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Primary membranoproliferative glomerulonephritis

MedGen UID:
988496
Concept ID:
CN304703
Disease or Syndrome
Synonyms: membranoproliferative glomerulonephritis; Mesangiocapillary glomerulonephritis; MPGN; primary membranoproliferative glomerulonephritis; Primary MPGN
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018904
Orphanet: ORPHA54370

Definition

A rare glomerular disease characterized by a pattern of glomerular injury on kidney biopsy with characteristic light microscopic changes: mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (GBM). On the basis of immunofluorescence (IF) the disorder is divided into C3 glomerulopathy (C3G) or immunoglobulin-mediated membranoproliferative glomerulonephritis. Through electron microscopy C3G is further divided into Dense deposit disease, with highly electrondense deposits in the glomerular basement membrane, and C3 glomerulonephritis, with mesangial, intramembranous, subendothelial and subepithelial deposits. Secondary causes (autoimmune, infectious, malignancies) are excluded. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary membranoproliferative glomerulonephritis

Professional guidelines

PubMed

Nakagawa N, Kimura T, Sakate R, Isaka Y, Narita I
Clin Exp Nephrol 2023 Nov;27(11):928-935. Epub 2023 Jul 29 doi: 10.1007/s10157-023-02387-1. PMID: 37515698Free PMC Article
Wei CC, Wang W, Smoyer WE, Licht C
Pediatr Nephrol 2012 Dec;27(12):2243-50. Epub 2012 Jul 18 doi: 10.1007/s00467-012-2249-y. PMID: 22802081

Recent clinical studies

Etiology

Honma S, Sato N, Sakaguchi R, Hashiguchi A, Uesugi N, Nakamura Y, Sasano H, Joh K
Med Mol Morphol 2024 Mar;57(1):23-34. Epub 2023 Oct 12 doi: 10.1007/s00795-023-00370-z. PMID: 37823929
Nakagawa N, Kimura T, Sakate R, Isaka Y, Narita I
Clin Exp Nephrol 2023 Nov;27(11):928-935. Epub 2023 Jul 29 doi: 10.1007/s10157-023-02387-1. PMID: 37515698Free PMC Article
Agrebi I, Kammoun K, Dammak N, Hachicha J, Boudawara T, Jarraya F, Hmida MB
Pan Afr Med J 2021;38:218. Epub 2021 Feb 25 doi: 10.11604/pamj.2021.38.218.21059. PMID: 34046124Free PMC Article
Ruggenenti P, Daina E, Gennarini A, Carrara C, Gamba S, Noris M, Rubis N, Peraro F, Gaspari F, Pasini A, Rigotti A, Lerchner RM, Santoro D, Pisani A, Pasi A, Remuzzi G; EAGLE Study Group
Am J Kidney Dis 2019 Aug;74(2):224-238. Epub 2019 Mar 28 doi: 10.1053/j.ajkd.2018.12.046. PMID: 30929851
Wei CC, Wang W, Smoyer WE, Licht C
Pediatr Nephrol 2012 Dec;27(12):2243-50. Epub 2012 Jul 18 doi: 10.1007/s00467-012-2249-y. PMID: 22802081

Diagnosis

Honma S, Sato N, Sakaguchi R, Hashiguchi A, Uesugi N, Nakamura Y, Sasano H, Joh K
Med Mol Morphol 2024 Mar;57(1):23-34. Epub 2023 Oct 12 doi: 10.1007/s00795-023-00370-z. PMID: 37823929
Wu Q, Liang D, Song T, Ni X, Wu X, Zhang M, Chen J, Cheng D, Xie K, Li X, Wen J
J Nephrol 2023 Jan;36(1):93-101. Epub 2022 Jul 22 doi: 10.1007/s40620-022-01385-y. PMID: 35867238
Agrebi I, Kammoun K, Dammak N, Hachicha J, Boudawara T, Jarraya F, Hmida MB
Pan Afr Med J 2021;38:218. Epub 2021 Feb 25 doi: 10.11604/pamj.2021.38.218.21059. PMID: 34046124Free PMC Article
Mizuno M, Suzuki Y, Ito Y
Clin Exp Nephrol 2018 Feb;22(1):3-14. Epub 2017 Mar 24 doi: 10.1007/s10157-017-1405-x. PMID: 28341889
Kawamura T, Usui J, Kaseda K, Takada K, Ebihara I, Ishizu T, Iitsuka T, Sakai K, Takemura K, Kobayashi M, Koyama A, Kanemoto K, Sumazaki R, Uesugi N, Noguchi M, Nagata M, Suka M, Yamagata K
Clin Exp Nephrol 2013 Apr;17(2):248-54. Epub 2012 Sep 13 doi: 10.1007/s10157-012-0690-7. PMID: 22971963

