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Nephrotic range proteinuria

MedGen UID:
619713
•Concept ID:
C0445118
•
Disease or Syndrome; Finding
Synonym: Nephrotic Range Proteinuria
SNOMED CT: Nephrotic range proteinuria (264867001)
 
HPO: HP:0012593

Definition

Severely increased amount of excretion of protein in the urine, defined as 3.5 grams per day or more in adults and 40 mg per meter-squared body surface area per hour in children. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Nephrotic range proteinuria

Conditions with this feature

Immunodeficiency, common variable, 6
MedGen UID:
462091
•Concept ID:
C3150741
•
Disease or Syndrome
Any common variable immunodeficiency in which the cause of the disease is a mutation in the CD81 gene.
See: Condition Record
Nephrotic syndrome, type 22
MedGen UID:
1745920
•Concept ID:
C5436909
•
Disease or Syndrome
Nephrotic syndrome type 22 (NPHS22) is an autosomal recessive renal disease characterized by onset of progressive kidney dysfunction in infancy. Affected individuals usually present with edema associated with hypoproteinemia, proteinuria, and microscopic hematuria. Renal biopsy shows effacement of the podocyte foot processes, glomerulosclerosis, and thickening of the glomerular basement membrane. The disease is steroid-resistant and progressive, resulting in end-stage renal disease usually necessitating kidney transplant (Majmundar et al., 2021). For a general phenotypic description and a discussion of genetic heterogeneity of nephrotic syndrome, see NPHS1 (256300).
See: Condition Record
Immunodeficiency, common variable, 6
Nephrotic syndrome, type 22

Professional guidelines

PubMed

Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.
Boyer O, Schaefer F, Haffner D, Bockenhauer D, Hölttä T, Bérody S, Webb H, Heselden M, Lipska-Zie Tkiewicz BS, Ozaltin F, Levtchenko E, Vivarelli M
Nat Rev Nephrol 2021 Apr;17(4):277-289. Epub 2021 Jan 29 doi: 10.1038/s41581-020-00384-1. PMID: 33514942Free PMC Article
2019 Update of the Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis.
Fanouriakis A, Kostopoulou M, Cheema K, Anders HJ, Aringer M, Bajema I, Boletis J, Frangou E, Houssiau FA, Hollis J, Karras A, Marchiori F, Marks SD, Moroni G, Mosca M, Parodis I, Praga M, Schneider M, Smolen JS, Tesar V, Trachana M, van Vollenhoven RF, Voskuyl AE, Teng YKO, van Leew B, Bertsias G, Jayne D, Boumpas DT
Ann Rheum Dis 2020 Jun;79(6):713-723. Epub 2020 Mar 27 doi: 10.1136/annrheumdis-2020-216924. PMID: 32220834
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

Diagnostic and Prognostic Comparison of Immune-Complex-Mediated Membranoproliferative Glomerulonephritis and C3 Glomerulopathy.
Kovala M, Seppälä M, Räisänen-Sokolowski A, Meri S, Honkanen E, Kaartinen K
Cells 2023 Feb 23;12(5) doi: 10.3390/cells12050712. PMID: 36899849Free PMC Article
Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.
Boyer O, Schaefer F, Haffner D, Bockenhauer D, Hölttä T, Bérody S, Webb H, Heselden M, Lipska-Zie Tkiewicz BS, Ozaltin F, Levtchenko E, Vivarelli M
Nat Rev Nephrol 2021 Apr;17(4):277-289. Epub 2021 Jan 29 doi: 10.1038/s41581-020-00384-1. PMID: 33514942Free PMC Article
Dysproteinemia and the Kidney: Core Curriculum 2019.
Hogan JJ, Alexander MP, Leung N
Am J Kidney Dis 2019 Dec;74(6):822-836. Epub 2019 Jul 19 doi: 10.1053/j.ajkd.2019.04.029. PMID: 31331759
Collapsing glomerulopathy: update.
Morales E, Alonso M, Gutiérrez E
Med Clin (Barc) 2019 May 3;152(9):361-367. Epub 2018 Dec 13 doi: 10.1016/j.medcli.2018.10.021. PMID: 30554809
Transplant glomerulopathy.
Filippone EJ, McCue PA, Farber JL
Mod Pathol 2018 Feb;31(2):235-252. Epub 2017 Oct 13 doi: 10.1038/modpathol.2017.123. PMID: 29027535

