2023
Understanding Nephrotic Syndrome Using Kidney Transcriptome Profiling and Computational Studies
Rangel P, Tian X. Understanding Nephrotic Syndrome Using Kidney Transcriptome Profiling and Computational Studies. Kidney360 2023, 4: e431-e433. PMID: 37103957, PMCID: PMC10371383, DOI: 10.34067/kid.0000000000000117.Commentaries, Editorials and Letters
2022
The scope of treatment of pediatric IgA vasculitis nephritis and its outcome: a Pediatric Nephrology Research Consortium study
Kallash M, Vogt BA, Zeid A, Khin E, Najjar M, Aldughiem A, Benoit E, Stotter B, Rheault M, Warejko JK, Daga A. The scope of treatment of pediatric IgA vasculitis nephritis and its outcome: a Pediatric Nephrology Research Consortium study. Pediatric Nephrology 2022, 37: 2687-2697. PMID: 35233641, DOI: 10.1007/s00467-022-05496-3.Peer-Reviewed Original ResearchConceptsKidney biopsyGraphical abstractA higher resolution versionPediatric nephrology research consortium studyUrine protein/creatinine ratioProtein/creatinine ratioAnti-metabolite agentsIgA vasculitis nephritisPercent of patientsMulticenter retrospective studyChildren ages 1Scope of treatmentIgAV nephritisIS therapyKidney outcomesVasculitis nephritisNormal eGFRMicroscopic hematuriaOlder patientsCreatinine ratioMedian ageHeavy proteinuriaImmunosuppressive agentsKidney functionProspective studyRetrospective studyRates of idiopathic childhood nephrotic syndrome relapse are lower during the COVID-19 pandemic
Crane C, Bakhoum C, Ingulli E. Rates of idiopathic childhood nephrotic syndrome relapse are lower during the COVID-19 pandemic. Pediatric Nephrology 2022, 37: 2679-2685. PMID: 35211788, PMCID: PMC8869345, DOI: 10.1007/s00467-022-05483-8.Peer-Reviewed Original ResearchMeSH KeywordsChildChronic DiseaseCOVID-19HumansNephrosis, LipoidNephrotic SyndromePandemicsRecurrenceRetrospective StudiesSteroidsConceptsSteroid-sensitive nephrotic syndromeRate of relapseNephrotic syndromeTransmission of infectionNS relapseGraphical abstractA higher resolution versionSingle-center retrospective chart reviewNephrotic syndrome relapseSteroid-sensitive NSRetrospective chart reviewLower relapse rateRate of hospitalizationInfection prevention measuresResultsOne hundred twentyCOVID-19 pandemicPre-pandemic periodChart reviewRelapse rateInfection preventionHundred twentyRelapseStudy periodSocial distancing periodConclusionsOur resultsDecreased rate
2021
An initiative to improve pneumococcal immunization counseling in children with nephrotic syndrome
Sandokji I, Anderson LS, Warejko JK, Emerson BL, Greenberg JH. An initiative to improve pneumococcal immunization counseling in children with nephrotic syndrome. Pediatric Nephrology 2021, 37: 1333-1338. PMID: 34734331, PMCID: PMC8565641, DOI: 10.1007/s00467-021-05305-3.Peer-Reviewed Original ResearchMeSH KeywordsChildCounselingHumansImmunizationNephrotic SyndromePneumococcal InfectionsPneumococcal VaccinesVaccinationConceptsNephrotic syndromeElectronic health recordsEHR remindersCounseling ratesProvider awarenessGraphical abstractA higher resolution versionLife-threatening pneumococcal infectionsCurrent practice guidelinesPrimary care providersQuality improvement projectVaccination counselingVaccine guidelinesProvider adherenceVaccine seriesNephrology clinicPneumococcal infectionAdditional vaccinesPractice guidelinesImmunization protocolCare providersImmunization counselingSyndromeFirst interventionHealth recordsAdherenceAMP-Kinase mediates regulation of glomerular volume and podocyte survival
Banu K, Lin Q, Basgen JM, Planoutene M, Wei C, Reghuvaran AC, Tian X, Shi H, Garzon F, Garzia A, Chun N, Cumpelik A, Santeusanio AD, Zhang W, Das B, Salem F, LI L, Ishibe S, Cantley LG, Kaufman L, Lemley KV, Ni Z, He JC, Murphy B, Menon MC. AMP-Kinase mediates regulation of glomerular volume and podocyte survival. JCI Insight 2021, 6: e150004. PMID: 34473647, PMCID: PMC8525649, DOI: 10.1172/jci.insight.150004.Peer-Reviewed Original ResearchAdenylate KinaseAdolescentAdultAgedAlbuminuriaAnimalsCell SizeCell SurvivalChildChild, PreschoolFemaleGene Knockdown TechniquesGlomerulonephritis, MembranousGlomerulosclerosis, Focal SegmentalHumansHypertrophyInfantKidney GlomerulusMaleMiceMicrofilament ProteinsMiddle AgedNephrectomyNephrosis, LipoidNephrotic SyndromePodocytesProportional Hazards ModelsProto-Oncogene Proteins c-fynYoung AdultSkimmed breast milk for treatment of hypertriglyceridemia in an infant with congenital nephrotic syndrome
Dahl A, Armellino A, Tran C, Tebben P. Skimmed breast milk for treatment of hypertriglyceridemia in an infant with congenital nephrotic syndrome. Nutrition In Clinical Practice 2021, 37: 383-387. PMID: 34486165, DOI: 10.1002/ncp.10759.Peer-Reviewed Case Reports and Technical NotesMeSH KeywordsChildFemaleHumansHyperlipidemiasHypertriglyceridemiaInfantInfant, NewbornMaleMilk, HumanNephrotic SyndromeTriglyceridesConceptsCongenital nephrotic syndromeSkimmed breast milkMaternal breast milkBreast milkTriglyceride concentrationsSevere hypertriglyceridemiaNephrotic syndromeTreatment of severe hypertriglyceridemiaLower extremity edemaNephrotic-range proteinuriaLow-fat formulaDeep venous thrombosisTreatment of hypertriglyceridemiaTreatment of dyslipidemiaBreast milk supplyElevation of serum cholesterolExtremity edemaNeonatal periodMedium-chain triglyceride oilNPHS1 geneTherapeutic optionsVenous thrombosisPathogenic variantsWeeks of ageLaboratory evaluationPediatric Nephrotic Syndrome: Pharmacologic and Nutrition Management
Hampson K, Gay M, Band M. Pediatric Nephrotic Syndrome: Pharmacologic and Nutrition Management. Nutrition In Clinical Practice 2021, 36: 331-343. PMID: 33469930, DOI: 10.1002/ncp.10622.Peer-Reviewed Original ResearchConceptsNephrotic syndromeSteroid-refractory diseaseLong-term complicationsSteroid-sparing therapiesMetabolic bone diseaseCommon kidney diseaseSodium restrictionFrequent relapsesMost patientsCalcineurin inhibitorsDietary modificationKidney diseaseGlomerular filtrationNutrition interventionsBone diseaseAppropriate managementNutrition cliniciansNutrition managementDiseaseMicronutrient deficienciesEdemaPatientsSyndromeSerious casesTherapy
2020
Primary membranous nephropathy in children and adolescents: a single-centre report from South Asia
Ramachandran R, Nayak S, Kumar V, Kumar A, Agrawal N, Bansal R, Tiewsoh K, Nada R, Rathi M, Kohli H. Primary membranous nephropathy in children and adolescents: a single-centre report from South Asia. Pediatric Nephrology 2020, 36: 1217-1226. PMID: 33108509, DOI: 10.1007/s00467-020-04798-8.