2011
Wilson 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
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
1991
Prognosis of Wilsonian chronic active hepatitis
Schilsky M, Scheinberg I, Sternlieb I. Prognosis of Wilsonian chronic active hepatitis. Gastroenterology 1991, 100: 762-767. PMID: 1993498, DOI: 10.1016/0016-5085(91)80023-3.Peer-Reviewed Original ResearchConceptsChronic active hepatitisActive hepatitisWilson's diseaseD-penicillamineAbnormal water retentionPresence of cirrhosisMonths of treatmentSuccessful pharmacological treatmentMajority of subjectsLiver transplantSalt restrictionLaboratory featuresSymptomatic improvementLiver diseaseTherapeutic regimenPharmacological treatmentAlanine aminotransferasePatientsAspartate aminotransferaseHepatitisNormal levelsDiseaseCirrhosisTrientinePrognosis