2024
#844 Renal manifestations of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: an updated systematic review
Lee K, Hwang E, Shin J, Park J. #844 Renal manifestations of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: an updated systematic review. Nephrology Dialysis Transplantation 2024, 39: gfae069-1209-844. DOI: 10.1093/ndt/gfae069.1209.Peer-Reviewed Original ResearchX-linked (IPEX) syndromeForkhead box protein 3Renal involvementIPEX syndromeIPEX patientsImmune dysregulationMembranous nephropathyCharacteristics of renal involvementManifestations of immune dysregulationDetecting renal involvementPatient outcomesCourse of glomerulonephritisGenotype-phenotype correlationResponse to treatmentMesangial proliferative glomerulonephritisRenal presentationRenal monitoringRenal manifestationsAutoimmune nephritisRenal biopsyNephrotic syndromeCase reportProliferative glomerulonephritisInterstitial nephritisPatients
2020
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: A systematic review
Park J, Lee K, Jeon B, Ochs H, Lee J, Gee H, Seo S, Geum D, Piccirillo C, Eisenhut M, van der Vliet H, Lee J, Kronbichler A, Ko Y, Shin J. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: A systematic review. Autoimmunity Reviews 2020, 19: 102526. PMID: 32234571, DOI: 10.1016/j.autrev.2020.102526.Peer-Reviewed Original ResearchConceptsForkhead box P3IPEX patientsRisk of deathSkin manifestationsX-linked (IPEX) syndromeFactors associated with increased risk of deathFOXP3 mutationsCD4<sup>+</sup> regulatory TAssociated with increased risk of deathAssociated with better survivalResponse to treatment strategiesAssociated with increased riskAutoimmune hemolytic anemiaImmune-related complicationsGenotype-phenotype correlationLoss-of-function mutationsImmunosuppressive therapyRegulatory TPrognostic factorsIPEX syndromeRenal involvementAutoimmune phenomenaBetter survivalImmune dysregulationSeptic shock
2015
Familial IPEX syndrome: Different glomerulopathy in two siblings
Park E, Chang H, Shin J, Lim B, Jeong H, Lee K, Moon K, Kang H, Ha I, Cheong H. Familial IPEX syndrome: Different glomerulopathy in two siblings. Pediatrics International 2015, 57: e59-e61. PMID: 25712815, DOI: 10.1111/ped.12570.Peer-Reviewed Original ResearchConceptsMembranous nephropathyX-linked (IPEX) syndromeSeveral autoimmune diseasesImmune regulatory systemFOXP3 mutationsIPEX syndromeRenal involvementImmune dysregulationClinical featuresNephrotic syndromeAutoimmune diseasesRenal lesionsHereditary disorderTubulointerstitial diseaseGlomerular lesionsSyndromeMCNSLesionsDiseaseFoxp3PolyendocrinopathyNephropathySiblingsGlomerulopathyIPEX
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