Diagnosis

Membranous nephropathy: new pathogenic mechanisms and their clinical implications.
Hoxha E, Reinhard L, Stahl RAK
Nat Rev Nephrol 2022 Jul;18(7):466-478. Epub 2022 Apr 28 doi: 10.1038/s41581-022-00564-1. PMID: 35484394
Lupus Podocytopathy: An Overview.
Oliva-Damaso N, Payan J, Oliva-Damaso E, Pereda T, Bomback AS
Adv Chronic Kidney Dis 2019 Sep;26(5):369-375. doi: 10.1053/j.ackd.2019.08.011. PMID: 31733721Free PMC Article
Dysproteinemia and the Kidney: Core Curriculum 2019.
Hogan JJ, Alexander MP, Leung N
Am J Kidney Dis 2019 Dec;74(6):822-836. Epub 2019 Jul 19 doi: 10.1053/j.ajkd.2019.04.029. PMID: 31331759
Collapsing glomerulopathy: update.
Morales E, Alonso M, Gutiérrez E
Med Clin (Barc) 2019 May 3;152(9):361-367. Epub 2018 Dec 13 doi: 10.1016/j.medcli.2018.10.021. PMID: 30554809
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

Therapy

Management of congenital nephrotic syndrome: consensus recommendations of the ERKNet-ESPN Working Group.
Boyer O, Schaefer F, Haffner D, Bockenhauer D, Hölttä T, Bérody S, Webb H, Heselden M, Lipska-Zie Tkiewicz BS, Ozaltin F, Levtchenko E, Vivarelli M
Nat Rev Nephrol 2021 Apr;17(4):277-289. Epub 2021 Jan 29 doi: 10.1038/s41581-020-00384-1. PMID: 33514942Free PMC Article
2019 Update of the Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis.
Fanouriakis A, Kostopoulou M, Cheema K, Anders HJ, Aringer M, Bajema I, Boletis J, Frangou E, Houssiau FA, Hollis J, Karras A, Marchiori F, Marks SD, Moroni G, Mosca M, Parodis I, Praga M, Schneider M, Smolen JS, Tesar V, Trachana M, van Vollenhoven RF, Voskuyl AE, Teng YKO, van Leew B, Bertsias G, Jayne D, Boumpas DT
Ann Rheum Dis 2020 Jun;79(6):713-723. Epub 2020 Mar 27 doi: 10.1136/annrheumdis-2020-216924. PMID: 32220834
Dysproteinemia and the Kidney: Core Curriculum 2019.
Hogan JJ, Alexander MP, Leung N
Am J Kidney Dis 2019 Dec;74(6):822-836. Epub 2019 Jul 19 doi: 10.1053/j.ajkd.2019.04.029. PMID: 31331759
Sudden Onset Nephrotic-Range Proteinuria.
Fromonot J, Marlinge M, Petit C, Burtey S, Guieu R
Clin Chem 2019 Apr;65(4):600-601. doi: 10.1373/clinchem.2018.295519. PMID: 30923065
Transplant glomerulopathy.
Filippone EJ, McCue PA, Farber JL
Mod Pathol 2018 Feb;31(2):235-252. Epub 2017 Oct 13 doi: 10.1038/modpathol.2017.123. PMID: 29027535

Prognosis

Membranous nephropathy: new pathogenic mechanisms and their clinical implications.
Hoxha E, Reinhard L, Stahl RAK
Nat Rev Nephrol 2022 Jul;18(7):466-478. Epub 2022 Apr 28 doi: 10.1038/s41581-022-00564-1. PMID: 35484394
2019 Update of the Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis.
Fanouriakis A, Kostopoulou M, Cheema K, Anders HJ, Aringer M, Bajema I, Boletis J, Frangou E, Houssiau FA, Hollis J, Karras A, Marchiori F, Marks SD, Moroni G, Mosca M, Parodis I, Praga M, Schneider M, Smolen JS, Tesar V, Trachana M, van Vollenhoven RF, Voskuyl AE, Teng YKO, van Leew B, Bertsias G, Jayne D, Boumpas DT
Ann Rheum Dis 2020 Jun;79(6):713-723. Epub 2020 Mar 27 doi: 10.1136/annrheumdis-2020-216924. PMID: 32220834
Lupus Podocytopathy: An Overview.
Oliva-Damaso N, Payan J, Oliva-Damaso E, Pereda T, Bomback AS
Adv Chronic Kidney Dis 2019 Sep;26(5):369-375. doi: 10.1053/j.ackd.2019.08.011. PMID: 31733721Free PMC Article
Collapsing glomerulopathy: update.
Morales E, Alonso M, Gutiérrez E
Med Clin (Barc) 2019 May 3;152(9):361-367. Epub 2018 Dec 13 doi: 10.1016/j.medcli.2018.10.021. PMID: 30554809
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

