2022
An update on ductal plate malformations and fibropolycystic diseases of the liver
Mirza H, Besse W, Somlo S, Weinreb J, Kenney B, Jain D. An update on ductal plate malformations and fibropolycystic diseases of the liver. Human Pathology 2022, 132: 102-113. PMID: 35777701, DOI: 10.1016/j.humpath.2022.06.022.Peer-Reviewed Original ResearchConceptsDuctal plate malformationLiver diseaseAdult polycystic liver diseaseAutosomal dominant polycystic kidneyFibropolycystic liver diseaseIsolated liver involvementCongenital hepatic fibrosisPolycystic liver diseaseVon Meyenburg complexesGenetic underpinningsMultiple clinical phenotypesFibropolycystic diseasePortal hypertensionCaroli's diseaseLiver involvementLiver cystsMeyenburg complexesHepatic fibrosisFibrocystic lesionsHepatocellular malignanciesCyst enlargementAbnormal organ developmentPolycystic kidneysAnimal modelsHepatocellular malignancy
2015
Total Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: A Biomarker of Disease Progression and Therapeutic Efficacy
Alam A, Dahl NK, Lipschutz JH, Rossetti S, Smith P, Sapir D, Weinstein J, McFarlane P, Bichet DG. Total Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: A Biomarker of Disease Progression and Therapeutic Efficacy. American Journal Of Kidney Diseases 2015, 66: 564-576. PMID: 25960302, DOI: 10.1053/j.ajkd.2015.01.030.Peer-Reviewed Original ResearchConceptsTotal kidney volumeAutosomal dominant polycystic kidney diseaseDisease progressionKidney enlargementKidney functionClinical trialsKidney volumeEnd pointAutosomal dominant polycystic kidneyKidney function measuresPrognostic end pointProgressive kidney enlargementEfficacy end pointGlomerular filtration rateDominant polycystic kidney diseasePolycystic kidney diseaseFluid-filled cystsKidney diseaseFiltration rateCurrent evidencePolycystic kidneysCyst growthFibrotic tissueTherapeutic efficacyTherapeutic interventions
2011
A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation
Fedeles SV, Tian X, Gallagher AR, Mitobe M, Nishio S, Lee SH, Cai Y, Geng L, Crews CM, Somlo S. A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nature Genetics 2011, 43: 639-647. PMID: 21685914, PMCID: PMC3547075, DOI: 10.1038/ng.860.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsApoptosisBlotting, WesternCell ProliferationCystsFemaleGlucosidasesImmunoenzyme TechniquesImmunoprecipitationIntracellular Signaling Peptides and ProteinsLiver DiseasesMaleMiceMice, Inbred C57BLMice, TransgenicMutationPolycystic Kidney DiseasesReceptors, Cell SurfaceTRPP Cation Channels
2004
PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells
Zhang MZ, Mai W, Li C, Cho SY, Hao C, Moeckel G, Zhao R, Kim I, Wang J, Xiong H, Wang H, Sato Y, Wu Y, Nakanuma Y, Lilova M, Pei Y, Harris RC, Li S, Coffey RJ, Sun L, Wu D, Chen XZ, Breyer MD, Zhao ZJ, McKanna JA, Wu G. PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells. Proceedings Of The National Academy Of Sciences Of The United States Of America 2004, 101: 2311-2316. PMID: 14983006, PMCID: PMC356947, DOI: 10.1073/pnas.0400073101.Peer-Reviewed Original ResearchConceptsAutosomal recessive polycystic kidney diseasePolycystic kidney diseaseKidney diseaseEpithelial cellsRecessive polycystic kidney diseaseHuman autosomal recessive polycystic kidney diseaseCultured renal cellsHepatic disease 1 (PKHD1) genePulmonary bronchiPCK rat kidneysRat modelDisease associatesPCK ratsPolycystic kidneysRenal epithelial cellsType 2Pathogenic basisRenal cellsRat kidneyPrimary ciliaHepatic cellsPolyclonal AbKidneyGene productsRats
1989
Hepatic fibropolycystic disease in Mexico. Study of 82 cases.
Torres-Barrera G, García-Tsao G, Quiroz F, Ferrari. Hepatic fibropolycystic disease in Mexico. Study of 82 cases. Revista De Investigación Clínica 1989, 41: 45-52. PMID: 2727432.Peer-Reviewed Original ResearchConceptsCongenital hepatic fibrosisFibropolycystic diseaseLiver function testsPolycystic liver diseaseHepatic fibrosisCholedochal cystCaroli's diseaseLiver diseaseFunction testsMean agePolycystic kidneysInstituto Nacional de la NutriciónRenal insufficiencyVariceal hemorrhageSalvador ZubiránPolycystic diseaseIsolated diseaseIndividual patientsLarge seriesFibrosisCholangitisDiseasePatientsCystsKidney
1986
Haemophilus parainfluenzae Liver Abscess in a Recipient of a Renal Transplant Who Had Polycystic Disease
Desir G, Helman D, Herlich M, Turka L, Bia M. Haemophilus parainfluenzae Liver Abscess in a Recipient of a Renal Transplant Who Had Polycystic Disease. JAMA 1986, 255: 1878-1878. PMID: 3512876, DOI: 10.1001/jama.1986.03370140076015.Peer-Reviewed Original ResearchConceptsPolycystic kidney diseaseKidney diseaseCyst infectionLiver cystsPersistent hepatitis B surface antigenemiaHepatitis B surface antigenemiaHepatitis B virus infectionLiver cyst infectionB virus infectionRenal cyst infectionAutosomal dominant polycystic kidney diseaseDominant polycystic kidney diseaseOne-month courseImmunosuppressive therapyKidney transplantationSurface antigenemiaRenal failurePercutaneous drainageCurrent admissionVirus infectionPolycystic kidneysThird monthPatientsHaemophilus parainfluenzaeEnteric organisms
1977
Gray scale ultrasonography, computerized tomography, and nephrotomography in evaluation of polycystic kidney and liver disease
Rosenfield A, Allen W, Curtis A, Siegel N, Putman C, Hsia Y, Taylor K. Gray scale ultrasonography, computerized tomography, and nephrotomography in evaluation of polycystic kidney and liver disease. Urology 1977, 9: 436-438. PMID: 855069, DOI: 10.1016/0090-4295(77)90225-4.Peer-Reviewed Original Research
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