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Idiopathic multidrug-resistant nephrotic syndrome

MedGen UID:
1842472
•Concept ID:
C5681293
•
Disease or Syndrome
Synonym: idiopathic multidrug-resistant nephrotic syndrome
 
Monarch Initiative: MONDO:0035459
Orphanet: ORPHA567550

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic multidrug-resistant nephrotic syndrome

Recent clinical studies

Etiology

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Remission of proteinuria in multidrug-resistant idiopathic nephrotic syndrome following immunoglobulin immunoadsorption.
Nattes E, Karaa D, Dehoux L, Peuchmaur M, Kwon T, Deschênes G
Acta Paediatr 2019 Apr;108(4):757-762. Epub 2018 Oct 15 doi: 10.1111/apa.14582. PMID: 30230027
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.
Trautmann A, Schnaidt S, Lipska-Ziętkiewicz BS, Bodria M, Ozaltin F, Emma F, Anarat A, Melk A, Azocar M, Oh J, Saeed B, Gheisari A, Caliskan S, Gellermann J, Higuita LMS, Jankauskiene A, Drozdz D, Mir S, Balat A, Szczepanska M, Paripovic D, Zurowska A, Bogdanovic R, Yilmaz A, Ranchin B, Baskin E, Erdogan O, Remuzzi G, Firszt-Adamczyk A, Kuzma-Mroczkowska E, Litwin M, Murer L, Tkaczyk M, Jardim H, Wasilewska A, Printza N, Fidan K, Simkova E, Borzecka H, Staude H, Hees K, Schaefer F; PodoNet Consortium
J Am Soc Nephrol 2017 Oct;28(10):3055-3065. Epub 2017 May 31 doi: 10.1681/ASN.2016101121. PMID: 28566477Free PMC Article
Saquinavir in steroid-dependent and -resistant nephrotic syndrome: a pilot study.
Coppo R, Camilla R, Porcellini MG, Peruzzi L, Gianoglio B, Amore A, Daprà V, Loiacono E, Fonsato V, Dal Canton A, Esposito C, Esposito P, Tovo PA
Nephrol Dial Transplant 2012 May;27(5):1902-10. Epub 2012 Mar 19 doi: 10.1093/ndt/gfs035. PMID: 22431705
Relationship between expression of Pad1 homologue and multidrug resistance of idiopathic nephrotic syndrome.
Ma Z, Gao X, Zhao W, Li Y, Li C, Li C
Pediatr Int 2009 Oct;51(5):732-5. Epub 2009 Mar 20 doi: 10.1111/j.1442-200X.2009.02845.x. PMID: 19419512

Diagnosis

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.
Trautmann A, Schnaidt S, Lipska-Ziętkiewicz BS, Bodria M, Ozaltin F, Emma F, Anarat A, Melk A, Azocar M, Oh J, Saeed B, Gheisari A, Caliskan S, Gellermann J, Higuita LMS, Jankauskiene A, Drozdz D, Mir S, Balat A, Szczepanska M, Paripovic D, Zurowska A, Bogdanovic R, Yilmaz A, Ranchin B, Baskin E, Erdogan O, Remuzzi G, Firszt-Adamczyk A, Kuzma-Mroczkowska E, Litwin M, Murer L, Tkaczyk M, Jardim H, Wasilewska A, Printza N, Fidan K, Simkova E, Borzecka H, Staude H, Hees K, Schaefer F; PodoNet Consortium
J Am Soc Nephrol 2017 Oct;28(10):3055-3065. Epub 2017 May 31 doi: 10.1681/ASN.2016101121. PMID: 28566477Free PMC Article
Focal segmental glomerulosclerosis and medullary nephrocalcinosis in children with ADCK4 mutations.
Park E, Kang HG, Choi YH, Lee KB, Moon KC, Jeong HJ, Nagata M, Cheong HI
Pediatr Nephrol 2017 Sep;32(9):1547-1554. Epub 2017 Apr 12 doi: 10.1007/s00467-017-3657-9. PMID: 28405841
Relationship between expression of Pad1 homologue and multidrug resistance of idiopathic nephrotic syndrome.
Ma Z, Gao X, Zhao W, Li Y, Li C, Li C
Pediatr Int 2009 Oct;51(5):732-5. Epub 2009 Mar 20 doi: 10.1111/j.1442-200X.2009.02845.x. PMID: 19419512