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAsiaChildGlomerulonephritis, MembranousHumansNephrotic SyndromeProspective StudiesReceptors, Phospholipase A2RituximabConceptsAssociated with clinical outcomesPartial remissionFollow-upImmunosuppressive therapyClinical outcomesMembranous nephropathyPatients treated with rituximabSymptom onset to presentationBiopsy-proven pMNSingle-centre reportsMedian Follow-UpFirst-line therapyPrimary membranous nephropathyMethodsThis prospective studyPercentage of patientsChildhood nephrotic syndromeMedian proteinuriaAnti-PLA2RMycophenolate mofetilMedian intervalMedian ageResistant diseaseClinical presentationNo significant differenceClinical behaviorRituximab 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 antibodiesEstablishment of a novel nomogram for the clinically diagnostic prediction of minimal change disease, −a common cause of nephrotic syndrome
Yan G, Liu G, Tian X, Tian L, Wang H, Ren P, Ma X, Fu R, Chen Z. Establishment of a novel nomogram for the clinically diagnostic prediction of minimal change disease, −a common cause of nephrotic syndrome. BMC Nephrology 2020, 21: 396. PMID: 32928127, PMCID: PMC7490860, DOI: 10.1186/s12882-020-02058-3.Peer-Reviewed Original ResearchMeSH KeywordsAdultArea Under CurveBlood PressureComplement C1qComplement C3Complement C4DiastoleFemaleGlomerular Filtration RateHemoglobinsHumansImmunoglobulin EImmunoglobulin GImmunoglobulin MMaleMiddle AgedNephrosis, LipoidNephrotic SyndromeNomogramsRegression AnalysisReproducibility of ResultsSensitivity and SpecificityYoung AdultConceptsDiastolic blood pressurePrimary glomerular diseaseNephrotic syndromeAdult patientsRenal biopsyChange diseaseGlomerular diseaseBackgroundMinimal change diseaseMinimal change diseaseLASSO regression analysisRenal biopsy procedureNon-MCD groupLogistic regression modelsDiagnostic prediction modelNovel nomogramPatient demographicsBlood pressureSerum levelsOverall incidenceClinical manifestationsMCD patientsMCD diagnosisCommon causeInvasive proceduresMethodA totalCurrent understandings in treating children with steroid-resistant nephrotic syndrome
Lee J, Kronbichler A, Shin J, Oh J. Current understandings in treating children with steroid-resistant nephrotic syndrome. Pediatric Nephrology 2020, 36: 747-761. PMID: 32086590, PMCID: PMC7910243, DOI: 10.1007/s00467-020-04476-9.Peer-Reviewed Original ResearchConceptsSteroid-resistant nephrotic syndromeNephrotic syndromeCalcineurin inhibitorsTreating childrenIdiopathic steroid-resistant nephrotic syndromeSteroid-sensitive nephrotic syndromeAngiotensin II receptor blockersNon-immune therapiesSteroid-sparing agentsAngiotensin-converting enzyme inhibitorsHeterogeneous disease entityII receptor blockersInduce remissionImmune-basedReceptor blockersAdjuvant agentTreatment optionsMonogenic aetiologyTreatment strategiesDisease entityPaediatric nephrologistsEnzyme inhibitorsMonoclonal antibodiesSyndromePatientsA Patient with Nephrotic Syndrome and Acute Flank Pain.
Perazella MA. A Patient with Nephrotic Syndrome and Acute Flank Pain. Kidney360 2020, 1: 74-75. PMID: 35372858, PMCID: PMC8808488, DOI: 10.34067/kid.0000482019.Peer-Reviewed Original Research
2019
Review on long‐term non‐renal complications of childhood nephrotic syndrome
Lee J, Kronbichler A, Shin J, Oh J. Review on long‐term non‐renal complications of childhood nephrotic syndrome. Acta Paediatrica 2019, 109: 460-470. PMID: 31561270, DOI: 10.1111/apa.15035.Peer-Reviewed Original ResearchConceptsNon-renal complicationsNephrotic syndromeChildhood NSLong-term renal prognosisNon-renal outcomesComplicated disease courseFollow-up periodBone mineral diseaseChildhood nephrotic syndromeImpaired longitudinal growthNonrenal outcomesOcular complicationsSteroid-dependentIdiopathic NSImmunosuppressive agentsPotential malignancyFrequent relapsesReduce proteinuriaDisease coursePubertal developmentProlonged treatmentMineral diseaseFertility outcomesHigh riskComplicationsA Rare and Extreme Case of Vulvar Swelling in the Setting of Nephrotic Syndrome in a Prepubertal Girl
