U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Idiopathic steroid-sensitive nephrotic syndrome

MedGen UID:
978326
•Concept ID:
CN294189
•
Disease or Syndrome
Synonym: idiopathic steroid-sensitive nephrotic syndrome
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0019067
Orphanet: ORPHA69061

Definition

A rare primary glomerulopathy of unknown cause characterized by edema, nephrotic-range proteinuria and hypoalbuminemia that responds to standard prednisone treatment within 4-6 weeks. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic steroid-sensitive nephrotic syndrome

Professional guidelines

PubMed

Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020.
Ehren R, Benz MR, Brinkkötter PT, Dötsch J, Eberl WR, Gellermann J, Hoyer PF, Jordans I, Kamrath C, Kemper MJ, Latta K, Müller D, Oh J, Tönshoff B, Weber S, Weber LT; German Society for Pediatric Nephrology
Pediatr Nephrol 2021 Oct;36(10):2971-2985. Epub 2021 Jun 6 doi: 10.1007/s00467-021-05135-3. PMID: 34091756Free PMC Article
Commentary on "Pediatric Idiopathic Steroid-sensitive Nephrotic Syndrome Diagnosis and Therapy - Short version of the updated German Best Practice Guideline (S2e)".
Ehren R, Benz MR, Brinkkötter PT, Dötsch J, Eberl WR, Gellermann J, Hoyer PF, Jordans I, Kamrath C, Kemper MJ, Latta K, Müller D, Oh J, Tönshoff B, Weber S, Weber LT; German Society for Pediatric Nephrology
Pediatr Nephrol 2021 Oct;36(10):2961-2966. Epub 2021 Jun 5 doi: 10.1007/s00467-021-05136-2. PMID: 34091755Free PMC Article
AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.
Alhasan KA, Al Khalifah R, Aloufi M, Almaiman W, Hamad M, Abdulmajeed N, Al Salloum A, Kari JA, AlJelaify M, Bassrawi RK, Al Hussain T, Alherbish A, Al Talhi A, Temsah MH, Sethi SK, Raina R, Joseph R, Amer YS; Saudi Society of Nephrology and Transplantation
Syst Rev 2021 May 10;10(1):144. doi: 10.1186/s13643-021-01666-w. PMID: 33971949Free PMC Article

Recent clinical studies

Etiology

Developmental Abnormalities of Teeth in Children With Nephrotic Syndrome.
Piekoszewska-Ziętek P, Olczak-Kowalczyk D, Pańczyk-Tomaszewska M, Gozdowski D
Int Dent J 2022 Aug;72(4):572-577. Epub 2022 Mar 8 doi: 10.1016/j.identj.2021.11.014. PMID: 35277264Free PMC Article
Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020.
Ehren R, Benz MR, Brinkkötter PT, Dötsch J, Eberl WR, Gellermann J, Hoyer PF, Jordans I, Kamrath C, Kemper MJ, Latta K, Müller D, Oh J, Tönshoff B, Weber S, Weber LT; German Society for Pediatric Nephrology
Pediatr Nephrol 2021 Oct;36(10):2971-2985. Epub 2021 Jun 6 doi: 10.1007/s00467-021-05135-3. PMID: 34091756Free PMC Article
Selected salivary parameters in children with idiopathic nephrotic syndrome: a preliminary study.
Kaczmarek U, Wrzyszcz-Kowalczyk A, Jankowska K, Prościak K, Mysiak-Dębska M, Przywitowska I, Makulska I
BMC Oral Health 2021 Jan 7;21(1):17. doi: 10.1186/s12903-020-01375-1. PMID: 33413282Free PMC Article
Th2 cells predominate in idiopathic steroid-sensitive nephrotic syndrome.
Kanai T, Shiraishi H, Yamagata T, Ito T, Odaka J, Saito T, Aoyagi J, Momoi MY
Clin Exp Nephrol 2010 Dec;14(6):578-83. Epub 2010 Aug 5 doi: 10.1007/s10157-010-0330-z. PMID: 20686809
Stem cell mobilization in idiopathic steroid-sensitive nephrotic syndrome.
Lapillonne H, Leclerc A, Ulinski T, Balu L, Garnier A, Dereuddre-Bosquet N, Watier H, Schlageter MH, Deschênes G
Pediatr Nephrol 2008 Aug;23(8):1251-6. Epub 2008 May 6 doi: 10.1007/s00467-008-0793-2. PMID: 18458957

