U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Steroid-dependent nephrotic syndrome

MedGen UID:
588370
Concept ID:
C0403398
Disease or Syndrome
Synonyms: Nephrotic Syndrome, Steroid-Dependent; Steroid Dependent Nephrotic Syndrome; Steroid-Dependent Nephrotic Syndrome; Steroid-Dependent Nephrotic Syndromes
SNOMED CT: Steroid-dependent nephrotic syndrome (236382007); SDNS - Steroid-dependent nephrotic syndrome (236382007)
 
HPO: HP:0025650

Definition

A type of nephrotic syndrome in which two consecutive relapses (albumin urine dipstick at least 3+ or proteinuria greater than 40 mg/m2/d or urinary protein to creatinine ratio at least 2000 mg/g for 3 consecutive days) occur during corticosteroid therapy, or within 14 days of ceasing therapy. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSteroid-dependent nephrotic syndrome

Conditions with this feature

X-linked intellectual disability, Cantagrel type
MedGen UID:
813060
Concept ID:
C3806730
Disease or Syndrome
X-linked intellectual developmental disorder-98 (XLID98) is a neurodevelopmental disorder characterized by delayed psychomotor development, poor speech, behavioral abnormalities, poor overall growth, dysmorphic facial features, and often early-onset seizures. Some carrier females are unaffected, whereas other females with mutations are affected; males tend to be more severely affected than females. It is believed that the phenotypic variability and disease manifestations in female carriers results from skewed X-inactivation or cellular mosaicism (summary by de Lange et al., 2016).

Professional guidelines

PubMed

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Larkins N, Kim S, Craig J, Hodson E
Arch Dis Child 2016 Apr;101(4):404-8. Epub 2015 Aug 19 doi: 10.1136/archdischild-2015-308924. PMID: 26289063
Wang X, Xu H
Contrib Nephrol 2013;181:119-30. Epub 2013 May 8 doi: 10.1159/000348470. PMID: 23689574

Recent clinical studies

Etiology

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
Ravani P, Lugani F, Drovandi S, Caridi G, Angeletti A, Ghiggeri GM
JAMA Pediatr 2021 Jun 1;175(6):631-632. doi: 10.1001/jamapediatrics.2020.6150. PMID: 33616641Free PMC Article
Iijima K, Sako M, Nozu K
Clin Exp Nephrol 2017 Apr;21(2):193-202. Epub 2016 Jul 15 doi: 10.1007/s10157-016-1313-5. PMID: 27422620Free PMC Article
Iijima K, Sako M, Nozu K, Mori R, Tuchida N, Kamei K, Miura K, Aya K, Nakanishi K, Ohtomo Y, Takahashi S, Tanaka R, Kaito H, Nakamura H, Ishikura K, Ito S, Ohashi Y; Rituximab for Childhood-onset Refractory Nephrotic Syndrome (RCRNS) Study Group
Lancet 2014 Oct 4;384(9950):1273-81. Epub 2014 Jun 22 doi: 10.1016/S0140-6736(14)60541-9. PMID: 24965823

Diagnosis

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Larkins NG, Liu ID, Willis NS, Craig JC, Hodson EM
Cochrane Database Syst Rev 2020 Apr 16;4(4):CD002290. doi: 10.1002/14651858.CD002290.pub5. PMID: 32297308Free PMC Article
Iijima K, Sako M, Kamei K, Nozu K
Pediatr Nephrol 2018 Sep;33(9):1449-1455. Epub 2017 Jul 17 doi: 10.1007/s00467-017-3746-9. PMID: 28717938Free PMC Article
Iijima K, Sako M, Nozu K
Clin Exp Nephrol 2017 Apr;21(2):193-202. Epub 2016 Jul 15 doi: 10.1007/s10157-016-1313-5. PMID: 27422620Free PMC Article
Iijima K, Sako M, Nozu K, Mori R, Tuchida N, Kamei K, Miura K, Aya K, Nakanishi K, Ohtomo Y, Takahashi S, Tanaka R, Kaito H, Nakamura H, Ishikura K, Ito S, Ohashi Y; Rituximab for Childhood-onset Refractory Nephrotic Syndrome (RCRNS) Study Group
Lancet 2014 Oct 4;384(9950):1273-81. Epub 2014 Jun 22 doi: 10.1016/S0140-6736(14)60541-9. PMID: 24965823

