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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
Schijvens AM, van der Weerd L, van Wijk JAE, Bouts AHM, Keijzer-Veen MG, Dorresteijn EM, Schreuder MF
Eur J Pediatr 2021 Jun;180(6):1885-1894. Epub 2021 Feb 3 doi: 10.1007/s00431-021-03958-8. PMID: 33532891Free 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

Recent clinical studies

Etiology

Dossier C, Bonneric S, Baudouin V, Kwon T, Prim B, Cambier A, Couderc A, Moreau C, Deschenes G, Hogan J
Clin J Am Soc Nephrol 2023 Dec 1;18(12):1555-1562. Epub 2023 Sep 6 doi: 10.2215/CJN.0000000000000288. PMID: 37678236Free PMC Article
Colucci M, Angeletti A, Zotta F, Carsetti R, Lugani F, Ravà L, Ravani P, Emma F, Ghiggeri GM, Vivarelli M
Kidney Int 2023 Sep;104(3):577-586. Epub 2023 Jun 28 doi: 10.1016/j.kint.2023.06.013. PMID: 37385541
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

Diagnosis

Robinson CH, Parekh RS
Clin J Am Soc Nephrol 2023 Dec 1;18(12):1527-1529. Epub 2023 Oct 26 doi: 10.2215/CJN.0000000000000344. PMID: 37883188Free PMC Article
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, Tullus K
Pediatr Nephrol 2021 Jun;36(6):1397-1405. Epub 2020 Jun 24 doi: 10.1007/s00467-020-04609-0. PMID: 32577808
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

Therapy

Dossier C, Bonneric S, Baudouin V, Kwon T, Prim B, Cambier A, Couderc A, Moreau C, Deschenes G, Hogan J
Clin J Am Soc Nephrol 2023 Dec 1;18(12):1555-1562. Epub 2023 Sep 6 doi: 10.2215/CJN.0000000000000288. PMID: 37678236Free PMC Article
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
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
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

Takemasa Y, Fujinaga S, Nakagawa M, Sakuraya K, Hirano D
Pediatr Nephrol 2024 Feb;39(2):473-482. Epub 2023 Aug 22 doi: 10.1007/s00467-023-06108-4. PMID: 37608237
Colucci M, Angeletti A, Zotta F, Carsetti R, Lugani F, Ravà L, Ravani P, Emma F, Ghiggeri GM, Vivarelli M
Kidney Int 2023 Sep;104(3):577-586. Epub 2023 Jun 28 doi: 10.1016/j.kint.2023.06.013. PMID: 37385541
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

Clinical prediction guides

Takemasa Y, Fujinaga S, Nakagawa M, Sakuraya K, Hirano D
Pediatr Nephrol 2024 Feb;39(2):473-482. Epub 2023 Aug 22 doi: 10.1007/s00467-023-06108-4. PMID: 37608237
Ohyama R, Fujinaga S, Sakuraya K, Hirano D, Ito S
Clin Exp Nephrol 2023 Oct;27(10):865-872. Epub 2023 Jul 21 doi: 10.1007/s10157-023-02374-6. PMID: 37477752
Colucci M, Angeletti A, Zotta F, Carsetti R, Lugani F, Ravà L, Ravani P, Emma F, Ghiggeri GM, Vivarelli M
Kidney Int 2023 Sep;104(3):577-586. Epub 2023 Jun 28 doi: 10.1016/j.kint.2023.06.013. PMID: 37385541
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
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

Recent systematic reviews

Zhu Y, Chen J, Zhang Y, Wang X, Wang J
Front Immunol 2024;15:1310032. Epub 2024 Feb 23 doi: 10.3389/fimmu.2024.1310032. PMID: 38464533Free PMC Article
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

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