Diagnosis

Developmental Abnormalities of Teeth in Children With Nephrotic Syndrome.
Piekoszewska-Ziętek P, Olczak-Kowalczyk D, Pańczyk-Tomaszewska M, Gozdowski D
Int Dent J 2022 Aug;72(4):572-577. Epub 2022 Mar 8 doi: 10.1016/j.identj.2021.11.014. PMID: 35277264Free PMC Article
Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020.
Ehren R, Benz MR, Brinkkötter PT, Dötsch J, Eberl WR, Gellermann J, Hoyer PF, Jordans I, Kamrath C, Kemper MJ, Latta K, Müller D, Oh J, Tönshoff B, Weber S, Weber LT; German Society for Pediatric Nephrology
Pediatr Nephrol 2021 Oct;36(10):2971-2985. Epub 2021 Jun 6 doi: 10.1007/s00467-021-05135-3. PMID: 34091756Free PMC Article
AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.
Alhasan KA, Al Khalifah R, Aloufi M, Almaiman W, Hamad M, Abdulmajeed N, Al Salloum A, Kari JA, AlJelaify M, Bassrawi RK, Al Hussain T, Alherbish A, Al Talhi A, Temsah MH, Sethi SK, Raina R, Joseph R, Amer YS; Saudi Society of Nephrology and Transplantation
Syst Rev 2021 May 10;10(1):144. doi: 10.1186/s13643-021-01666-w. PMID: 33971949Free PMC Article
Selected salivary parameters in children with idiopathic nephrotic syndrome: a preliminary study.
Kaczmarek U, Wrzyszcz-Kowalczyk A, Jankowska K, Prościak K, Mysiak-Dębska M, Przywitowska I, Makulska I
BMC Oral Health 2021 Jan 7;21(1):17. doi: 10.1186/s12903-020-01375-1. PMID: 33413282Free PMC Article
Human leukocyte antigen association with familial steroid-sensitive nephrotic syndrome.
Korsgaard T, Joshi S, Andersen RF, Moeller K, Seeman T, Podracká L, Eiberg H, Rittig S
Eur J Pediatr 2020 Sep;179(9):1481-1486. Epub 2020 Mar 20 doi: 10.1007/s00431-020-03634-3. PMID: 32198629

Therapy

Developmental Abnormalities of Teeth in Children With Nephrotic Syndrome.
Piekoszewska-Ziętek P, Olczak-Kowalczyk D, Pańczyk-Tomaszewska M, Gozdowski D
Int Dent J 2022 Aug;72(4):572-577. Epub 2022 Mar 8 doi: 10.1016/j.identj.2021.11.014. PMID: 35277264Free PMC Article
Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020.
Ehren R, Benz MR, Brinkkötter PT, Dötsch J, Eberl WR, Gellermann J, Hoyer PF, Jordans I, Kamrath C, Kemper MJ, Latta K, Müller D, Oh J, Tönshoff B, Weber S, Weber LT; German Society for Pediatric Nephrology
Pediatr Nephrol 2021 Oct;36(10):2971-2985. Epub 2021 Jun 6 doi: 10.1007/s00467-021-05135-3. PMID: 34091756Free PMC Article
AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.
Alhasan KA, Al Khalifah R, Aloufi M, Almaiman W, Hamad M, Abdulmajeed N, Al Salloum A, Kari JA, AlJelaify M, Bassrawi RK, Al Hussain T, Alherbish A, Al Talhi A, Temsah MH, Sethi SK, Raina R, Joseph R, Amer YS; Saudi Society of Nephrology and Transplantation
Syst Rev 2021 May 10;10(1):144. doi: 10.1186/s13643-021-01666-w. PMID: 33971949Free PMC Article
Th2 cells predominate in idiopathic steroid-sensitive nephrotic syndrome.
Kanai T, Shiraishi H, Yamagata T, Ito T, Odaka J, Saito T, Aoyagi J, Momoi MY
Clin Exp Nephrol 2010 Dec;14(6):578-83. Epub 2010 Aug 5 doi: 10.1007/s10157-010-0330-z. PMID: 20686809
Stem cell mobilization in idiopathic steroid-sensitive nephrotic syndrome.
Lapillonne H, Leclerc A, Ulinski T, Balu L, Garnier A, Dereuddre-Bosquet N, Watier H, Schlageter MH, Deschênes G
Pediatr Nephrol 2008 Aug;23(8):1251-6. Epub 2008 May 6 doi: 10.1007/s00467-008-0793-2. PMID: 18458957

