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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

Treatment of childhood steroid-resistant nephrotic syndrome with pulse methylprednisolone and cyclophosphamide.
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

Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.
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
Long-Term Efficacy and Safety of Repeated Rituximab to Maintain Remission in Idiopathic Childhood Nephrotic Syndrome: An International Study.
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
Mycophenolate Mofetil after Rituximab for Childhood-Onset Complicated Frequently-Relapsing or Steroid-Dependent Nephrotic Syndrome.
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
Nephrotic Syndrome.
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Nephrotic syndrome.
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

Diagnosis

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
Nephrotic Syndrome.
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Nephrotic syndrome in infants and children: pathophysiology and management.
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
Nephrotic syndrome.
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
Nephrotic syndrome in childhood.
Eddy AA, Symons JM
Lancet 2003 Aug 23;362(9384):629-39. doi: 10.1016/S0140-6736(03)14184-0. PMID: 12944064

Therapy

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
Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.
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
Mycophenolate Mofetil after Rituximab for Childhood-Onset Complicated Frequently-Relapsing or Steroid-Dependent Nephrotic Syndrome.
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
Nephrotic Syndrome.
Wang CS, Greenbaum LA
Pediatr Clin North Am 2019 Feb;66(1):73-85. doi: 10.1016/j.pcl.2018.08.006. PMID: 30454752
Nephrotic syndrome in childhood.
Eddy AA, Symons JM
Lancet 2003 Aug 23;362(9384):629-39. doi: 10.1016/S0140-6736(03)14184-0. PMID: 12944064

Prognosis

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
Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.
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
Long-Term Efficacy and Safety of Repeated Rituximab to Maintain Remission in Idiopathic Childhood Nephrotic Syndrome: An International Study.
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
Nephrotic syndrome in infants and children: pathophysiology and management.
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
Nephrotic syndrome in childhood.
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

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
Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.
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
MicroRNAs in childhood nephrotic syndrome.
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
Nephrotic syndrome.
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
Chlorambucil-induced seizures.
Salloum E, Khan KK, Cooper DL
Cancer 1997 Mar 1;79(5):1009-13. doi: 10.1002/(sici)1097-0142(19970301)79:5<1009::aid-cncr18>3.0.co;2-5. PMID: 9041164

Recent systematic reviews

Interventions for preventing and treating kidney disease in IgA vasculitis.
Hahn D, Hodson EM, Craig JC
Cochrane Database Syst Rev 2023 Feb 28;2(2):CD005128. doi: 10.1002/14651858.CD005128.pub4. PMID: 36853224Free PMC Article
Childhood nephrotic syndrome and the clinical profile of thromboembolism: a systematic review and meta-analysis.
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
Neurological complications in childhood nephrotic syndrome: A systematic review.
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
Comparison of second-line immunosuppressants for childhood refractory nephrotic syndrome: a systematic review and network meta-analysis.
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
Rituximab in the treatment of nephrotic syndrome: a systematic review.
Otukesh H, Hoseini R, Rahimzadeh N, Fazel M
Iran J Kidney Dis 2013 Jul;7(4):249-56. PMID: 23880800

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