Spectrum of renal disease in scleroderma other than scleroderma renal crisis: A review of the literature.
Farrukh L, Steen V, Shapiro L, Faddoul G, Mehta S
Clin Nephrol 2024 Aug;102(2):97-106. doi: 10.5414/CN111243. PMID: 38699985
Nephrotic-range proteinuria: a potential risk factor for failure of tixagevimab-cilgavimab prophylaxis.
Al Jurdi A, El Mouhayyar C, Efe O, Jeyabalan A, Riella LV
J Nephrol 2024 Jan;37(1):141-147. Epub 2023 Sep 2 doi: 10.1007/s40620-023-01750-5. PMID: 37658973
Diagnostic and Prognostic Comparison of Immune-Complex-Mediated Membranoproliferative Glomerulonephritis and C3 Glomerulopathy.
Kovala M, Seppälä M, Räisänen-Sokolowski A, Meri S, Honkanen E, Kaartinen K
Cells 2023 Feb 23;12(5) doi: 10.3390/cells12050712. PMID: 36899849Free PMC Article
Nephrotic Range Proteinuria in Renal Transplantation: Clinical and Histologic Correlates in a 10-year Retrospective Study.
Leal R, Pinto H, Galvão A, Santos L, Romãozinho C, Macário F, Alves R, Pratas J, Sousa V, Marinho C, Prado E Castro L, Campos M, Mota A, Figueiredo A
Transplant Proc 2017 May;49(4):792-794. doi: 10.1016/j.transproceed.2017.01.066. PMID: 28457396
Predictors of HIV-associated nephropathy.
Waheed S, Atta MG
Expert Rev Anti Infect Ther 2014 May;12(5):555-63. Epub 2014 Mar 21 doi: 10.1586/14787210.2014.901170. PMID: 24655211

Recent systematic reviews

Management of lupus nephritis: a systematic literature review informing the 2019 update of the joint EULAR and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations.
Kostopoulou M, Fanouriakis A, Cheema K, Boletis J, Bertsias G, Jayne D, Boumpas DT
RMD Open 2020 Jul;6(2) doi: 10.1136/rmdopen-2020-001263. PMID: 32699043Free PMC Article
Treatment Strategies of Adult Primary Focal Segmental Glomerulosclerosis: A Systematic Review Focusing on the Last Two Decades.
Beer A, Mayer G, Kronbichler A
Biomed Res Int 2016;2016:4192578. Epub 2016 Apr 7 doi: 10.1155/2016/4192578. PMID: 27144166Free PMC Article
Dengue-induced Acute Kidney Injury (DAKI): A Neglected and Fatal Complication of Dengue Viral Infection--A Systematic Review.
Mallhi TH, Sarriff A, Adnan AS, Khan YH, Hamzah AA, Jummaat F, Khan AH
J Coll Physicians Surg Pak 2015 Nov;25(11):828-34. PMID: 26577971
Collapsing glomerulopathy in collagen vascular-like disease.
Nasr R, Johns C, Gertner E
Lupus 2014;23(1):75-80. Epub 2013 Nov 27 doi: 10.1177/0961203313513509. PMID: 24285096
Mercury-associated nephrotic syndrome: a case report and systematic review of the literature.
Miller S, Pallan S, Gangji AS, Lukic D, Clase CM
Am J Kidney Dis 2013 Jul;62(1):135-8. Epub 2013 Apr 18 doi: 10.1053/j.ajkd.2013.02.372. PMID: 23602193

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