Solotke MT, Soble W, Young C, Marsenic O, Vash-Margita A. A Rare and Extreme Case of Vulvar Swelling in the Setting of Nephrotic Syndrome in a Prepubertal Girl. Journal Of Pediatric And Adolescent Gynecology 2019, 33: 93-95. PMID: 31585165, DOI: 10.1016/j.jpag.2019.09.009.Peer-Reviewed Original ResearchImmunoadsorption 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 optionsIdentification of novel mutations and phenotype in the steroid resistant nephrotic syndrome gene NUP93: a case report
Sandokji I, Marquez J, Ji W, Zerillo CA, Konstantino M, Lakhani SA, Khokha MK, Warejko JK. Identification of novel mutations and phenotype in the steroid resistant nephrotic syndrome gene NUP93: a case report. BMC Nephrology 2019, 20: 271. PMID: 31315584, PMCID: PMC6637548, DOI: 10.1186/s12882-019-1458-z.Peer-Reviewed Case Reports and Technical Notes
2018
Efficacy and safety of rituximab in childhood-onset, difficult-to-treat nephrotic syndrome
Ahn Y, Kim S, Han K, Choi H, Cho H, Lee J, Shin J, Cho M, Lee J, Park Y, Ha I, Cheong H, Kim S, Lee S, Kang H. Efficacy and safety of rituximab in childhood-onset, difficult-to-treat nephrotic syndrome. Medicine 2018, 97: e13157. PMID: 30431588, PMCID: PMC6257685, DOI: 10.1097/md.0000000000013157.Peer-Reviewed Original ResearchConceptsDifficult-to-treat nephrotic syndromeDifficult-to-treat NSSafety of rituximabRituximab groupSingle-arm studyNephrotic syndromeB cellsRituximab administrationCalcineurin inhibitorsAnti-CD20 monoclonal antibody rituximabControl groupDoses of intravenous rituximabDepletion of B cellsMulticenter open-label trialDose of rituximabTreated with rituximabB-cell depletionDeplete B cellsResistant to steroidsCD19(+) cellsMonoclonal antibody rituximabOpen-label trialPediatric nephrology centersRituximab doseRandomized Controlled TrialsMutations in multiple components of the nuclear pore complex cause nephrotic syndrome
Braun DA, Lovric S, Schapiro D, Schneider R, Marquez J, Asif M, Hussain MS, Daga A, Widmeier E, Rao J, Ashraf S, Tan W, Lusk CP, Kolb A, Jobst-Schwan T, Schmidt JM, Hoogstraten CA, Eddy K, Kitzler TM, Shril S, Moawia A, Schrage K, Khayyat AIA, Lawson JA, Gee HY, Warejko JK, Hermle T, Majmundar AJ, Hugo H, Budde B, Motameny S, Altmüller J, Noegel AA, Fathy HM, Gale DP, Waseem SS, Khan A, Kerecuk L, Hashmi S, Mohebbi N, Ettenger R, Serdaroğlu E, Alhasan KA, Hashem M, Goncalves S, Ariceta G, Ubetagoyena M, Antonin W, Baig SM, Alkuraya FS, Shen Q, Xu H, Antignac C, Lifton RP, Mane S, Nürnberg P, Khokha MK, Hildebrandt F. Mutations in multiple components of the nuclear pore complex cause nephrotic syndrome. Journal Of Clinical Investigation 2018, 128: 4313-4328. PMID: 30179222, PMCID: PMC6159964, DOI: 10.1172/jci98688.Peer-Reviewed Original ResearchConceptsNuclear pore complexSteroid-resistant nephrotic syndromeCRISPR/Cas9 knockoutOrgan-specific phenotypesNephrotic syndromeRing subunitsMorpholino knockdownEssential genesEnd-stage renal diseasePore complexNPC subunitsCoimmunoprecipitation experimentsAllelic effectsNUP85CRISPR/Nup107Hypomorphic mutationsNup133WT mRNAEarly lethalityGenesImportant effectorsSubunitsMutationsRenal diseaseGAPVD1 and ANKFY1 Mutations Implicate RAB5 Regulation in Nephrotic Syndrome
