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Nephrotic syndrome 15(NPHS15)

MedGen UID:
1620414
•Concept ID:
C4539896
•
Disease or Syndrome
Synonyms: NEPHROTIC SYNDROME, TYPE 15; NPHS15
 
Gene (location): MAGI2 (7q21.11)
 
Monarch Initiative: MONDO:0033262
OMIM®: 617609

Definition

Nephrotic syndrome type 15 (NPHS15) is an autosomal recessive renal disorder characterized by onset of impaired kidney function with proteinuria in the first months of life. The disease course and severity varies widely. Some patients show rapid progression to end-stage renal failure necessitating transplant, whereas others have a more benign course that can be managed with medication. Renal biopsy tends to show glomerular sclerosis and effacement of podocyte foot processes (summary by Bierzynska et al., 2017). For a discussion of genetic heterogeneity of nephrotic syndrome and FSGS, see NPHS1 (256300). [from OMIM]

Clinical features

From HPO
Proteinuria
MedGen UID:
10976
•Concept ID:
C0033687
•
Finding
Increased levels of protein in the urine.
See: Feature record | Search on this feature
Steroid-resistant nephrotic syndrome
MedGen UID:
588369
•Concept ID:
C0403397
•
Disease or Syndrome
A form of nephrotic syndrome that does not respond to treatment with steroid medication, defined as persistent proteinuria despite 60mg/m2 or 2mg/kg for 8 weeks, after insuring no infection or non-adherence to medication.
See: Feature record | Search on this feature
Stage 5 chronic kidney disease
MedGen UID:
384526
•Concept ID:
C2316810
•
Disease or Syndrome
A degree of kidney failure severe enough to require dialysis or kidney transplantation for survival characterized by a severe reduction in glomerular filtration rate (less than 15 ml/min/1.73 m2) and other manifestations including increased serum creatinine.
See: Feature record | Search on this feature
Lipoid nephrosis
MedGen UID:
10307
•Concept ID:
C0027721
•
Disease or Syndrome
The presence of minimal changes visible by light microscopy but flattened and fused podocyte foot processes on electron microscopy in a person with nephrotic range proteinuria.
See: Feature record | Search on this feature
Hypoalbuminemia
MedGen UID:
68694
•Concept ID:
C0239981
•
Finding
Reduction in the concentration of albumin in the blood.
See: Feature record | Search on this feature
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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
Immunosuppressive treatment for primary membranous nephropathy in adults with nephrotic syndrome.
von Groote TC, Williams G, Au EH, Chen Y, Mathew AT, Hodson EM, Tunnicliffe DJ
Cochrane Database Syst Rev 2021 Nov 15;11(11):CD004293. doi: 10.1002/14651858.CD004293.pub4. PMID: 34778952Free PMC Article
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

Recent clinical studies

Etiology

Interventions for minimal change disease in adults with nephrotic syndrome.
Azukaitis K, Palmer SC, Strippoli GF, Hodson EM
Cochrane Database Syst Rev 2022 Mar 1;3(3):CD001537. doi: 10.1002/14651858.CD001537.pub5. PMID: 35230699Free PMC Article
Evaluation of Daily Low-Dose Prednisolone During Upper Respiratory Tract Infection to Prevent Relapse in Children With Relapsing Steroid-Sensitive Nephrotic Syndrome: The PREDNOS 2 Randomized Clinical Trial.
Christian MT, Webb NJA, Mehta S, Woolley RL, Afentou N, Frew E, Brettell EA, Khan AR, Milford DV, Bockenhauer D, Saleem MA, Hall AS, Koziell A, Maxwell H, Hegde S, Prajapati H, Gilbert RD, Jones C, McKeever K, Cook W, Ives N
JAMA Pediatr 2022 Mar 1;176(3):236-243. doi: 10.1001/jamapediatrics.2021.5189. PMID: 34928294Free PMC Article
Immunosuppressive treatment for primary membranous nephropathy in adults with nephrotic syndrome.
von Groote TC, Williams G, Au EH, Chen Y, Mathew AT, Hodson EM, Tunnicliffe DJ
Cochrane Database Syst Rev 2021 Nov 15;11(11):CD004293. doi: 10.1002/14651858.CD004293.pub4. PMID: 34778952Free 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
The incidence of the nephrotic syndrome in childhood in Germany.
Franke I, Aydin M, Llamas Lopez CE, Kurylowicz L, Ganschow R, Lentze M, Born M
Clin Exp Nephrol 2018 Feb;22(1):126-132. Epub 2017 Jun 22 doi: 10.1007/s10157-017-1433-6. PMID: 28643120

