2023
S1506 Hepatic Decompensation & High-Intensity Statin Therapy Are Associated with Statin-Induced Rhabdomyolysis in Patients With Cirrhosis
Chen G, Alvarado M, Cohen E, Ventura K, To U. S1506 Hepatic Decompensation & High-Intensity Statin Therapy Are Associated with Statin-Induced Rhabdomyolysis in Patients With Cirrhosis. The American Journal Of Gastroenterology 2023, 118: s1141-s1142. DOI: 10.14309/01.ajg.0000955664.89611.a1.Peer-Reviewed Original Research“Because it is a rare disease…it needs to be brought to attention that there are things out of the norm”: a qualitative study of patient and physician experiences of Wilson disease diagnosis and management in the US
Bailey K, Sahota N, To U, Hedera P. “Because it is a rare disease…it needs to be brought to attention that there are things out of the norm”: a qualitative study of patient and physician experiences of Wilson disease diagnosis and management in the US. Orphanet Journal Of Rare Diseases 2023, 18: 158. PMID: 37349760, PMCID: PMC10288732, DOI: 10.1186/s13023-023-02778-3.Peer-Reviewed Original ResearchConceptsPhysician experienceCoordination of careLow copper dietWilson's disease diagnosisLiver transplantationLifelong treatmentNeurological symptomsHepatic symptomsDefinitive diagnosisDietary regimenInsurance barriersWide patientPsychiatric symptomsPatientsRare diseaseCommunity outreach programsDiagnostic journeyQualitative studyMultidisciplinary teamWD patientsMedicationsMultiple specialtiesPhysiciansDiagnosisSymptoms
2022
Major Depressive Disorder in an International Multisite Wilson Disease Registry
Camarata M, Ala A, Coskun A, Deng Y, Embel V, Gonzalez-Peralta R, Maciejewski K, Patel A, Rubman S, To U, Tomlin R, Schilsky M, Zimbrean P. Major Depressive Disorder in an International Multisite Wilson Disease Registry. Journal Of The Academy Of Consultation-Liaison Psychiatry 2022, 64: 106-117. PMID: 36521682, DOI: 10.1016/j.jaclp.2022.12.001.Peer-Reviewed Original ResearchConceptsMajor depressive disorderWilson's diseaseDepressive disorderLifetime major depressive disorderMental health QOLPhysical health QoLMajor depressive episodeMental health qualityStructured psychiatric evaluationSignificant differencesCross-sectional reportsLiver testsLiver diseaseNeurological assessmentLife scoresClinical correlatesDepressive episodePsychiatric symptomsPsychiatric evaluationDisease RegistrySevere anxietyLaboratory testsLifetime historySignificant associationPatientsPlasma Proteomic Profiling Illustrates Endothelial and Complement Signature in Patients with Alcohol-Associated Cirrhosis
Allen C, Butt A, Zhao X, Li F, Bahel P, Chang C, Chun H, Gu S, Hwa J, Jakab S, Chen B, Garcia-Tsao G, Rinder H, To U, McConnell M, Lee A, Pine A. Plasma Proteomic Profiling Illustrates Endothelial and Complement Signature in Patients with Alcohol-Associated Cirrhosis. Blood 2022, 140: 8382-8383. DOI: 10.1182/blood-2022-162633.Peer-Reviewed Original ResearchTrientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial
Schilsky ML, Czlonkowska A, Zuin M, Cassiman D, Twardowschy C, Poujois A, de Assis A Gondim F, Denk G, Cury RG, Ott P, Moore J, Ala A, D'Inca R, Couchonnal-Bedoya E, D'Hollander K, Dubois N, Kamlin COF, Weiss KH, investigators C, To U, Patel A, Hettiarachchi D, Giorgini A, Monico S, Litwin T, Piechal A, Skowronska M, Lachaux A, Belmalih A, Boogers A, Mohr I, Langel A, Freitas C, Barbosa E, Sandahl T, Gerdes L, Obadia A, Rahli D, Cosgrove J. Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial. The Lancet Gastroenterology & Hepatology 2022, 7: 1092-1102. PMID: 36183738, DOI: 10.1016/s2468-1253(22)00270-9.Peer-Reviewed Original ResearchConceptsUrinary copper excretionNon-inferiority marginWilson's diseaseCopper excretionMean differenceStable Wilson's diseaseHealth care centersOral penicillaminePenicillamine groupPenicillamine intoleranceMaintenance therapyPrimary endpointStable patientsDaily doseStudy treatmentTreat approachClinical assessmentAlanine aminotransferaseBaseline valuesSite investigatorsPatientsInherited disorderExtension periodDiseaseGeneral linear modelSa1384: PATIENT AND PHYSICIAN EXPERIENCES OF WILSON DISEASE (WD) DIAGNOSIS AND MANAGEMENT IN THE USA: A QUALITATIVE STUDY
Bailey K, Sahota N, To U, Hedera P. Sa1384: PATIENT AND PHYSICIAN EXPERIENCES OF WILSON DISEASE (WD) DIAGNOSIS AND MANAGEMENT IN THE USA: A QUALITATIVE STUDY. Gastroenterology 2022, 162: s-1149-s-1150. DOI: 10.1016/s0016-5085(22)63443-7.Peer-Reviewed Original Research
2021
Changes in Ascitic Fluid Polymorphonuclear Cell Count After Antibiotics Are Associated With Mortality in Spontaneous Bacterial Peritonitis
