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
Treating Other Symptoms of Wilson Disease: The Liver
Camarata M, Schilsky M. Treating Other Symptoms of Wilson Disease: The Liver. Clinical Gastroenterology 2018, 87-106. DOI: 10.1007/978-3-319-91527-2_5.Peer-Reviewed Original ResearchWilson's diseaseLiver diseaseLiver cancerEnd-stage liver diseaseAcute liver failureMinority of patientsTime of diagnosisRoutine laboratory testingWilson's disease patientsPortal hypertensionInflamed liverLiver failurePrompt initiationAppropriate therapyChronic inflammationDisease patientsFamily screeningDisease progressionTimely initiationSevere symptomsPathological accumulationCirrhosisPatientsDiseaseLiverChapter 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
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