Therapy

Villa G, Daina E, Brambilla P, Gamba S, Leone VF, Carrara C, Rizzo P, Noris M, Remuzzi G, Remuzzi A, Caroli A
Nephron 2024;148(6):367-378. Epub 2023 Nov 3 doi: 10.1159/000534893. PMID: 37926085Free PMC Article
Nakagawa N, Kimura T, Sakate R, Isaka Y, Narita I
Clin Exp Nephrol 2023 Nov;27(11):928-935. Epub 2023 Jul 29 doi: 10.1007/s10157-023-02387-1. PMID: 37515698Free PMC Article
Wu Q, Liang D, Song T, Ni X, Wu X, Zhang M, Chen J, Cheng D, Xie K, Li X, Wen J
J Nephrol 2023 Jan;36(1):93-101. Epub 2022 Jul 22 doi: 10.1007/s40620-022-01385-y. PMID: 35867238
Ruggenenti P, Daina E, Gennarini A, Carrara C, Gamba S, Noris M, Rubis N, Peraro F, Gaspari F, Pasini A, Rigotti A, Lerchner RM, Santoro D, Pisani A, Pasi A, Remuzzi G; EAGLE Study Group
Am J Kidney Dis 2019 Aug;74(2):224-238. Epub 2019 Mar 28 doi: 10.1053/j.ajkd.2018.12.046. PMID: 30929851
Harmankaya O, Baştürk T, Oztürk Y, Karabiber N, Obek A
Int Urol Nephrol 2001;33(3):583-7. doi: 10.1023/a:1019546617485. PMID: 12230299

Prognosis

Wu Q, Liang D, Song T, Ni X, Wu X, Zhang M, Chen J, Cheng D, Xie K, Li X, Wen J
J Nephrol 2023 Jan;36(1):93-101. Epub 2022 Jul 22 doi: 10.1007/s40620-022-01385-y. PMID: 35867238
Agrebi I, Kammoun K, Dammak N, Hachicha J, Boudawara T, Jarraya F, Hmida MB
Pan Afr Med J 2021;38:218. Epub 2021 Feb 25 doi: 10.11604/pamj.2021.38.218.21059. PMID: 34046124Free PMC Article
Kawamura T, Usui J, Kaseda K, Takada K, Ebihara I, Ishizu T, Iitsuka T, Sakai K, Takemura K, Kobayashi M, Koyama A, Kanemoto K, Sumazaki R, Uesugi N, Noguchi M, Nagata M, Suka M, Yamagata K
Clin Exp Nephrol 2013 Apr;17(2):248-54. Epub 2012 Sep 13 doi: 10.1007/s10157-012-0690-7. PMID: 22971963
Wei CC, Wang W, Smoyer WE, Licht C
Pediatr Nephrol 2012 Dec;27(12):2243-50. Epub 2012 Jul 18 doi: 10.1007/s00467-012-2249-y. PMID: 22802081
Wu MJ, Shu KH, Chan LP, Lu YS, Cheng CH, Sheu SS, Hsu YH, Lian JD
Zhonghua Yi Xue Za Zhi (Taipei) 1996 Jan;57(1):34-41. PMID: 8820034

Clinical prediction guides

Wu Q, Liang D, Song T, Ni X, Wu X, Zhang M, Chen J, Cheng D, Xie K, Li X, Wen J
J Nephrol 2023 Jan;36(1):93-101. Epub 2022 Jul 22 doi: 10.1007/s40620-022-01385-y. PMID: 35867238
Agrebi I, