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Steroid resistant nephrotic syndrome of childhood

MedGen UID:
757877
Concept ID:
C3266102
Disease or Syndrome
Synonym: Nephrotic Syndrome of Childhood - Steroid Resistant
SNOMED CT: Steroid resistant nephrotic syndrome of childhood (449820008)

Definition

Nephrotic syndrome, occurring in the pediatric population, in which proteinuria does not normalize with administration of steroids; this condition is unresponsive to a minimum of four weeks administration of oral corticosteroids. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSteroid resistant nephrotic syndrome of childhood

Professional guidelines

PubMed

Sa GA, Luis JP, Mendonca E, Almeida M, Rosa FC
Pediatr Nephrol 1996 Apr;10(2):250. doi: 10.1007/BF00862097. PMID: 8703725

Recent clinical studies

Etiology

Barry A, McNulty MT, Jia X, Gupta Y, Debiec H, Luo Y, Nagano C, Horinouchi T, Jung S, Colucci M, Ahram DF, Mitrotti A, Sinha A, Teeninga N, Jin G, Shril S, Caridi G, Bodria M, Lim TY, Westland R, Zanoni F, Marasa M, Turudic D, Giordano M, Gesualdo L, Magistroni R, Pisani I, Fiaccadori E, Reiterova J, Maringhini S, Morello W, Montini G, Weng PL, Scolari F, Saraga M, Tasic V, Santoro D, van Wijk JAE, Milošević D, Kawai Y, Kiryluk K, Pollak MR, Gharavi A, Lin F, Simœs E Silva AC, Loos RJF, Kenny EE, Schreuder MF, Zurowska A, Dossier C, Ariceta G, Drozynska-Duklas M, Hogan J, Jankauskiene A, Hildebrandt F, Prikhodina L, Song K, Bagga A, Cheong H 2nd, Ghiggeri GM, Vachvanichsanong P, Nozu K, Lee D, Vivarelli M, Raychaudhuri S, Tokunaga K, Sanna-Cherchi S, Ronco P, Iijima K, Sampson MG
Nat Commun 2023 Apr 29;14(1):2481. doi: 10.1038/s41467-023-37985-w. PMID: 37120605Free PMC Article
Chan EY, Yap DY, Colucci M, Ma AL, Parekh RS, Tullus K
Clin J Am Soc Nephrol 2023 Apr 1;18(4):533-548. Epub 2023 Feb 22 doi: 10.2215/CJN.08570722. PMID: 36456193Free PMC Article
Chan EY, Yu ELM, Angeletti A, Arslan Z, Basu B, Boyer O, Chan CY, Colucci M, Dorval G, Dossier C, Drovandi S, Ghiggeri GM, Gipson DS, Hamada R, Hogan J, Ishikura K, Kamei K, Kemper MJ, Ma AL, Parekh RS, Radhakrishnan S, Saini P, Shen Q, Sinha R, Subun C, Teo S, Vivarelli M, Webb H, Xu H, Yap HK, Tullus K
J Am Soc Nephrol 2022 Jun;33(6):1193-1207. Epub 2022 Mar 30 doi: 10.1681/ASN.2021111472. PMID: 35354600Free PMC Article
Iijima K, Sako M, Oba M, Tanaka S, Hamada R, Sakai T, Ohwada Y, Ninchoji T, Yamamura T, Machida H, Shima Y, Tanaka R, Kaito H, Araki Y, Morohashi T, Kumagai N, Gotoh Y, Ikezumi Y, Kubota T, Kamei K, Fujita N, Ohtsuka Y, Okamoto T, Yamada T, Tanaka E, Shimizu M, Horinochi T, Konishi A, Omori T, Nakanishi K, Ishikura K, Ito S, Nakamura H, Nozu K; Japanese Study Group of Kidney Disease in Children
J Am Soc Nephrol 2022 Feb;33(2):401-419. Epub 2021 Dec 8 doi: 10.1681/ASN.2021050643. PMID: 34880074Free PMC Article
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752

Diagnosis

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
Barry A, McNulty MT, Jia X, Gupta Y, Debiec H, Luo Y, Nagano C, Horinouchi T, Jung S, Colucci M, Ahram DF, Mitrotti A, Sinha A, Teeninga N, Jin G, Shril S, Caridi G, Bodria M, Lim TY, Westland R, Zanoni F, Marasa M, Turudic D, Giordano M, Gesualdo L, Magistroni R, Pisani I, Fiaccadori E, Reiterova J, Maringhini S, Morello W, Montini G, Weng PL, Scolari F, Saraga M, Tasic V, Santoro D, van Wijk JAE, Milošević D, Kawai Y, Kiryluk K, Pollak MR, Gharavi A, Lin F, Simœs E Silva AC, Loos RJF, Kenny EE, Schreuder MF, Zurowska A, Dossier C, Ariceta G, Drozynska-Duklas M, Hogan J, Jankauskiene A, Hildebrandt F, Prikhodina L, Song K, Bagga A, Cheong H 2nd, Ghiggeri GM, Vachvanichsanong P, Nozu K, Lee D, Vivarelli M, Raychaudhuri S, Tokunaga K, Sanna-Cherchi S, Ronco P, Iijima K, Sampson MG
Nat Commun 2023 Apr 29;14(1):2481. doi: 10.1038/s41467-023-37985-w. PMID: 37120605Free PMC Article
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Downie ML, Gallibois C, Parekh RS, Noone DG
Paediatr Int Child Health 2017 Nov;37(4):248-258. Epub 2017 Sep 15 doi: 10.1080/20469047.2017.1374003. PMID: 28914167
Eddy AA, Symons JM
Lancet 2003 Aug 23;362(9384):629-39. doi: 10.1016/S0140-6736(03)14184-0. PMID: 12944064

Therapy

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
Chan EY, Yap DY, Colucci M, Ma AL, Parekh RS, Tullus K
Clin J Am Soc Nephrol 2023 Apr 1;18(4):533-548. Epub 2023 Feb 22 doi: 10.2215/CJN.08570722. PMID: 36456193Free PMC Article
Iijima K, Sako M, Oba M, Tanaka S, Hamada R, Sakai T, Ohwada Y, Ninchoji T, Yamamura T, Machida H, Shima Y, Tanaka R, Kaito H, Araki Y, Morohashi T, Kumagai N, Gotoh Y, Ikezumi Y, Kubota T, Kamei K, Fujita N, Ohtsuka Y, Okamoto T, Yamada T, Tanaka E, Shimizu M, Horinochi T, Konishi A, Omori T, Nakanishi K, Ishikura K, Ito S, Nakamura H, Nozu K; Japanese Study Group of Kidney Disease in Children
J Am Soc Nephrol 2022 Feb;33(2):401-419. Epub 2021 Dec 8 doi: 10.1681/ASN.2021050643. PMID: 34880074Free PMC Article
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Eddy AA, Symons JM
Lancet 2003 Aug 23;362(9384):629-39. doi: 10.1016/S0140-6736(03)14184-0. PMID: 12944064

Prognosis

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
Chan EY, Yap DY, Colucci M, Ma AL, Parekh RS, Tullus K
Clin J Am Soc Nephrol 2023 Apr 1;18(4):533-548. Epub 2023 Feb 22 doi: 10.2215/CJN.08570722. PMID: 36456193Free PMC Article
Chan EY, Yu ELM, Angeletti A, Arslan Z, Basu B, Boyer O, Chan CY, Colucci M, Dorval G, Dossier C, Drovandi S, Ghiggeri GM, Gipson DS, Hamada R, Hogan J, Ishikura K, Kamei K, Kemper MJ, Ma AL, Parekh RS, Radhakrishnan S, Saini P, Shen Q, Sinha R, Subun C, Teo S, Vivarelli M, Webb H, Xu H, Yap HK, Tullus K
J Am Soc Nephrol 2022 Jun;33(6):1193-1207. Epub 2022 Mar 30 doi: 10.1681/ASN.2021111472. PMID: 35354600Free PMC Article
Downie ML, Gallibois C, Parekh RS, Noone DG
Paediatr Int Child Health 2017 Nov;37(4):248-258. Epub 2017 Sep 15 doi: 10.1080/20469047.2017.1374003. PMID: 28914167
Eddy AA, Symons JM
Lancet 2003 Aug 23;362(9384):629-39. doi: 10.1016/S0140-6736(03)14184-0. PMID: 12944064