Therapy

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.
Trautmann A, Schnaidt S, Lipska-Ziętkiewicz BS, Bodria M, Ozaltin F, Emma F, Anarat A, Melk A, Azocar M, Oh J, Saeed B, Gheisari A, Caliskan S, Gellermann J, Higuita LMS, Jankauskiene A, Drozdz D, Mir S, Balat A, Szczepanska M, Paripovic D, Zurowska A, Bogdanovic R, Yilmaz A, Ranchin B, Baskin E, Erdogan O, Remuzzi G, Firszt-Adamczyk A, Kuzma-Mroczkowska E, Litwin M, Murer L, Tkaczyk M, Jardim H, Wasilewska A, Printza N, Fidan K, Simkova E, Borzecka H, Staude H, Hees K, Schaefer F; PodoNet Consortium
J Am Soc Nephrol 2017 Oct;28(10):3055-3065. Epub 2017 May 31 doi: 10.1681/ASN.2016101121. PMID: 28566477Free PMC Article
Saquinavir in steroid-dependent and -resistant nephrotic syndrome: a pilot study.
Coppo R, Camilla R, Porcellini MG, Peruzzi L, Gianoglio B, Amore A, Daprà V, Loiacono E, Fonsato V, Dal Canton A, Esposito C, Esposito P, Tovo PA
Nephrol Dial Transplant 2012 May;27(5):1902-10. Epub 2012 Mar 19 doi: 10.1093/ndt/gfs035. PMID: 22431705
Relationship between expression of Pad1 homologue and multidrug resistance of idiopathic nephrotic syndrome.
Ma Z, Gao X, Zhao W, Li Y, Li C, Li C
Pediatr Int 2009 Oct;51(5):732-5. Epub 2009 Mar 20 doi: 10.1111/j.1442-200X.2009.02845.x. PMID: 19419512

Prognosis

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.
Trautmann A, Schnaidt S, Lipska-Ziętkiewicz BS, Bodria M, Ozaltin F, Emma F, Anarat A, Melk A, Azocar M, Oh J, Saeed B, Gheisari A, Caliskan S, Gellermann J, Higuita LMS, Jankauskiene A, Drozdz D, Mir S, Balat A, Szczepanska M, Paripovic D, Zurowska A, Bogdanovic R, Yilmaz A, Ranchin B, Baskin E, Erdogan O, Remuzzi G, Firszt-Adamczyk A, Kuzma-Mroczkowska E, Litwin M, Murer L, Tkaczyk M, Jardim H, Wasilewska A, Printza N, Fidan K, Simkova E, Borzecka H, Staude H, Hees K, Schaefer F; PodoNet Consortium
J Am Soc Nephrol 2017 Oct;28(10):3055-3065. Epub 2017 May 31 doi: 10.1681/ASN.2016101121. PMID: 28566477Free PMC Article
Focal segmental glomerulosclerosis and medullary nephrocalcinosis in children with ADCK4 mutations.
Park E, Kang HG, Choi YH, Lee KB, Moon KC, Jeong HJ, Nagata M, Cheong HI
Pediatr Nephrol 2017 Sep;32(9):1547-1554. Epub 2017 Apr 12 doi: 10.1007/s00467-017-3657-9. PMID: 28405841
Saquinavir in steroid-dependent and -resistant nephrotic syndrome: a pilot study.
Coppo R, Camilla R, Porcellini MG, Peruzzi L, Gianoglio B, Amore A, Daprà V, Loiacono E, Fonsato V, Dal Canton A, Esposito C, Esposito P, Tovo PA
Nephrol Dial Transplant 2012 May;27(5):1902-10. Epub 2012 Mar 19 doi: 10.1093/ndt/gfs035. PMID: 22431705

Clinical prediction guides

Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Saquinavir in steroid-dependent and -resistant nephrotic syndrome: a pilot study.
Coppo R, Camilla R, Porcellini MG, Peruzzi L, Gianoglio B, Amore A, Daprà V, Loiacono E, Fonsato V, Dal Canton A, Esposito C, Esposito P, Tovo PA
Nephrol Dial Transplant 2012 May;27(5):1902-10. Epub 2012 Mar 19 doi: 10.1093/ndt/gfs035. PMID: 22431705

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