2024
Rationale and Design of a Phase 2, Double-blind, Placebo-Controlled, Randomized Trial Evaluating AMP Kinase-Activation by Metformin in Focal Segmental Glomerulosclerosis
Barsotti G, Luciano R, Kumar A, Meliambro K, Kakade V, Tokita J, Naik A, Fu J, Peck E, Pell J, Reghuvaran A, Tanvir E, Patel P, Zhang W, Li F, Moeckel G, Perincheri S, Cantley L, Moledina D, Wilson F, He J, Menon M. Rationale and Design of a Phase 2, Double-blind, Placebo-Controlled, Randomized Trial Evaluating AMP Kinase-Activation by Metformin in Focal Segmental Glomerulosclerosis. Kidney International Reports 2024, 9: 1354-1368. PMID: 38707807, PMCID: PMC11068976, DOI: 10.1016/j.ekir.2024.02.006.Peer-Reviewed Original ResearchMinimal change diseaseRandomized Controlled TrialsSafety of metforminDouble-blindPodocyte injuryAdjunctive therapyPlacebo-controlled randomized controlled trialsPhase III studyPhase II trialPrimary glomerular diseaseFocal segmental glomerulosclerosisEffect of metforminPhase IIPlacebo-ControlledPreclinical dataNovel urineChange diseaseTissue markersRandomized trialsSegmental glomerulosclerosisGlomerular diseaseMechanistic biomarkersObservational studyFSGSInexpensive agentUnderstanding the podocyte immune responses in proteinuric kidney diseases: from pathogenesis to therapy
Jiang H, Shen Z, Zhuang J, Lu C, Qu Y, Xu C, Yang S, Tian X. Understanding the podocyte immune responses in proteinuric kidney diseases: from pathogenesis to therapy. Frontiers In Immunology 2024, 14: 1335936. PMID: 38288116, PMCID: PMC10822972, DOI: 10.3389/fimmu.2023.1335936.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsChronic kidney diseaseGlomerular filtration barrierProteinuric glomerular diseasesKidney diseaseGlomerular diseaseImmune responseFiltration barrierTherapeutic targetCell-like characteristicsFocal segmental glomerulosclerosisProteinuric kidney diseaseTargets of immune responsesDamage to podocytesLupus nephritisPotential therapeutic targetGlomerular basement membraneImmune injuryGlomerular injuryMembranous nephropathyFenestrated endothelial cellsKidney functionSegmental glomerulosclerosisAdaptive immunityEpithelial cellsPathogenic mechanisms
2021
Case Report: The Monogenic Familial Steroid-Resistant Nephrotic Syndrome Caused by a Novel Missense Mutation of NPHS2 Gene A593C in a Chinese Family
Bai L, Zhuang J, Zhang C, Lu C, Tian X, Jiang H. Case Report: The Monogenic Familial Steroid-Resistant Nephrotic Syndrome Caused by a Novel Missense Mutation of NPHS2 Gene A593C in a Chinese Family. Frontiers In Pediatrics 2021, 9: 692727. PMID: 34631609, PMCID: PMC8497038, DOI: 10.3389/fped.2021.692727.Peer-Reviewed Case Reports and Technical NotesSteroid-resistant nephrotic syndromeEnd-stage kidney diseaseFocal segmental glomerulosclerosisNephrotic syndromeHeterozygous asymptomatic carriersRenal pathological lesionsGene variantsTrio whole-exome sequencingPrediction of prognosisAutosomal recessive steroid-resistant nephrotic syndromeWhole-exome sequencingMissense mutationsRituximab treatmentProgressive proteinuriaGlomerular filtration barrierMale patientsCalcineurin inhibitorsKidney diseaseAsymptomatic carriersSegmental glomerulosclerosisHemodialysis treatmentTreatment strategiesFamilial steroid-resistant nephrotic syndromePathological lesionsConsanguineous Chinese family
2020
Rituximab in adult minimal change disease and focal segmental glomerulosclerosis - What is known and what is still unknown?
Gauckler P, Shin J, Alberici F, Audard V, Bruchfeld A, Busch M, Cheung C, Crnogorac M, Delbarba E, Eller K, Faguer S, Galesic K, Griffin S, Hrušková Z, Jeyabalan A, Karras A, King C, Kohli H, Maas R, Mayer G, Moiseev S, Muto M, Odler B, Pepper R, Quintana L, Radhakrishnan J, Ramachandran R, Salama A, Segelmark M, Tesař V, Wetzels J, Willcocks L, Windpessl M, Zand L, Zonozi R, Kronbichler A, group F. Rituximab in adult minimal change disease and focal segmental glomerulosclerosis - What is known and what is still unknown? Autoimmunity Reviews 2020, 19: 102671. PMID: 32942039, DOI: 10.1016/j.autrev.2020.102671.Peer-Reviewed Original ResearchConceptsFocal segmental glomerulosclerosisSegmental glomerulosclerosisB-cell-depleting monoclonal antibodyAdult minimal change diseaseSteroid-sparing therapyToxic immunosuppressive agentsLow level of evidenceLong-term efficacyMinimal change diseaseLevel of evidenceRituximab-treatmentRandomized Controlled TrialsImmunosuppressive treatmentSteroid-dependentImmunosuppressive agentsOptimal regimenMulticenter studyNephrotic syndromeAdult patientsChange diseaseDisease courseOff-labelSide effectsControlled TrialsMonoclonal antibodiesGenetic Study in Korean Pediatric Patients with Steroid-Resistant Nephrotic Syndrome or Focal Segmental Glomerulosclerosis
Park E, Lee C, Kim N, Ahn Y, Park Y, Lee J, Kim S, Cho M, Cho H, Yoo K, Shin J, Kang H, Ha I, Park W, Cheong H. Genetic Study in Korean Pediatric Patients with Steroid-Resistant Nephrotic Syndrome or Focal Segmental Glomerulosclerosis. Journal Of Clinical Medicine 2020, 9: 2013. PMID: 32604935, PMCID: PMC7355646, DOI: 10.3390/jcm9062013.Peer-Reviewed Original ResearchSteroid-resistant nephrotic syndromeMutation detection rateFocal segmental glomerulosclerosisKorean pediatric patientsEnd-stage renal diseasePediatric patientsNephrotic syndromeSegmental glomerulosclerosisGenotype-phenotype correlation studiesAssociated with focal segmental glomerulosclerosisPrediction of prognosisGenotype-phenotype correlationDetection rateSteroid treatmentCongenital onsetStudy cohortRenal diseaseGenetic studiesGenetic diagnosisCausative genesMonogenic causePatientsTreatment policyMutationsCohort
2019
Immunoadsorption in nephrotic syndrome: Where are we now and where are we going from here?
Kronbichler A, Gauckler P, Lee K, Shin J, Malvezzi P, Mayer G. Immunoadsorption in nephrotic syndrome: Where are we now and where are we going from here? Atherosclerosis Plus 2019, 40: 55-60. PMID: 31447217, DOI: 10.1016/j.atherosclerosissup.2019.08.027.Peer-Reviewed Original ResearchConceptsIdiopathic nephrotic syndromeFocal segmental glomerulosclerosisPlasma exchangeMembranous nephropathyNephrotic syndromeKidney transplantationManagement of idiopathic nephrotic syndromeThrombospondin type-1 domain-containing 7AClinical trialsPrimary focal segmental glomerulosclerosisPhase II clinical trialPhospholipase A2 receptorTreatment-resistant casesAbsence of genetic testingII clinical trialsTreatment formsPrescribed immunosuppressive drugsRemission ratePLA2R-AbPrimary MNImmunosuppressive drugsLDL apheresisA2 receptorsSafety profileTreatment optionsIncreased Serum Soluble Urokinase‐Type Plasminogen Activator Receptor (suPAR) Levels in FSGS: A Meta‐Analysis
Lee J, Yang J, Kronbichler A, Eisenhut M, Kim G, Lee K, Shin J. Increased Serum Soluble Urokinase‐Type Plasminogen Activator Receptor (suPAR) Levels in FSGS: A Meta‐Analysis. Journal Of Immunology Research 2019, 2019: 5679518. PMID: 31089477, PMCID: PMC6476117, DOI: 10.1155/2019/5679518.Peer-Reviewed Original ResearchConceptsSoluble urokinase-type plasminogen activator receptorSoluble urokinase-type plasminogen activator receptor levelsPrimary focal segmental glomerulosclerosisUrokinase-type plasminogen activator receptorSerum suPAR levelsPlasminogen activator receptorSuPAR levelsStandardized mean differenceAdult patientsSerum soluble urokinase-type plasminogen activator receptorMeta-analysisHealthy controlsActivator receptorComparison to healthy controlsNewcastle-Ottawa Quality Assessment ScaleHigher suPAR levelsMinimal change diseaseFocal segmental glomerulosclerosisPeriod of diagnosisQuality Assessment ScaleRandom-effects modelRisk of biasNo significant differencePediatric groupChange diseaseMedication-Associated Glomerular Disease