Hermle T, Schneider R, Schapiro D, Braun DA, van der Ven AT, Warejko JK, Daga A, Widmeier E, Nakayama M, Jobst-Schwan T, Majmundar AJ, Ashraf S, Rao J, Finn LS, Tasic V, Hernandez JD, Bagga A, Jalalah SM, El Desoky S, Kari JA, Laricchia KM, Lek M, Rehm HL, MacArthur DG, Mane S, Lifton RP, Shril S, Hildebrandt F. GAPVD1 and ANKFY1 Mutations Implicate RAB5 Regulation in Nephrotic Syndrome. Journal Of The American Society Of Nephrology 2018, 29: 2123-2138. PMID: 29959197, PMCID: PMC6065084, DOI: 10.1681/asn.2017121312.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCell MovementCells, CulturedCohort StudiesDisease ProgressionDrosophila melanogasterExome SequencingFemaleGene Expression RegulationGenetic Predisposition to DiseaseHumansMaleMass ScreeningMembrane ProteinsMutation, MissenseNephrotic SyndromePedigreePhosphate-Binding ProteinsPodocytesrab5 GTP-Binding ProteinsReal-Time Polymerase Chain ReactionRenal Insufficiency, ChronicRNA, Small InterferingConceptsSteroid-resistant nephrotic syndromeNovel monogenic causesCoimmunoprecipitation assaysHomozygous missense mutationPatient-derived mutationsMissense mutationsMonogenic causesHEK293T cellsActive Rab5GAPVD1Nephrotic syndromePodocyte migration rateEctopic expressionCases of SRNSPartial colocalizationSpecific pathogenetic pathwaysWhole-exome sequencingEarly-onset NSHuman NFunctional significancePodocyte migrationProteinMutationsPhysical interactionRab5Mutations in six nephrosis genes delineate a pathogenic pathway amenable to treatment
Ashraf S, Kudo H, Rao J, Kikuchi A, Widmeier E, Lawson JA, Tan W, Hermle T, Warejko JK, Shril S, Airik M, Jobst-Schwan T, Lovric S, Braun DA, Gee HY, Schapiro D, Majmundar AJ, Sadowski CE, Pabst WL, Daga A, van der Ven AT, Schmidt JM, Low BC, Gupta AB, Tripathi BK, Wong J, Campbell K, Metcalfe K, Schanze D, Niihori T, Kaito H, Nozu K, Tsukaguchi H, Tanaka R, Hamahira K, Kobayashi Y, Takizawa T, Funayama R, Nakayama K, Aoki Y, Kumagai N, Iijima K, Fehrenbach H, Kari JA, El Desoky S, Jalalah S, Bogdanovic R, Stajić N, Zappel H, Rakhmetova A, Wassmer SR, Jungraithmayr T, Strehlau J, Kumar AS, Bagga A, Soliman NA, Mane SM, Kaufman L, Lowy DR, Jairajpuri MA, Lifton RP, Pei Y, Zenker M, Kure S, Hildebrandt F. Mutations in six nephrosis genes delineate a pathogenic pathway amenable to treatment. Nature Communications 2018, 9: 1960. PMID: 29773874, PMCID: PMC5958119, DOI: 10.1038/s41467-018-04193-w.Peer-Reviewed Original ResearchMeSH KeywordsAdultAnimalsChildChild, PreschoolDisease Models, AnimalDNA Mutational AnalysisDrug ResistanceExome SequencingFemaleGene Knockdown TechniquesGlucocorticoidsHEK293 CellsHigh-Throughput Nucleotide SequencingHumansInfantMaleMiceMice, Inbred C57BLMice, KnockoutMiddle AgedMutationNephrotic SyndromePedigreePodocytesProtein Interaction MapsrhoA GTP-Binding ProteinRNA, Small InterferingTreatment OutcomeConceptsKnockdown of DLC1Small GTPase activityExchange factorNephrotic syndromeRhoA regulationGTPase activityDifferent genesDLC1GenesNS phenotypePotential therapeutic targetChronic kidney diseaseMutationsCultured podocytesKnockdownTherapeutic targetMigration rateSteroid treatmentKidney diseaseKnockout micePathogenic pathwaysFrequent causeITSN1Cdc42ITSN2
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