Saffo S, To UK, Santoiemma PP, Laurito M, Haque L, Rabiee A, Verna EC, Angarone MP, Garcia-Tsao G. Changes in Ascitic Fluid Polymorphonuclear Cell Count After Antibiotics Are Associated With Mortality in Spontaneous Bacterial Peritonitis. Clinical Gastroenterology And Hepatology 2021, 20: e1201-e1204. PMID: 34273564, PMCID: PMC11090177, DOI: 10.1016/j.cgh.2021.07.019.Peer-Reviewed Original ResearchRecent advances in Wilson disease
Moini M, To U, Schilsky ML. Recent advances in Wilson disease. Translational Gastroenterology And Hepatology 2021, 6: 21-21. PMID: 33824925, PMCID: PMC7838529, DOI: 10.21037/tgh-2020-02.Peer-Reviewed Original ResearchWilson's diseaseMedical treatmentBis-choline tetrathiomolybdateLife-long therapyCurrent medical treatmentDiagnosis of WDFuture potential therapiesIndividualization of treatmentPre-clinical studiesRare genetic disorderNeurologic involvementMedical therapyOphthalmologic signsAvailable therapiesIndividualizing therapyNew therapiesPotential therapyBlood samplesAnimal studiesUnmet needTherapyNewborn screeningPatientsTreatment monitoringEarly detection
2020
P12 Effect of liver disease, neurological disease and mental health issues on quality of life in patients with wilson disease
Camarata M, Ala A, Maciejewski K, To U, Zimbrean P, Rubman S, Coskun A, Patel A, Wadhwa A, Apdik T, Tomlin R, Deng Y, Gonzalez-Peralta R, Schilsky M. P12 Effect of liver disease, neurological disease and mental health issues on quality of life in patients with wilson disease. Gut 2020, 69: a13-a13. DOI: 10.1136/gutjnl-2020-basl.23.Peer-Reviewed Original ResearchMental health issuesWilson's diseaseHealth issuesSeverity of liverMental health QOLPhysical health QoLReview of imagingP-QOL scoresQuality of lifeFIB4 scoreAdult patientsRegistry studyNeurological assessmentPhysical health issuesChronic diseasesOutcome measuresPatientsNeurological diseasesWD patientsQoLAssess qualityDiseaseCirrhosisSignificant differencesScoresP13 Major depressive disorder in patients with wilson’s disease: relationship with liver disease, neurological disease and quality of life
Camarata M, Ala A, Maciejewski K, To U, Zimbrean P, Rubman S, Patel A, Wadhwa A, Coskun A, Apdik T, Tomlin R, Deng Y, Schilsky M. P13 Major depressive disorder in patients with wilson’s disease: relationship with liver disease, neurological disease and quality of life. Gut 2020, 69: a13-a13. DOI: 10.1136/gutjnl-2020-basl.24.Peer-Reviewed Original ResearchMajor depressive disorderQuality of lifeMental health QOLWilson's diseaseNeurological diseasesLiver diseaseDepressive disorderFirst presentationExact testDepression symptomologySeverity of liverLifetime major depressive disorderLiver disease severityFisher's exact testPhysical health QOLSignificant differencesWilcoxon rank sum testMental health problemsRank sum testFIB4 scoreClinical featuresNeurological assessmentPHQ-9PatientsCurrent depressionA window into portal hemodynamics in adult fontan patients?
Simmons MA, Revzin M, To U, Liapakis A, Fahey J, Elder RW. A window into portal hemodynamics in adult fontan patients? International Journal Of Cardiology 2020, 323: 61-64. PMID: 32979426, DOI: 10.1016/j.ijcard.2020.09.047.Peer-Reviewed Original ResearchConceptsAdult Fontan patientsFontan patientsSpleen stiffnessCongenital heart diseaseLiver stiffnessShear wave elastographyControl patientsPortal hemodynamicsLiver diseaseHeart diseaseFontan-associated liver diseaseLimited subgroup analysisPortal venous hemodynamicsAssociated liver diseasePassive hepatic congestionUltrasound shear wave elastographyFontan circulationStudy cohortHealthy patientsHepatic congestionVenous hemodynamicsSubgroup analysisLiver fibrosisHealthy controlsPatientsFRI303 Major depressive disorder in patients with Wilson’s disease: relationship with liver disease, neurological disease and quality of life
Camarata M, Ala A, To U, Zimbrean P, Rubman S, Patel A, Wadhwa A, Maciejewski K, Song X, Deng Y, Tomlin R, Apdik T, Coskun A, Schilsky M. FRI303 Major depressive disorder in patients with Wilson’s disease: relationship with liver disease, neurological disease and quality of life. Journal Of Hepatology 2020, 73: s550-s551. DOI: 10.1016/s0168-8278(20)31573-7.Peer-Reviewed Original Research
2016
Acute-on-chronic liver failure in cirrhosis
Arroyo V, Moreau R, Kamath PS, Jalan R, Ginès P, Nevens F, Fernández J, To U, García-Tsao G, Schnabl B. Acute-on-chronic liver failure in cirrhosis. Nature Reviews Disease Primers 2016, 2: 16041. PMID: 27277335, DOI: 10.1038/nrdp.2016.41.Peer-Reviewed Original ResearchConceptsChronic liver failureOrgan failureAcute decompensationLiver failureHigh short-term mortalityACLF grade 2ACLF grade 3Short-term mortalityAcute liver damageCommon organ failureLiver transplantationRespiratory failureRenal failureStandard therapySystemic inflammationViral hepatitisIntestinal translocationLiver damageACLFGrade 3Consortium guidelinesMortality ratePatientsGrade 2Bacterial products