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Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance

MedGen UID:
1830098
•Concept ID:
C5680131
•
Disease or Syndrome
Synonyms: Idiopathic SSNS (steroid-sensitive nephrotic syndrome) with secondary steroid resistance; Idiopathic SSNS with secondary steroid resistance; idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance; Secondary SRNS; Secondary steroid-resistant nephrotic syndrome
SNOMED CT: Secondary steroid-resistant nephrotic syndrome (1260198000); Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance (1260198000); Idiopathic SSNS (steroid-sensitive nephrotic syndrome) with secondary steroid resistance (1260198000)
 
Monarch Initiative: MONDO:0035764
Orphanet: ORPHA567546

Definition

A rare idiopathic nephrotic syndrome characterised by paediatric onset of proteinuria, hypoalbuminaemia and oedema. Patients respond successfully to the initial standard course of corticosteroids, but are resistant to standard therapy for a subsequent relapse and following this relapse remain steroid-resistant. [from SNOMEDCT_US]

Term Hierarchy

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

Professional guidelines

PubMed

Application of next-generation sequencing technology to diagnosis and treatment of focal segmental glomerulosclerosis.
Harita Y
Clin Exp Nephrol 2018 Jun;22(3):491-500. Epub 2017 Jul 27 doi: 10.1007/s10157-017-1449-y. PMID: 28752288Free PMC Article
TACROLIMUS DRUG LEVEL AND RESPONSE TO TREATMENT IN IDIOPATHIC CHILDHOOD STEROID RESISTANT NEPHROTIC SYNDROME.
Shah SS, Hafeez F, Akhtar N
J Ayub Med Coll Abbottabad 2015 Oct-Dec;27(4):784-7. PMID: 27004322
The treatment of idiopathic focal segmental glomerulosclerosis in adults.
Hogan J, Radhakrishnan J
Adv Chronic Kidney Dis 2014 Sep;21(5):434-41. doi: 10.1053/j.ackd.2014.03.016. PMID: 25168833

Recent clinical studies

Etiology

Renal and Urinary Conditions: Nephrotic Syndrome.
Abellada AMP
FP Essent 2024 Aug;543:18-23. PMID: 39163011
Idiopathic nephrotic syndrome and rituximab: may we predict circulating B lymphocytes recovery?
Delbet JD, Leclerc G, Ulinski T
Pediatr Nephrol 2019 Mar;34(3):529-532. Epub 2018 Dec 12 doi: 10.1007/s00467-018-4139-4. PMID: 30542932
Difficult-to-treat idiopathic nephrotic syndrome: established drugs, open questions and future options.
Kemper MJ, Valentin L, van Husen M
Pediatr Nephrol 2018 Oct;33(10):1641-1649. Epub 2017 Sep 6 doi: 10.1007/s00467-017-3780-7. PMID: 28879428
Recurrence and Treatment after Renal Transplantation in Children with FSGS.
Kang HG, Ha IS, Cheong HI
Biomed Res Int 2016;2016:6832971. Epub 2016 Apr 24 doi: 10.1155/2016/6832971. PMID: 27213154Free PMC Article
The treatment of idiopathic focal segmental glomerulosclerosis in adults.
Hogan J, Radhakrishnan J
Adv Chronic Kidney Dis 2014 Sep;21(5):434-41. doi: 10.1053/j.ackd.2014.03.016. PMID: 25168833

Diagnosis

Renal and Urinary Conditions: Nephrotic Syndrome.
Abellada AMP
FP Essent 2024 Aug;543:18-23. PMID: 39163011
Idiopathic nephrotic syndrome and rituximab: may we predict circulating B lymphocytes recovery?
Delbet JD, Leclerc G, Ulinski T
Pediatr Nephrol 2019 Mar;34(3):529-532. Epub 2018 Dec 12 doi: 10.1007/s00467-018-4139-4. PMID: 30542932
Difficult-to-treat idiopathic nephrotic syndrome: established drugs, open questions and future options.
Kemper MJ, Valentin L, van Husen M
Pediatr Nephrol 2018 Oct;33(10):1641-1649. Epub 2017 Sep 6 doi: 10.1007/s00467-017-3780-7. PMID: 28879428
Childhood nephrotic syndrome in tropical Africa: then and now.
Olowu WA, Ademola A, Ajite AB, Saad YM
Paediatr Int Child Health 2017 Nov;37(4):259-268. Epub 2017 Sep 26 doi: 10.1080/20469047.2017.1374002. PMID: 28949280
Recurrence and Treatment after Renal Transplantation in Children with FSGS.
Kang HG, Ha IS, Cheong HI
Biomed Res Int 2016;2016:6832971. Epub 2016 Apr 24 doi: 10.1155/2016/6832971. PMID: 27213154Free PMC Article

Therapy

Renal and Urinary Conditions: Nephrotic Syndrome.
Abellada AMP
FP Essent 2024 Aug;543:18-23. PMID: 39163011
Idiopathic nephrotic syndrome and rituximab: may we predict circulating B lymphocytes recovery?
Delbet JD, Leclerc G, Ulinski T
Pediatr Nephrol 2019 Mar;34(3):529-532. Epub 2018 Dec 12 doi: 10.1007/s00467-018-4139-4. PMID: 30542932
Treating the idiopathic nephrotic syndrome: are steroids the answer?
Deschênes G, Dossier C, Hogan J
Pediatr Nephrol 2019 May;34(5):777-785. Epub 2018 Jun 4 doi: 10.1007/s00467-018-3963-x. PMID: 29869116
Difficult-to-treat idiopathic nephrotic syndrome: established drugs, open questions and future options.
Kemper MJ, Valentin L, van Husen M
Pediatr Nephrol 2018 Oct;33(10):1641-1649. Epub 2017 Sep 6 doi: 10.1007/s00467-017-3780-7. PMID: 28879428
The treatment of idiopathic focal segmental glomerulosclerosis in adults.
Hogan J, Radhakrishnan J
Adv Chronic Kidney Dis 2014 Sep;21(5):434-41. doi: 10.1053/j.ackd.2014.03.016. PMID: 25168833

Prognosis

Predictive Value of Serum Interleukins in Children with Idiopathic Nephrotic Syndrome.
Nickavar A, Valavi E, Safaeian B, Amoori P, Moosavian M
Iran J Allergy Asthma Immunol 2020 Dec 19;19(6):632-639. doi: 10.18502/ijaai.v19i6.4932. PMID: 33463132
Idiopathic nephrotic syndrome and rituximab: may we predict circulating B lymphocytes recovery?
Delbet JD, Leclerc G, Ulinski T
Pediatr Nephrol 2019 Mar;34(3):529-532. Epub 2018 Dec 12 doi: 10.1007/s00467-018-4139-4. PMID: 30542932
Difficult-to-treat idiopathic nephrotic syndrome: established drugs, open questions and future options.
Kemper MJ, Valentin L, van Husen M
Pediatr Nephrol 2018 Oct;33(10):1641-1649. Epub 2017 Sep 6 doi: 10.1007/s00467-017-3780-7. PMID: 28879428
Childhood nephrotic syndrome in tropical Africa: then and now.
Olowu WA, Ademola A, Ajite AB, Saad YM
Paediatr Int Child Health 2017 Nov;37(4):259-268. Epub 2017 Sep 26 doi: 10.1080/20469047.2017.1374002. PMID: 28949280
The treatment of idiopathic focal segmental glomerulosclerosis in adults.
Hogan J, Radhakrishnan J
Adv Chronic Kidney Dis 2014 Sep;21(5):434-41. doi: 10.1053/j.ackd.2014.03.016. PMID: 25168833

Clinical prediction guides

Predictive Value of Serum Interleukins in Children with Idiopathic Nephrotic Syndrome.
Nickavar A, Valavi E, Safaeian B, Amoori P, Moosavian M
Iran J Allergy Asthma Immunol 2020 Dec 19;19(6):632-639. doi: 10.18502/ijaai.v19i6.4932. PMID: 33463132
Idiopathic nephrotic syndrome and rituximab: may we predict circulating B lymphocytes recovery?
Delbet JD, Leclerc G, Ulinski T
Pediatr Nephrol 2019 Mar;34(3):529-532. Epub 2018 Dec 12 doi: 10.1007/s00467-018-4139-4. PMID: 30542932
Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management.
Bierzynska A, McCarthy HJ, Soderquest K, Sen ES, Colby E, Ding WY, Nabhan MM, Kerecuk L, Hegde S, Hughes D, Marks S, Feather S, Jones C, Webb NJ, Ognjanovic M, Christian M, Gilbert RD, Sinha MD, Lord GM, Simpson M, Koziell AB, Welsh GI, Saleem MA
Kidney Int 2017 Apr;91(4):937-947. Epub 2017 Jan 20 doi: 10.1016/j.kint.2016.10.013. PMID: 28117080
Initial steroid sensitivity in children with steroid-resistant nephrotic syndrome predicts post-transplant recurrence.
Ding WY, Koziell A, McCarthy HJ, Bierzynska A, Bhagavatula MK, Dudley JA, Inward CD, Coward RJ, Tizard J, Reid C, Antignac C, Boyer O, Saleem MA
J Am Soc Nephrol 2014 Jun;25(6):1342-8. Epub 2014 Feb 7 doi: 10.1681/ASN.2013080852. PMID: 24511128Free PMC Article
Saquinavir in steroid-dependent and -resistant nephrotic syndrome: a pilot study.
Coppo R, Camilla R, Porcellini MG, Peruzzi L, Gianoglio B, Amore A, Daprà V, Loiacono E, Fonsato V, Dal Canton A, Esposito C, Esposito P, Tovo PA
Nephrol Dial Transplant 2012 May;27(5):1902-10. Epub 2012 Mar 19 doi: 10.1093/ndt/gfs035. PMID: 22431705

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