Prognosis

Pediatric idiopathic steroid-sensitive nephrotic syndrome: diagnosis and therapy -short version of the updated German best practice guideline (S2e) - AWMF register no. 166-001, 6/2020.
Ehren R, Benz MR, Brinkkötter PT, Dötsch J, Eberl WR, Gellermann J, Hoyer PF, Jordans I, Kamrath C, Kemper MJ, Latta K, Müller D, Oh J, Tönshoff B, Weber S, Weber LT; German Society for Pediatric Nephrology
Pediatr Nephrol 2021 Oct;36(10):2971-2985. Epub 2021 Jun 6 doi: 10.1007/s00467-021-05135-3. PMID: 34091756Free PMC Article
Selected salivary parameters in children with idiopathic nephrotic syndrome: a preliminary study.
Kaczmarek U, Wrzyszcz-Kowalczyk A, Jankowska K, Prościak K, Mysiak-Dębska M, Przywitowska I, Makulska I
BMC Oral Health 2021 Jan 7;21(1):17. doi: 10.1186/s12903-020-01375-1. PMID: 33413282Free PMC Article
Long term tapering versus standard prednisolone treatment for first episode of childhood nephrotic syndrome: phase III randomised controlled trial and economic evaluation.
Webb NJA, Woolley RL, Lambe T, Frew E, Brettell EA, Barsoum EN, Trompeter RS, Cummins C, Deeks JJ, Wheatley K, Ives NJ; PREDNOS Collaborative Group
BMJ 2019 May 23;365:l1800. doi: 10.1136/bmj.l1800. PMID: 31335316Free PMC Article
Can we predict relapses in children with idiopathic steroid-sensitive nephrotic syndrome?
Mishra OP, Abhinay A, Mishra RN, Prasad R, Pohl M
J Trop Pediatr 2013 Oct;59(5):343-9. Epub 2013 Apr 24 doi: 10.1093/tropej/fmt029. PMID: 23619602
Elevated serum interleukin-7 level in idiopathic steroid-sensitive nephrotic syndrome.
Kanai T, Shiraishi H, Yamagata T, Ito T, Odaka J, Saito T, Aoyagi J, Momoi MY
Pediatr Int 2011 Dec;53(6):906-9. doi: 10.1111/j.1442-200X.2011.03380.x. PMID: 21486379

Clinical prediction guides

AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.
Alhasan KA, Al Khalifah R, Aloufi M, Almaiman W, Hamad M, Abdulmajeed N, Al Salloum A, Kari JA, AlJelaify M, Bassrawi RK, Al Hussain T, Alherbish A, Al Talhi A, Temsah MH, Sethi SK, Raina R, Joseph R, Amer YS; Saudi Society of Nephrology and Transplantation
Syst Rev 2021 May 10;10(1):144. doi: 10.1186/s13643-021-01666-w. PMID: 33971949Free PMC Article
Glucocorticoid effects on bone strength in children with renal diseases.
Aoyagi J, Kanai T, Ito T, Odaka J, Saito T, Betsui H, Kurosaki M, Maru T, Yamagata T
Nephrology (Carlton) 2021 Feb;26(2):119-125. Epub 2020 Dec 28 doi: 10.1111/nep.13832. PMID: 33207023
Human leukocyte antigen association with familial steroid-sensitive nephrotic syndrome.
Korsgaard T, Joshi S, Andersen RF, Moeller K, Seeman T, Podracká L, Eiberg H, Rittig S
Eur J Pediatr 2020 Sep;179(9):1481-1486. Epub 2020 Mar 20 doi: 10.1007/s00431-020-03634-3. PMID: 32198629
Autoantibodies against podocytic UCHL1 are associated with idiopathic nephrotic syndrome relapses and induce proteinuria in mice.
Jamin A, Berthelot L, Couderc A, Chemouny JM, Boedec E, Dehoux L, Abbad L, Dossier C, Daugas E, Monteiro RC, Deschênes G
J Autoimmun 2018 May;89:149-161. Epub 2018 Jan 4 doi: 10.1016/j.jaut.2017.12.014. PMID: 29307588
Can we predict relapses in children with idiopathic steroid-sensitive nephrotic syndrome?
Mishra OP, Abhinay A, Mishra RN, Prasad R, Pohl M
J Trop Pediatr 2013 Oct;59(5):343-9. Epub 2013 Apr 24 doi: 10.1093/tropej/fmt029. PMID: 23619602

Recent systematic reviews

AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children.
Alhasan KA, Al Khalifah R, Aloufi M, Almaiman W, Hamad M, Abdulmajeed N, Al Salloum A, Kari JA, AlJelaify M, Bassrawi RK, Al Hussain T, Alherbish A, Al Talhi A, Temsah MH, Sethi SK, Raina R, Joseph R, Amer YS; Saudi Society of Nephrology and Transplantation
Syst Rev 2021 May 10;10(1):144. doi: 10.1186/s13643-021-01666-w. PMID: 33971949Free PMC Article

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.

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...