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Idiopathic nephrotic syndrome(NPHS2)

MedGen UID:
501252
•Concept ID:
C3496337
•
Disease or Syndrome
Synonym: Nephrotic syndrome, idiopathic, steroid-resistant
 
Monarch Initiative: MONDO:0018170
OMIM®: 600995; 604766
Orphanet: ORPHA357502

Definition

A rare primary glomerular group of diseases characterized by the triad of edema, massive, or nephrotic-range, proteinuria and hypoalbuminemia, for which there is no known cause. Depending on response to treatment, disease is distinguished into steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS), with the latter being further divided, depending on occurrence, into familial or sporadic forms. [from ORDO]

Professional guidelines

PubMed

IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.
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
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.
Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer F, Hui NK, Boyer O, Saleem MA, Feltran L, Müller-Deile J, Becker JU, Cano F, Xu H, Lim YN, Smoyer W, Anochie I, Nakanishi K, Hodson E, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2020 Aug;35(8):1529-1561. Epub 2020 May 7 doi: 10.1007/s00467-020-04519-1. PMID: 32382828Free 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

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
Discovery of Autoantibodies Targeting Nephrin in Minimal Change Disease Supports a Novel Autoimmune Etiology.
Watts AJB, Keller KH, Lerner G, Rosales I, Collins AB, Sekulic M, Waikar SS, Chandraker A, Riella LV, Alexander MP, Troost JP, Chen J, Fermin D, Yee JL, Sampson MG, Beck LH Jr, Henderson JM, Greka A, Rennke HG, Weins A
J Am Soc Nephrol 2022 Jan;33(1):238-252. Epub 2021 Nov 3 doi: 10.1681/ASN.2021060794. PMID: 34732507Free PMC Article
Idiopathic nephrotic syndrome in children.
Noone DG, Iijima K, Parekh R
Lancet 2018 Jul 7;392(10141):61-74. Epub 2018 Jun 14 doi: 10.1016/S0140-6736(18)30536-1. PMID: 29910038
Primary Membranous Nephropathy.
Couser WG
Clin J Am Soc Nephrol 2017 Jun 7;12(6):983-997. Epub 2017 May 26 doi: 10.2215/CJN.11761116. PMID: 28550082Free PMC Article
Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome.
Ruggenenti P, Ruggiero B, Cravedi P, Vivarelli M, Massella L, Marasà M, Chianca A, Rubis N, Ene-Iordache B, Rudnicki M, Pollastro RM, Capasso G, Pisani A, Pennesi M, Emma F, Remuzzi G; Rituximab in Nephrotic Syndrome of Steroid-Dependent or Frequently Relapsing Minimal Change Disease Or Focal Segmental Glomerulosclerosis (NEMO) Study Group
J Am Soc Nephrol 2014 Apr;25(4):850-63. Epub 2014 Jan 30 doi: 10.1681/ASN.2013030251. PMID: 24480824Free PMC Article

Diagnosis

IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.
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
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.
Trautmann A, Vivarelli M, Samuel S, Gipson D, Sinha A, Schaefer F, Hui NK, Boyer O, Saleem MA, Feltran L, Müller-Deile J, Becker JU, Cano F, Xu H, Lim YN, Smoyer W, Anochie I, Nakanishi K, Hodson E, Haffner D; International Pediatric Nephrology Association
Pediatr Nephrol 2020 Aug;35(8):1529-1561. Epub 2020 May 7 doi: 10.1007/s00467-020-04519-1. PMID: 32382828Free 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
Primary Membranous Nephropathy.
Couser WG
Clin J Am Soc Nephrol 2017 Jun 7;12(6):983-997. Epub 2017 May 26 doi: 10.2215/CJN.11761116. PMID: 28550082Free PMC Article
Minimal Change Disease.
Vivarelli M, Massella L, Ruggiero B, Emma F
Clin J Am Soc Nephrol 2017 Feb 7;12(2):332-345. Epub 2016 Dec 9 doi: 10.2215/CJN.05000516. PMID: 27940460Free PMC Article

Therapy

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
Vaccines and Disease Relapses in Children with Nephrotic Syndrome.
Angeletti A, Bruschi M, Bianchin S, Bonato I, Montobbio C, Verrina E, Lugani F, Cravedi P, Ghiggeri GM
Clin J Am Soc Nephrol 2021 Jun;16(6):937-938. Epub 2021 Jun 11 doi: 10.2215/CJN.01890221. PMID: 34117084Free PMC Article
Idiopathic nephrotic syndrome in children.
Noone DG, Iijima K, Parekh R
Lancet 2018 Jul 7;392(10141):61-74. Epub 2018 Jun 14 doi: 10.1016/S0140-6736(18)30536-1. PMID: 29910038
Minimal Change Disease.
Vivarelli M, Massella L, Ruggiero B, Emma F
Clin J Am Soc Nephrol 2017 Feb 7;12(2):332-345. Epub 2016 Dec 9 doi: 10.2215/CJN.05000516. PMID: 27940460Free PMC Article
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
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.
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
Idiopathic nephrotic syndrome in children.
Noone DG, Iijima K, Parekh R
Lancet 2018 Jul 7;392(10141):61-74. Epub 2018 Jun 14 doi: 10.1016/S0140-6736(18)30536-1. PMID: 29910038
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

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
The Immune System and Idiopathic Nephrotic Syndrome.
Campbell RE, Thurman JM
Clin J Am Soc Nephrol 2022 Dec;17(12):1823-1834. Epub 2022 Oct 5 doi: 10.2215/CJN.07180622. PMID: 36198505Free PMC Article
Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome.
Ruggenenti P, Ruggiero B, Cravedi P, Vivarelli M, Massella L, Marasà M, Chianca A, Rubis N, Ene-Iordache B, Rudnicki M, Pollastro RM, Capasso G, Pisani A, Pennesi M, Emma F, Remuzzi G; Rituximab in Nephrotic Syndrome of Steroid-Dependent or Frequently Relapsing Minimal Change Disease Or Focal Segmental Glomerulosclerosis (NEMO) Study Group
J Am Soc Nephrol 2014 Apr;25(4):850-63. Epub 2014 Jan 30 doi: 10.1681/ASN.2013030251. PMID: 24480824Free PMC Article
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

Recent systematic reviews

IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome.
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
COVID-19 and idiopathic nephrotic syndrome in children: systematic review of the literature and recommendations from a highly affected area.
Morello W, Vianello FA, Proverbio E, Peruzzi L, Pasini A, Montini G
Pediatr Nephrol 2022 Apr;37(4):757-764. Epub 2021 Oct 23 doi: 10.1007/s00467-021-05330-2. PMID: 34687377Free PMC Article
Incidence and Relapse of Idiopathic Nephrotic Syndrome: Meta-analysis.
Veltkamp F, Rensma LR, Bouts AHM; LEARNS consortium
Pediatrics 2021 Jul;148(1) Epub 2021 Jun 30 doi: 10.1542/peds.2020-029249. PMID: 34193618
Interventions for idiopathic steroid-resistant nephrotic syndrome in children.
Liu ID, Willis NS, Craig JC, Hodson EM
Cochrane Database Syst Rev 2019 Nov 21;2019(11) doi: 10.1002/14651858.CD003594.pub6. PMID: 31749142Free PMC Article
Interventions for idiopathic steroid-resistant nephrotic syndrome in children.
Hodson EM, Wong SC, Willis NS, Craig JC
Cochrane Database Syst Rev 2016 Oct 11;10(10):CD003594. doi: 10.1002/14651858.CD003594.pub5. PMID: 27726125Free PMC Article

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