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
Evaluation of Daily Low-Dose Prednisolone During Upper Respiratory Tract Infection to Prevent Relapse in Children With Relapsing Steroid-Sensitive Nephrotic Syndrome: The PREDNOS 2 Randomized Clinical Trial.
Christian MT, Webb NJA, Mehta S, Woolley RL, Afentou N, Frew E, Brettell EA, Khan AR, Milford DV, Bockenhauer D, Saleem MA, Hall AS, Koziell A, Maxwell H, Hegde S, Prajapati H, Gilbert RD, Jones C, McKeever K, Cook W, Ives N
JAMA Pediatr 2022 Mar 1;176(3):236-243. doi: 10.1001/jamapediatrics.2021.5189. PMID: 34928294Free PMC Article
The incidence of the nephrotic syndrome in childhood in Germany.
Franke I, Aydin M, Llamas Lopez CE, Kurylowicz L, Ganschow R, Lentze M, Born M
Clin Exp Nephrol 2018 Feb;22(1):126-132. Epub 2017 Jun 22 doi: 10.1007/s10157-017-1433-6. PMID: 28643120
Hypothyroidism and Nephrotic Syndrome: Why, When and How to Treat.
Mario FD, Pofi R, Gigante A, Rivoli L, Rosato E, Isidori AM, Cianci R, Barbano B
Curr Vasc Pharmacol 2017;15(5):398-403. doi: 10.2174/1570161115999170207114706. PMID: 28176633
Nephrotic syndrome.
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763

Therapy

EditorialAssignment.
Xu Y, Yang Q, Fu C, Han E, Gao Y
BMJ Open 2023 Jan 18;13(1):e064220. doi: 10.1136/bmjopen-2022-064220. PMID: 36657752Free 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
Hypothyroidism and Nephrotic Syndrome: Why, When and How to Treat.
Mario FD, Pofi R, Gigante A, Rivoli L, Rosato E, Isidori AM, Cianci R, Barbano B
Curr Vasc Pharmacol 2017;15(5):398-403. doi: 10.2174/1570161115999170207114706. PMID: 28176633
Nephrotic syndrome.
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763

Prognosis

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.
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763
Idiopathic membranous nephropathy: outline and rationale of a treatment strategy.
du Buf-Vereijken PW, Branten AJ, Wetzels JF
Am J Kidney Dis 2005 Dec;46(6):1012-29. doi: 10.1053/j.ajkd.2005.08.020. PMID: 16310567
Denys-Drash syndrome.
Lin HC, Lin SK, Wen MC, Tseng CF, Fu LS, Chi CS
J Formos Med Assoc 2004 Jan;103(1):71-4. PMID: 15026863

Clinical prediction guides

Association between hypothyroidism and nephrotic syndrome: a bidirectional two-sample Mendelian randomization analysis.
Liu C, Shen J, Ding Z, Duan S, Dai E
Ren Fail 2024 Dec;46(2):2390558. Epub 2024 Aug 14 doi: 10.1080/0886022X.2024.2390558. PMID: 39143823Free PMC Article
Bevacizumab-associated glomerular microangiopathy.
Person F, Rinschen MM, Brix SR, Wulf S, Noriega MLM, Fehrle W, Schmitz J, Schwarz A, Ivanyi P, Steinmetz OM, Reinhard L, Hoxha E, Zipfel PF, Bräsen JH, Wiech T
Mod Pathol 2019 May;32(5):684-700. Epub 2018 Dec 14 doi: 10.1038/s41379-018-0186-4. PMID: 30552416
Nephrotic syndrome.
Andolino TP, Reid-Adam J
Pediatr Rev 2015 Mar;36(3):117-25; quiz 126, 129. doi: 10.1542/pir.36-3-117. PMID: 25733763
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
Idiopathic membranous nephropathy: outline and rationale of a treatment strategy.
du Buf-Vereijken PW, Branten AJ, Wetzels JF
Am J Kidney Dis 2005 Dec;46(6):1012-29. doi: 10.1053/j.ajkd.2005.08.020. PMID: 16310567

Recent systematic reviews

Interventions for preventing and treating kidney disease in IgA vasculitis.
Hahn D, Hodson EM, Craig JC
Cochrane Database Syst Rev 2023 Feb 28;2(2):CD005128. doi: 10.1002/14651858.CD005128.pub4. PMID: 36853224Free 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
Interventions for focal segmental glomerulosclerosis in adults.
Hodson EM, Sinha A, Cooper TE
Cochrane Database Syst Rev 2022 Feb 28;2(2):CD003233. doi: 10.1002/14651858.CD003233.pub3. PMID: 35224732Free PMC Article
Immunosuppressive treatment for primary membranous nephropathy in adults with nephrotic syndrome.
von Groote TC, Williams G, Au EH, Chen Y, Mathew AT, Hodson EM, Tunnicliffe DJ
Cochrane Database Syst Rev 2021 Nov 15;11(11):CD004293. doi: 10.1002/14651858.CD004293.pub4. PMID: 34778952Free PMC Article
Rituximab therapy for focal segmental glomerulosclerosis and minimal change disease in adults: a systematic review and meta-analysis.
Hansrivijit P, Cheungpasitporn W, Thongprayoon C, Ghahramani N
BMC Nephrol 2020 Apr 15;21(1):134. doi: 10.1186/s12882-020-01797-7. PMID: 32293308Free PMC Article

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