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Proteinuria, chronic benign(PROCHOB)

MedGen UID:
1714078
•Concept ID:
C5394384
•
Finding
Synonyms: PROCHOB; PROTEINURIA, CHRONIC BENIGN
 
Gene (location): CUBN (10p13)
 
Monarch Initiative: MONDO:0030042
OMIM®: 618884

Definition

Chronic benign proteinuria (PROCHOB) is an autosomal recessive condition characterized by onset of isolated proteinuria in the first decade of life. The proteinuria is nonprogressive; affected individuals do not develop renal disease or impaired kidney function, and they do not have additional associated abnormalities, such as hypertension. The correct diagnosis is important to avoid inefficient or invasive intervention, such as medication or renal biopsy (summary by Bedin et al., 2020). [from OMIM]

Clinical features

From HPO
Albuminuria
MedGen UID:
1394
•Concept ID:
C0001925
•
Finding
Increased concentration of albumin in the urine.
See: Feature record | Search on this feature
Proteinuria
MedGen UID:
10976
•Concept ID:
C0033687
•
Finding
Increased levels of protein in the urine.
See: Feature record | Search on this feature
Renal insufficiency
MedGen UID:
332529
•Concept ID:
C1565489
•
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
See: Feature record | Search on this feature

Professional guidelines

PubMed

Proteinuria in Children: Evaluation and Differential Diagnosis.
Leung AK, Wong AH, Barg SS
Am Fam Physician 2017 Feb 15;95(4):248-254. PMID: 28290633
Treatment of steroid sensitive nephrotic syndrome.
Abeyagunawardena AS
Indian J Pediatr 2005 Sep;72(9):763-9. doi: 10.1007/BF02734149. PMID: 16186679
Management of acute renal failure in the elderly. Treatment options.
Mandal AK, Baig M, Koutoubi Z
Drugs Aging 1996 Oct;9(4):226-50. doi: 10.2165/00002512-199609040-00002. PMID: 8894522

Recent clinical studies

Etiology

My journey on the path to understanding IgA nephropathy: From bench to bedside.
Tomino Y
Nephrology (Carlton) 2024 Sep;29 Suppl 2:51-54. doi: 10.1111/nep.14346. PMID: 39327760
Alport syndrome and Alport kidney diseases - elucidating the disease spectrum.
Puapatanakul P, Miner JH
Curr Opin Nephrol Hypertens 2024 May 1;33(3):283-290. Epub 2024 Mar 13 doi: 10.1097/MNH.0000000000000983. PMID: 38477333Free PMC Article
IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects.
Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B
Autoimmun Rev 2015 Jul;14(7):579-85. Epub 2015 Feb 14 doi: 10.1016/j.autrev.2015.02.003. PMID: 25688001
Safety of statins.
Brown WV
Curr Opin Lipidol 2008 Dec;19(6):558-62. doi: 10.1097/MOL.0b013e328319baba. PMID: 18957877
Short-term prognosis of severe proliferative lupus nephritis.
Kasinath BS, Neilson EG, Hebert L, Schwartz MM, Lewis EJ
Am J Kidney Dis 1986 Oct;8(4):239-43. doi: 10.1016/s0272-6386(86)80032-4. PMID: 3766530

Diagnosis

My journey on the path to understanding IgA nephropathy: From bench to bedside.
Tomino Y
Nephrology (Carlton) 2024 Sep;29 Suppl 2:51-54. doi: 10.1111/nep.14346. PMID: 39327760
Alport syndrome and Alport kidney diseases - elucidating the disease spectrum.
Puapatanakul P, Miner JH
Curr Opin Nephrol Hypertens 2024 May 1;33(3):283-290. Epub 2024 Mar 13 doi: 10.1097/MNH.0000000000000983. PMID: 38477333Free PMC Article
Human C-terminal CUBN variants associate with chronic proteinuria and normal renal function.
Bedin M, Boyer O, Servais A, Li Y, Villoing-Gaudé L, Tête MJ, Cambier A, Hogan J, Baudouin V, Krid S, Bensman A, Lammens F, Louillet F, Ranchin B, Vigneau C, Bouteau I, Isnard-Bagnis C, Mache CJ, Schäfer T, Pape L, Gödel M, Huber TB, Benz M, Klaus G, Hansen M, Latta K, Gribouval O, Morinière V, Tournant C, Grohmann M, Kuhn E, Wagner T, Bole-Feysot C, Jabot-Hanin F, Nitschké P, Ahluwalia TS, Köttgen A, Andersen CBF, Bergmann C, Antignac C, Simons M
J Clin Invest 2020 Jan 2;130(1):335-344. doi: 10.1172/JCI129937. PMID: 31613795Free PMC Article
IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects.
Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B
Autoimmun Rev 2015 Jul;14(7):579-85. Epub 2015 Feb 14 doi: 10.1016/j.autrev.2015.02.003. PMID: 25688001
Short-term prognosis of severe proliferative lupus nephritis.
Kasinath BS, Neilson EG, Hebert L, Schwartz MM, Lewis EJ
Am J Kidney Dis 1986 Oct;8(4):239-43. doi: 10.1016/s0272-6386(86)80032-4. PMID: 3766530

