2023
Liver Transplantation for Mahvash Disease, an Inborn Error of Metabolism
Mistry P, Garcia-Tsao G. Liver Transplantation for Mahvash Disease, an Inborn Error of Metabolism. New England Journal Of Medicine 2023, 389: 2010-2013. PMID: 37991861, DOI: 10.1056/nejme2310332.Peer-Reviewed Original Research
2022
Transjugular Intrahepatic Portosystemic Shunt for Refractory Ascites in Gaucher Disease
Adhyaru K, Menezes S, Mistry PK, Nagral A, Mistry P. Transjugular Intrahepatic Portosystemic Shunt for Refractory Ascites in Gaucher Disease. Cureus 2022, 14: e23941. PMID: 35535294, PMCID: PMC9079779, DOI: 10.7759/cureus.23941.Peer-Reviewed Original ResearchHepatic venous pressure gradientGaucher diseaseRefractory ascitesTransjugular intrahepatic portosystemic shuntTransjugular intrahepatic portosystemic shunt (TIPS) procedureAdvanced hepatic diseaseVenous pressure gradientIntrahepatic portosystemic shuntAnterior abdominal wallPortosystemic shunt procedureEnzyme replacement therapyAvascular osteonecrosisLiver transplantationLysosomal storage disorderPortal hypertensionVariceal bleedingSevere cytopeniaHepatic encephalopathyVenous collateralsLiver diseaseShunt procedurePortosystemic shuntReplacement therapyHepatic diseaseAbdominal wall
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
2011
Haematological Disorders of the Liver
Mistry P, Jain D. Haematological Disorders of the Liver. 2011, 48-69. DOI: 10.1002/9781444341294.ch4.Peer-Reviewed Original ResearchLiver diseaseHaematological disordersEnd-stage liver diseasePrimary haematological disordersPrimary liver diseaseAcute liver failureMajor clinical significanceE hepatitisLiver transplantationLiver involvementLiver failureNormal physiological interactionsErythrocyte dysfunctionClinical significanceAbnormal plateletsHaematological effectsBone marrowCoagulation pathwayDiseaseDisease statesMembrane abnormalitiesStorage diseaseDisordersLiverPhysiological interactions
2010
Long-Term Outcomes of Liver Transplantation in Type 1 Gaucher Disease
Ayto RM, Hughes DA, Jeevaratnam P, Rolles K, Burroughs AK, Mistry PK, Mehta AB, Pastores GM. Long-Term Outcomes of Liver Transplantation in Type 1 Gaucher Disease. American Journal Of Transplantation 2010, 10: 1934-1939. PMID: 20659098, DOI: 10.1111/j.1600-6143.2010.03168.x.Peer-Reviewed Original ResearchConceptsEnzyme replacement therapyLiver transplantationGaucher diseaseLong-term enzyme replacement therapyEnd-stage liver diseaseType 1 Gaucher's diseaseSevere hepatic impairmentOrthotopic liver transplantationSuccessful liver transplantationLife-saving treatmentPrevalent lysosomal storage disorderHepatic impairmentLysosomal storage disorderYears postprocedureLiver failureLiver diseaseTerm outcomesReplacement therapyExcellent outcomesDemonstrable efficacyPatientsTransplantationPathological manifestationsDiseaseStorage disorderLiver transplantation for inherited metabolic disorders of the liver
Moini M, Mistry P, Schilsky ML. Liver transplantation for inherited metabolic disorders of the liver. Current Opinion In Organ Transplantation 2010, 15: 269-276. PMID: 20489626, DOI: 10.1097/mot.0b013e3283399dbd.Peer-Reviewed Original ResearchConceptsLiver transplantExtrahepatic diseaseLiver transplantationGlycogen storage disease type ICareful patient selectionOutcome of transplantationImportant therapeutic modalityMultisystemic genetic disorderMajor hepatic diseasesUrea cycle defectsGene replacement therapyHepatic complicationsSurgical managementLiver replacementMetabolic managementPatient selectionPrimary hyperoxaluriaReplacement therapyHepatic diseaseImproved outcomesTherapeutic modalitiesMetabolic disordersMetabolic diseasesTransplantLiver results
