011, 31: 245-259. PMID: 21901655, DOI: 10.1055/s-0031-1286056.Peer-Reviewed Original ResearchConceptsPathologic findingsWilson's diseaseHepatocyte cell transplantationLife-long treatmentInitiation of treatmentKayser-Fleischer ringsFirst-degree relativesPresence of signsBiliary copper excretionLiver transplantationMedical therapyLow ceruloplasminCell transplantationElevated urineLenticular degenerationHistologic changesDisease progressionClinical signsFatal disorderNeurologic diseaseAnimal modelsClinical considerationsCopper excretionDiseaseEarly detection
2010
Wilson’s Disease
Schilsky M, Mitchell K. Wilson’s Disease. Molecular Pathology Library 2010, 5: 655-663. DOI: 10.1007/978-1-4419-7107-4_43.Peer-Reviewed Original ResearchWilson's diseaseAcute hepatic necrosisFuture human studiesAutosomal recessive disorderLiver transplantationClinical findingsHepatic necrosisPathological changesFuture therapiesAnimal modelsHuman studiesHepatocyte transplantationMolecular pathogenesisUltrastructural evaluationLiver tissueDiseaseClinical methodsRecessive disorderTransplantationCopper metabolismPathogenesisTherapyGene therapyDisordersMolecular diagnostics
2009
ICU Management of Acute Liver Failure
Schilsky ML, Honiden S, Arnott L, Emre S. ICU Management of Acute Liver Failure. Clinics In Chest Medicine 2009, 30: 71-87. PMID: 19186281, DOI: 10.1016/j.ccm.2008.10.001.Peer-Reviewed Original ResearchConceptsAcute liver failureLiver failureTeam approachGood ICU careOrganized team approachAcute liver injurySurvival of patientsBetter patient outcomesEarly disease recognitionLiver transplantationICU careLiver injuryICU managementPatient outcomesCritical carePatient managementSupportive measuresPatientsCareDisease recognitionICUTransplantationFailurePathophysiologyInjury
2008
Screening for Wilson disease in acute liver failure: A comparison of currently available diagnostic tests
Korman JD, Volenberg I, Balko J, Webster J, Schiodt FV, Squires RH, Fontana RJ, Lee WM, Schilsky ML, Groups P. Screening for Wilson disease in acute liver failure: A comparison of currently available diagnostic tests. Hepatology 2008, 48: 1167-1174. PMID: 18798336, PMCID: PMC4881751, DOI: 10.1002/hep.22446.Peer-Reviewed Original ResearchConceptsAcute liver failureDiagnosis of ALFSerum copper levelsFulminant Wilson's diseaseWilson's diseaseLiver failureDiagnostic sensitivityEmergency liver transplantationChronic liver diseaseAlkaline phosphataseAvailable diagnostic testsCopper levelsAvailable laboratory testsALF patientsTransplant listingLikelihood ratioLiver transplantationSerum aminotransferasesLiver diseaseALT ratioSerum ceruloplasminBilirubin ratioPatientsSerum CpRapid diagnosis
2005
Survival after liver transplantation in patients with hepatic iron overload: the national hemochromatosis transplant registry.
Kowdley K, Brandhagen D, Gish R, Bass N, Weinstein J, Schilsky M, Fontana R, McCashland T, Cotler S, Bacon B, Keeffe E, Gordon F, Polissar N. Survival after liver transplantation in patients with hepatic iron overload: the national hemochromatosis transplant registry. Gastroenterology 2005, 129: 494-503. PMID: 16083706, DOI: 10.1016/j.gastro.2005.05.004.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedAnalysis of VarianceCause of DeathCohort StudiesConfidence IntervalsFemaleGenetic MarkersGenotypeHemochromatosisHistocompatibility Antigens Class IHumansIron OverloadLiver Function TestsLiver TransplantationMaleMiddle AgedMutationProbabilityPrognosisProportional Hazards ModelsRegistriesSensitivity and SpecificitySeverity of Illness IndexSurvival AnalysisConceptsHepatic iron overloadHepatic iron indexHepatic iron concentrationLiver transplantationIron overloadPosttransplantation survivalC282Y/H63D mutationsEnd-stage liver diseaseHFE gene testingHFE mutation statusLiver transplantation centerNon-HH patientsOrgan Sharing statusYear of transplantationWild-type patientsPostliver transplantationTransplant RegistryHazard ratioTransplantation centersUncontrolled studiesLiver diseasePoor outcomeUnited NetworkIron indicesEarly diagnosis
2001
ORTHOTOPIC LIVER TRANSPLANTATION FOR WILSON’S DISEASE
Emre S, Atillasoy E, Ozdemir S, Schilsky M, Varma C, Thung S, Sternlieb I, Guy S, Sheiner P, Schwartz M, Miller C. ORTHOTOPIC LIVER TRANSPLANTATION FOR WILSON’S DISEASE. Transplantation 2001, 72: 1232-1236. PMID: 11602847, DOI: 10.1097/00007890-200110150-00008.Peer-Reviewed Original ResearchConceptsOrthotopic liver transplantationOne-year patientFulminant Wilson's diseaseLiver transplantationWilson's diseaseGraft survivalRenal failureDisease cureAcute renal failureLate postoperative complicationsChronic liver diseaseFulminant liver failureNormal liver functionLong-term survivalHepatic complicationsPost-OLTBiliary copper excretionPostoperative complicationsPatient agePatient demographicsSupportive careDisease recurrenceLiver failureLiver injuryMedical therapy
1999
Wilson’s disease
Tavill A, Schilsky M. Wilson’s disease. Current Treatment Options In Gastroenterology 1999, 2: 68-71. PMID: 11096575, DOI: 10.1007/s11938-999-0021-6.Peer-Reviewed Original ResearchPharmacologic therapyWilson's diseaseOrthotopic liver transplantationFulminant hepatic failureLiver transplantationCompliant patientsPreventive therapyHepatic failureHepatic insufficiencyMedical therapyOrgan damageMaintenance treatmentTherapyPatientsDiseaseTransplantationPrognosisInsufficiencyDiagnosis