âThe evolution of the classification of nephrotic syndrome and the ...

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CG: etiology <strong>and</strong> clinical associations• Idiopathic• Genetic• Syndromic - action myoclonus renal failure• Non-Syndormic - CoQ2 NP• Reactive• Virus associated- HIV- parvovirus B19- CMV• Infections - filariasis- leishmania- TB• Autoimmune - Still’s disease- lupus like- RA- mixed connective tissue• Malignancy (myeloma, AML)• Medications - pamidronate- interferon- valproic acid• Vascular insult - TMA• Permeability factor
TAXONOMY OF PODOCYTOPATHIESidiopathic genetic reactiveMCNIdiopathic•Steroid-sensitive•Steroid-resistantNon-syndromic•NPHS1•NPHS2Syndromic•DYSFClinical association(immunologic stimuli, Tumors)Medications(NSAID,gold, penicillamine, lithium,IF, pamidronate)FSGSIdiopathic•Steroid-sensitive•Steroid-resistantNon-syndromicITGB4, NPSH2, NPHS3, NPHS1 +NPHS2, COQ2, MHY9, ACTN4,CD2AP, TRCP6, WT-1, SYNPO, INF2SyndromicMtDNA, WT1, PAX2, COQ6,COL4,GLA, LMBX1Post-adaptive•nephron mass•Initially normal nephron massMedications(tacrolimus, lithium, IF, pamidronate)DMS Idiopathic Non-syndromicWT1, NPHS1, NPSH2, NPHS3,LAMB2,SyndromicWT1, LAMB2, COQ6,CG Idiopathic Non-syndromicCOQ2MHY9SyndromicSCARBInfections(viruses, TB, o<strong>the</strong>rs)Clinical association•Autoimmune, TMA, tumorsMedications(IF, pamidronate, valproic acid)
Page 1 and 2: “The evolution of the classificat
Page 3 and 4: Nephrotic syndrome - the 80’s and
Page 5 and 6: Limitations of the morphologicclass
Page 7 and 8: PodocytopathiesDEFINITION: Proteinu
Page 9: Podocytopathies:4 morphologic patte
Page 13 and 14: Hypothesis #1:Injured podocytes can
Page 15 and 16: Hypothesis #2The role of CD24+CD133
Page 17 and 18: Minimal Change NephropathyDEFINITIO
Page 19 and 20: Can pathologists discriminatebetwee
Page 21 and 22: DG staining in steroid sensitive an
Page 23 and 24: FOCAL SEGMENTALGLOMERULOSCLEROSIS
Page 25 and 26: Idiopathic FSGSIs idiopathic really
Page 27 and 28: Reactive forms:Hyperfiltration-medi
Page 29 and 30: FSGS: From podocyte hypertrophy to
Page 31 and 32: DMSDEFINITION:Diffuse increase of m
Page 33 and 34: NPHS3-PLCE1Chromosome 10q23.32-q24.
Page 35: CG Definition: GBM collapse andpseu

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