Tucker B, Luciano R. Medication-Associated Glomerular Disease. 2019, 735-772. DOI: 10.1007/978-3-319-49379-4_46.Peer-Reviewed Original ResearchGlomerular diseaseTubule-interstitial diseaseMinimal change diseaseFocal segmental glomerulosclerosisKidney injuryThrombotic microangiopathyAcute hemodynamicsCrystalline nephropathyChange diseaseHistologic patternSegmental glomerulosclerosisAdditional interventionsMedicationsDisease severitySpecific drugsDiseaseInjuryDrugsVasculitisHematuriaMicroangiopathyProteinuriaDiscontinuationGlomerulonephritisNephropathyLoss of Subpodocytic Space Predicts Poor Response to Tacrolimus in Steroid-Resistant Calcineurin Inhibitor-Naïve Adult-Onset Primary Focal Segmental Glomerulosclerosis
Agrawal P, Nada R, Ramachandran R, Rayat C, Kumar A, Kohli H. Loss of Subpodocytic Space Predicts Poor Response to Tacrolimus in Steroid-Resistant Calcineurin Inhibitor-Naïve Adult-Onset Primary Focal Segmental Glomerulosclerosis. Indian Journal Of Nephrology 2019, 29: 90-94. PMID: 30983748, PMCID: PMC6440328, DOI: 10.4103/ijn.ijn_422_17.Peer-Reviewed Original ResearchFocal segmental glomerulosclerosisSegmental glomerulosclerosisCause of adult-onset nephrotic syndromePrediction of response to treatmentPrimary focal segmental glomerulosclerosisAdult-onset nephrotic syndromeResponse to tacrolimusTreated with tacrolimusResponse to treatmentDuration of illnessTacrolimus responsePartial responseDiagnostic biopsySubpodocyte spaceRetrospective studyNephrotic syndromeSurrogate markerTacrolimusResponse casesPodocyte phenotypePatientsOlder ageGlomerulosclerosisCompare presenceMigratory phenotype
2018
Drug-Induced Acute Kidney Injury
Luciano R, Perazella M. Drug-Induced Acute Kidney Injury. 2018, 145-163. DOI: 10.1007/978-1-4939-8628-6_9.Peer-Reviewed Original ResearchDrug-induced acute kidney injuryAcute kidney injuryKidney injuryAcute tubular injury/necrosisAcute interstitial nephritisFocal segmental glomerulosclerosisDrugs/substancesOsmotic nephropathyRisk patientsInterstitial nephritisKidney functionPrescribed medicationsSegmental glomerulosclerosisEarly identificationRenal compartmentsInjuryHarmful drugsTherapeutic agentsAdverse effectsCommon formDrugsMedicationsKidneyCommon problemDiagnostic agentsCancer drugs and the glomerulus
Shah H, Uppal N, Perazella M. Cancer drugs and the glomerulus. Journal Of Onco-Nephrology 2018, 2: 78-91. DOI: 10.1177/2399369318815418.Peer-Reviewed Original ResearchGlomerular lesionsCancer drugsAnti-vascular endothelial growth factor inhibitorsPoor renal outcomeAcute interstitial nephritisAcute kidney injuryAcute tubular necrosisGrowth factor inhibitorsMinimal change diseaseFocal segmental glomerulosclerosisTyrosine kinase inhibitorsNovel cancer drugsRenal outcomesKidney injuryThrombotic microangiopathyNovel immunotherapiesTubular injuryTubular necrosisInterstitial nephritisGlomerular toxicityChange diseaseFactor inhibitorsSegmental glomerulosclerosisGlomerular diseasePodocyte injuryRecent advances of animal model of focal segmental glomerulosclerosis