Clinical prediction guides

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
Barry A, McNulty MT, Jia X, Gupta Y, Debiec H, Luo Y, Nagano C, Horinouchi T, Jung S, Colucci M, Ahram DF, Mitrotti A, Sinha A, Teeninga N, Jin G, Shril S, Caridi G, Bodria M, Lim TY, Westland R, Zanoni F, Marasa M, Turudic D, Giordano M, Gesualdo L, Magistroni R, Pisani I, Fiaccadori E, Reiterova J, Maringhini S, Morello W, Montini G, Weng PL, Scolari F, Saraga M, Tasic V, Santoro D, van Wijk JAE, Milošević D, Kawai Y, Kiryluk K, Pollak MR, Gharavi A, Lin F, Simœs E Silva AC, Loos RJF, Kenny EE, Schreuder MF, Zurowska A, Dossier C, Ariceta G, Drozynska-Duklas M, Hogan J, Jankauskiene A, Hildebrandt F, Prikhodina L, Song K, Bagga A, Cheong H 2nd, Ghiggeri GM, Vachvanichsanong P, Nozu K, Lee D, Vivarelli M, Raychaudhuri S, Tokunaga K, Sanna-Cherchi S, Ronco P, Iijima K, Sampson MG
Nat Commun 2023 Apr 29;14(1):2481. doi: 10.1038/s41467-023-37985-w. PMID: 37120605Free PMC Article
Chan EY, Yap DY, Colucci M, Ma AL, Parekh RS, Tullus K
Clin J Am Soc Nephrol 2023 Apr 1;18(4):533-548. Epub 2023 Feb 22 doi: 10.2215/CJN.08570722. PMID: 36456193Free PMC Article
Dhandapani MC, Venkatesan V, Pricilla C
J Cell Physiol 2021 Oct;236(10):7186-7210. Epub 2021 Apr 5 doi: 10.1002/jcp.30374. PMID: 33819345
Sinha A, Bagga A
Indian J Pediatr 2012 Aug;79(8):1045-55. Epub 2012 May 30 doi: 10.1007/s12098-012-0776-y. PMID: 22644544

Recent systematic reviews

Dadgar K, Xue Y, Chung J, Sangar S, Bhatt M, Chan AKC, Geddie H, Chanchlani R
Pediatr Res 2023 May;93(6):1463-1469. Epub 2022 Sep 20 doi: 10.1038/s41390-022-02302-6. PMID: 36127405
Stabouli S, Chrysaidou K, Kupferman JC, Zafeiriou DI
Eur J Paediatr Neurol 2019 May;23(3):384-391. Epub 2019 Feb 22 doi: 10.1016/j.ejpn.2019.02.007. PMID: 30837193
Pasini A, Benetti E, Conti G, Ghio L, Lepore M, Massella L, Molino D, Peruzzi L, Emma F, Fede C, Trivelli A, Maringhini S, Materassi M, Messina G, Montini G, Murer L, Pecoraro C, Pennesi M
Ital J Pediatr 2017 Apr 21;43(1):41. doi: 10.1186/s13052-017-0356-x. PMID: 28427453Free PMC Article
Fu HD, Qian GL, Jiang ZY
J Investig Med 2017 Jan;65(1):65-71. Epub 2016 Aug 3 doi: 10.1136/jim-2016-000163. PMID: 27489255
Otukesh H, Hoseini R, Rahimzadeh N, Fazel M
Iran J Kidney Dis 2013 Jul;7(4):249-56. PMID: 23880800

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