2005
Hepatocellular Carcinoma in Type 1 Gaucher Disease: A Case Report with Review of the Literature
Xu R, Mistry P, Mckenna G, Emre S, Schiano T, Bu-Ghanim M, Levi G, Fiel M. Hepatocellular Carcinoma in Type 1 Gaucher Disease: A Case Report with Review of the Literature. Seminars In Liver Disease 2005, 25: 226-229. PMID: 15918150, DOI: 10.1055/s-2005-871201.Peer-Reviewed Original ResearchConceptsType 1 Gaucher diseaseHepatocellular carcinomaGaucher diseaseLiver transplantationInborn errorsType 1 Gaucher's diseaseNecessitating liver transplantationNon-neuronopathic diseaseDevelopment of cirrhosisMajority of patientsStandard of careRisk of malignancyEnzyme replacement therapyDeficiency of glucocerebrosidaseRare inborn errorVariety of neoplasmsAutosomal recessive disorderMarrow infiltrationPulmonary diseaseSupplemental therapyReplacement therapyCase reportLysosomal storage diseaseTissue macrophagesType 2
1998
Ulcerative colitis has an aggressive course after orthotopic liver transplantation for primary sclerosing cholangitis
Papatheodoridis GV, Hamilton M, Mistry PK, Davidson B, Rolles K, Burroughs AK. Ulcerative colitis has an aggressive course after orthotopic liver transplantation for primary sclerosing cholangitis. Gut 1998, 43: 639. PMID: 9824344, PMCID: PMC1727300, DOI: 10.1136/gut.43.5.639.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisInflammatory bowel diseaseLong-term steroidsUlcerative colitisSclerosing cholangitisBowel diseaseLiver transplantationAggressive courseTerm steroidsDe novo ulcerative colitisNew-onset ulcerative colitisInflammatory bowel disease activityBowel disease activityOnset ulcerative colitisOrthotopic liver transplantationEarly colorectal neoplasmsMaintenance immunosuppressionQuiescent courseDisease activityTransplant patientsPrevious colectomyColorectal cancerColorectal neoplasmsColitisPatientsOutcome of orthotopic liver transplantation in patients with haemophilia
Gordon FH, Mistry PK, Sabin CA, Lee CA. Outcome of orthotopic liver transplantation in patients with haemophilia. Gut 1998, 42: 744. PMID: 9659174, PMCID: PMC1727096, DOI: 10.1136/gut.42.5.744.Peer-Reviewed Original ResearchConceptsOrthotopic liver transplantationHepatitis C cirrhosisHuman immunodeficiency virusLiver transplantationC cirrhosisViral hepatitisHepatocellular carcinomaHIV-negative recipientsRecurrent viral hepatitisChronic viral hepatitisHIV-positive recipientsKaplan-Meier analysisLong-term outcomesLong-term cureMedical database searchHaemophilic menPositive recipientsHepatitis BBiliary atresiaCommon indicationDisease recurrenceHIV infectionTransplant centersMedian timeNegative recipients
1990
Intravenous albumin infusion is an effective therapy for hyponatraemia in cirrhotic patients with ascites.
McCormick PA, Mistry P, Kaye G, Burroughs AK, McIntyre N. Intravenous albumin infusion is an effective therapy for hyponatraemia in cirrhotic patients with ascites. Gut 1990, 31: 204. PMID: 2311979, PMCID: PMC1378381, DOI: 10.1136/gut.31.2.204.Peer-Reviewed Original ResearchConceptsIntravenous albumin infusionAlbumin infusionEffective therapyDecompensated liver diseaseFulminant hepatitis BLiver transplantationCirrhotic patientsHepatitis BSevere hyponatraemiaLiver diseaseIntravenous infusionHyponatraemiaPatientsPreliminary experienceInfusionCirrhosisAscitesTherapyTreatmentSurgeryTransplantationDisease