Yang J, Dettmar A, Kronbichler A, Gee H, Saleem M, Kim S, Shin J. Recent advances of animal model of focal segmental glomerulosclerosis. Clinical And Experimental Nephrology 2018, 22: 752-763. PMID: 29556761, DOI: 10.1007/s10157-018-1552-8.Peer-Reviewed Original ResearchConceptsAnimal models of focal segmental glomerulosclerosisFocal segmental glomerulosclerosisGroups of animal modelsModel of focal segmental glomerulosclerosisAnimal modelsSegmental glomerulosclerosisCD2-associated proteinCombination of genetic mutationsPathophysiology of focal segmental glomerulosclerosisGenetic engineering techniquesStudy focal segmental glomerulosclerosisGene disruptionGenetic basisGenetic animal modelsHuman diseasesTRPC6 channelsRenal ablationPodocyte toxinGenetic mutationsComplex pathophysiologyMouse backgroundSlit diaphragmDiphtheria toxinPodocyte injuryAdaptive responseKidney disease in the setting of HIV infection: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference
Swanepoel C, Atta M, D’Agati V, Estrella M, Fogo A, Naicker S, Post F, Wearne N, Winkler C, Cheung M, Wheeler D, Winkelmayer W, Wyatt C, Participants C, Abu-Alfa A, Adu D, Agodoa L, Alpers C, Arogundade F, Ashuntantang G, Bagnis C, Bhimma R, Brocheriou I, Cohen A, Cohen K, Cook H, de Seigneux S, Fabian J, Finkelstein F, Haas M, Hamzah L, Hendry B, Imonje V, Jennette J, Kimmel P, Klotman M, Klotman P, Larsen C, McCulloch M, Mosiane P, Nast C, Okpechi I, Ray P, Rosenberg A, Ross M, Ryom L, Truong L, Ulasi I, Vogt L, Zeier M. Kidney disease in the setting of HIV infection: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney International 2018, 93: 545-559. PMID: 29398134, PMCID: PMC5983378, DOI: 10.1016/j.kint.2017.11.007.Peer-Reviewed Original ResearchConceptsHIV-positive individualsKidney diseaseAntiretroviral therapyHIV infectionImmune complex kidney diseaseKidney disease preventionHIV-associated nephropathyFocal segmental glomerulosclerosisUnique HIVKidney injuryOpportunistic infectionsHIV treatmentClinical guidelinesRenal pathologySegmental glomerulosclerosisGeneral populationRole of geneticsGlobal outcomeDisease preventionNatural historyInfectious diseasesDiseaseInfectionGenetic factorsPrevention
2017
A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy
Her S, Lee K, Shin J. A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy. Childhood Kidney Diseases 2017, 21: 165-168. DOI: 10.3339/jkspn.2017.21.2.165.Peer-Reviewed Original ResearchMedication-Associated Glomerular Disease
Tucker B, Luciano R. Medication-Associated Glomerular Disease. 2017, 1-36. DOI: 10.1007/978-3-319-27334-1_46-1.Peer-Reviewed Original ResearchGlomerular diseaseTubule-interstitial diseaseMinimal change diseaseFocal segmental glomerulosclerosisKidney injuryThrombotic microangiopathyAcute hemodynamicsCrystalline nephropathyChange diseaseHistologic patternSegmental glomerulosclerosisAdditional interventionsMedicationsDisease severitySpecific drugsDiseaseInjuryDrugsVasculitisHematuriaMicroangiopathyProteinuriaDiscontinuationGlomerulonephritisNephropathy
2016
Collapsing glomerulopathy in a young woman with APOL1 risk alleles following acute parvovirus B19 infection: a case report investigation
Besse W, Mansour S, Jatwani K, Nast CC, Brewster UC. Collapsing glomerulopathy in a young woman with APOL1 risk alleles following acute parvovirus B19 infection: a case report investigation. BMC Nephrology 2016, 17: 125. PMID: 27600725, PMCID: PMC5013576, DOI: 10.1186/s12882-016-0330-7.Peer-Reviewed Case Reports and Technical NotesConceptsParvovirus B19 infectionAcute parvovirus B19 infectionFocal segmental glomerulosclerosisHuman immunodeficiency virusAPOL1 risk allelesB19 infectionRisk allelesCollapsing variant of focal segmental glomerulosclerosisVariant of focal segmental glomerulosclerosisPublished case reportsHuman immunodeficiency virus (HIV) infectionCaribbean-American womenInfection of podocytesAcute parvovirus infectionImmunodeficiency virus infectionRapid clinical courseFetal demiseForms of FSGSLight microscopy findingsClinical courseHIV-negativeImmunodeficiency virusRenal biopsyKidney biopsyParvovirus B19Soluble Urokinase Receptors in Focal Segmental Glomerulosclerosis: A Review on the Scientific Point of View