Therapy

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Basu B, Angeletti A, Islam B, Ghiggeri GM
Front Immunol 2022;13:805697. Epub 2022 Feb 11 doi: 10.3389/fimmu.2022.805697. PMID: 35222385Free PMC Article
Kamei K, Ishikura K, Sako M, Ito S, Nozu K, Iijima K
Pediatr Nephrol 2020 Jan;35(1):17-24. Epub 2018 Dec 18 doi: 10.1007/s00467-018-4166-1. PMID: 30564879
Iijima K, Sako M, Nozu K
Clin Exp Nephrol 2017 Apr;21(2):193-202. Epub 2016 Jul 15 doi: 10.1007/s10157-016-1313-5. PMID: 27422620Free PMC Article
Sinha A, Bagga A
Indian J Pediatr 2008 Oct;75(10):1057-66. Epub 2008 Nov 21 doi: 10.1007/s12098-008-0210-7. PMID: 19023530

Prognosis

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free 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
Bertrand Q, Mignot S, Kwon T, Couderc A, Maisin A, Cambier A, Baudouin V, Peyneau M, Deschênes G, Hogan J, Dossier C
Pediatr Nephrol 2022 Feb;37(2):357-365. Epub 2021 Jun 16 doi: 10.1007/s00467-021-05069-w. PMID: 34132894
Ravani P, Colucci M, Bruschi M, Vivarelli M, Cioni M, DiDonato A, Cravedi P, Lugani F, Antonini F, Prunotto M, Emma F, Angeletti A, Ghiggeri GM
J Am Soc Nephrol 2021 Oct;32(10):2652-2663. Epub 2021 Sep 20 doi: 10.1681/ASN.2021040561. PMID: 34544820Free PMC Article
Larkins NG, Liu ID, Willis NS, Craig JC, Hodson EM
Cochrane Database Syst Rev 2020 Apr 16;4(4):CD002290. doi: 10.1002/14651858.CD002290.pub5. PMID: 32297308Free PMC Article

Clinical prediction guides

Guzmán Morais B, Ordóñez Álvarez FÁ, Santos Rodríguez F, Martín Ramos S, Fernández Novo G
An Pediatr (Engl Ed) 2022 Feb;96(2):83-90. Epub 2022 Feb 8 doi: 10.1016/j.anpede.2020.12.019. PMID: 35144901
Bertrand Q, Mignot S, Kwon T, Couderc A, Maisin A, Cambier A, Baudouin V, Peyneau M, Deschênes G, Hogan J, Dossier C
Pediatr Nephrol 2022 Feb;37(2):357-365. Epub 2021 Jun 16 doi: 10.1007/s00467-021-05069-w. PMID: 34132894
Ravani P, Colucci M, Bruschi M, Vivarelli M, Cioni M, DiDonato A, Cravedi P, Lugani F, Antonini F, Prunotto M, Emma F, Angeletti A, Ghiggeri GM
J Am Soc Nephrol 2021 Oct;32(10):2652-2663. Epub 2021 Sep 20 doi: 10.1681/ASN.2021040561. PMID: 34544820Free PMC Article
Kamei K, Ishikura K, Sako M, Ito S, Nozu K, Iijima K
Pediatr Nephrol 2020 Jan;35(1):17-24. Epub 2018 Dec 18 doi: 10.1007/s00467-018-4166-1. PMID: 30564879
Gorsane I, Helal I, Yacoub I, Hamida FB, Abderrahim E, Abdallah TB
Saudi J Kidney Dis Transpl 2016 Sep-Oct;27(5):958-965. doi: 10.4103/1319-2442.190864. PMID: 27752004

Recent systematic reviews

Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, Wetzels J, Alhasan K, Banerjee S, Bhimma R, Bonilla-Felix M, Cano F, Christian M, Hahn D, Kang HG, Nakanishi K, Safouh H, Trachtman H, Xu H, Cook W, Vivarelli M, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2023 Mar;38(3):877-919. Epub 2022 Oct 21 doi: 10.1007/s00467-022-05739-3. PMID: 36269406Free PMC Article
Larkins NG, Liu ID, Willis NS, Craig JC, Hodson EM
Cochrane Database Syst Rev 2020 Apr 16;4(4):CD002290. doi: 10.1002/14651858.CD002290.pub5. PMID: 32297308Free PMC Article
Kronbichler A, Kerschbaum J, Fernandez-Fresnedo G, Hoxha E, Kurschat CE, Busch M, Bruchfeld A, Mayer G, Rudnicki M
Am J Nephrol 2014;39(4):322-30. Epub 2014 Apr 15 doi: 10.1159/000360908. PMID: 24751753

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...