Therapy

Human C-terminal CUBN variants associate with chronic proteinuria and normal renal function.
Bedin M, Boyer O, Servais A, Li Y, Villoing-Gaudé L, Tête MJ, Cambier A, Hogan J, Baudouin V, Krid S, Bensman A, Lammens F, Louillet F, Ranchin B, Vigneau C, Bouteau I, Isnard-Bagnis C, Mache CJ, Schäfer T, Pape L, Gödel M, Huber TB, Benz M, Klaus G, Hansen M, Latta K, Gribouval O, Morinière V, Tournant C, Grohmann M, Kuhn E, Wagner T, Bole-Feysot C, Jabot-Hanin F, Nitschké P, Ahluwalia TS, Köttgen A, Andersen CBF, Bergmann C, Antignac C, Simons M
J Clin Invest 2020 Jan 2;130(1):335-344. doi: 10.1172/JCI129937. PMID: 31613795Free PMC Article
Metabolic phenotypes of obese, overweight, and normal weight individuals and risk of chronic kidney disease: a systematic review and meta-analysis.
Alizadeh S, Esmaeili H, Alizadeh M, Daneshzad E, Sharifi L, Radfar H, Radaei MK
Arch Endocrinol Metab 2019 Jul 29;63(4):427-437. doi: 10.20945/2359-3997000000149. PMID: 31365625Free PMC Article
IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects.
Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B
Autoimmun Rev 2015 Jul;14(7):579-85. Epub 2015 Feb 14 doi: 10.1016/j.autrev.2015.02.003. PMID: 25688001
Safety of statins.
Brown WV
Curr Opin Lipidol 2008 Dec;19(6):558-62. doi: 10.1097/MOL.0b013e328319baba. PMID: 18957877
Systemic and glomerular hypertension and progression of chronic renal disease: the dilemma of nephrosclerosis.
Marín R, Gorostidi M, Fernández-Vega F, Alvarez-Navascués R
Kidney Int Suppl 2005 Dec;(99):S52-6. doi: 10.1111/j.1523-1755.2005.09910.x. PMID: 16336577

Prognosis

Time-averaged proteinuria during follow-up and renal prognosis in patients with biopsy-proven benign nephrosclerosis.
Amano H, Koike K, Haruhara K, Tsuboi N, Ogura M, Yokoo T
Clin Exp Nephrol 2020 Aug;24(8):688-695. Epub 2020 Apr 9 doi: 10.1007/s10157-020-01885-w. PMID: 32274596
IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects.
Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B
Autoimmun Rev 2015 Jul;14(7):579-85. Epub 2015 Feb 14 doi: 10.1016/j.autrev.2015.02.003. PMID: 25688001
Long-term renal outcome of snake bite and acute kidney injury: a single-center experience.
Waikhom R, Sircar D, Patil K, Bennikal M, Gupta SD, Pandey R
Ren Fail 2012;34(3):271-4. Epub 2012 Jan 20 doi: 10.3109/0886022X.2011.647297. PMID: 22260154
Systemic and glomerular hypertension and progression of chronic renal disease: the dilemma of nephrosclerosis.
Marín R, Gorostidi M, Fernández-Vega F, Alvarez-Navascués R
Kidney Int Suppl 2005 Dec;(99):S52-6. doi: 10.1111/j.1523-1755.2005.09910.x. PMID: 16336577
Short-term prognosis of severe proliferative lupus nephritis.
Kasinath BS, Neilson EG, Hebert L, Schwartz MM, Lewis EJ
Am J Kidney Dis 1986 Oct;8(4):239-43. doi: 10.1016/s0272-6386(86)80032-4. PMID: 3766530

Clinical prediction guides

Time-averaged proteinuria during follow-up and renal prognosis in patients with biopsy-proven benign nephrosclerosis.
Amano H, Koike K, Haruhara K, Tsuboi N, Ogura M, Yokoo T
Clin Exp Nephrol 2020 Aug;24(8):688-695. Epub 2020 Apr 9 doi: 10.1007/s10157-020-01885-w. PMID: 32274596
Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review.
Naik RP, Smith-Whitley K, Hassell KL, Umeh NI, de Montalembert M, Sahota P, Haywood C Jr, Jenkins J, Lloyd-Puryear MA, Joiner CH, Bonham VL, Kato GJ
Ann Intern Med 2018 Nov 6;169(9):619-627. Epub 2018 Oct 30 doi: 10.7326/M18-1161. PMID: 30383109Free PMC Article
Prevalence of Proteinuria and Other Abnormalities in Urinalysis Performed in the Urology Clinic.
Bezinque A, Noyes SL, Kirmiz S, Parker J, Dey S, Kahnoski RJ, Lane BR
Urology 2017 May;103:34-38. Epub 2017 Feb 14 doi: 10.1016/j.urology.2017.02.011. PMID: 28212852
Sickle cell disease and the kidney.
Scheinman JI
Nat Clin Pract Nephrol 2009 Feb;5(2):78-88. Epub 2008 Dec 2 doi: 10.1038/ncpneph1008. PMID: 19048000
Safety of statins.
Brown WV
Curr Opin Lipidol 2008 Dec;19(6):558-62. doi: 10.1097/MOL.0b013e328319baba. PMID: 18957877

Recent systematic reviews

Metabolic phenotypes of obese, overweight, and normal weight individuals and risk of chronic kidney disease: a systematic review and meta-analysis.
Alizadeh S, Esmaeili H, Alizadeh M, Daneshzad E, Sharifi L, Radfar H, Radaei MK
Arch Endocrinol Metab 2019 Jul 29;63(4):427-437. doi: 10.20945/2359-3997000000149. PMID: 31365625Free PMC Article
Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review.
Naik RP, Smith-Whitley K, Hassell KL, Umeh NI, de Montalembert M, Sahota P, Haywood C Jr, Jenkins J, Lloyd-Puryear MA, Joiner CH, Bonham VL, Kato GJ
Ann Intern Med 2018 Nov 6;169(9):619-627. Epub 2018 Oct 30 doi: 10.7326/M18-1161. PMID: 30383109Free PMC Article

Supplemental Content

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    • ClinVar
      Related medical variations
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      Related information in NCBI Gene
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      Related information in GTR
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      Clinical tests in GTR
    • OMIM
      Related records in OMIM
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
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