Kronbichler A, Saleem M, Meijers B, Shin J. Soluble Urokinase Receptors in Focal Segmental Glomerulosclerosis: A Review on the Scientific Point of View. Journal Of Immunology Research 2016, 2016: 2068691. PMID: 27504461, PMCID: PMC4967695, DOI: 10.1155/2016/2068691.Peer-Reviewed Original ResearchConceptsSoluble urokinase-type plasminogen activator receptorFocal segmental glomerulosclerosisPathogenesis of focal segmental glomerulosclerosisSoluble urokinase-type plasminogen activator receptor levelsSegmental glomerulosclerosisProgression to end-stage renal failureEnd-stage renal failureUrokinase-type plasminogen activator receptorSerum suPAR levelsSoluble urokinase receptorIn Vivo Mouse ModelPlasminogen activator receptorImmunologically active cellsPrimary glomerular disordersSuPAR levelsRenal transplantationRenal failureSecondary causesMouse modelCirculating factorsGlomerular diseaseGlomerular disordersActivator receptorUrokinase receptorHuman studiesA prospective study of collapsing focal segmental glomerulosclerosis
Raja R, Nada R, Yadav A, Kumar A, Goyal A, Kumar V, Rathi M, Kohli H, Gupta K, Sakhuja V, Jha V. A prospective study of collapsing focal segmental glomerulosclerosis. Renal Failure 2016, 38: 894-898. PMID: 27266801, DOI: 10.3109/0886022x.2016.1164063.Peer-Reviewed Original ResearchConceptsProgression to end-stage renal diseaseCollapsing focal segmental glomerulosclerosisEnd-stage renal diseaseFocal segmental glomerulosclerosisSegmental glomerulosclerosisRate of progression to end-stage renal diseaseNoncollapsing focal segmental glomerulosclerosisResistant focal segmental glomerulosclerosisLong-term prognosisResponse to treatmentComplete remissionPartial remissionSteroid resistanceGlomerular collapseTubulointerstitial changesSerum creatinineProspective studyClinicopathological spectrumHistological featuresRenal diseaseWT-1Podocyte markersCase of steroidsPatientsSteroidsImmunologic Changes Implicated in the Pathogenesis of Focal Segmental Glomerulosclerosis
Kronbichler A, Leierer J, Oh J, Meijers B, Shin J. Immunologic Changes Implicated in the Pathogenesis of Focal Segmental Glomerulosclerosis. BioMed Research International 2016, 2016: 2150451. PMID: 26989679, PMCID: PMC4773535, DOI: 10.1155/2016/2150451.Peer-Reviewed Original ResearchConceptsFocal segmental glomerulosclerosisImmunological changesTGF-ss1TNF-aSegmental glomerulosclerosisTransforming growth factor-ss1End-stage renal diseaseClinical practiceTumor necrosis factor-aPathogenesis of focal segmental glomerulosclerosisDevelopment of proteinuriaB7-1Primary FSGSProfibrotic milieuTumor necrosisImmunosuppressive measuresResistant patientsRenal biopsyT cellsImmunological factorsHistological patternInterleukin-1ssRenal diseaseDisease progressionDisease onsetRenal involvement in patients with mucolipidosis IIIalpha/beta: Causal relation or co‐occurrence?
Tüysüz B, Ercan-Sencicek AG, Canpolat N, Koparır A, Yılmaz S, Kılıçaslan I, Gülez B, Bilguvar K, Günel M. Renal involvement in patients with mucolipidosis IIIalpha/beta: Causal relation or co‐occurrence? American Journal Of Medical Genetics Part A 2016, 170: 1187-1195. PMID: 26749367, DOI: 10.1002/ajmg.a.37543.Peer-Reviewed Original ResearchConceptsRenal involvementFlexion contractureNormal renal functionCause of proteinuriaNephrotic range proteinuriaFocal segmental glomerulosclerosisRare lysosomal storage disorderHereditary kidney diseaseGlomerular visceral epithelial cellsNovel homozygous missense mutationVisceral epithelial cellsWhole-exome sequencingLysosomal storage disorderRenal functionBiopsy findingsRenal biopsyKidney diseaseSegmental glomerulosclerosisFamily historyChildhood onsetGNPTAB geneHealthy siblingsHomozygous missense mutationLarge jointsMild short stature
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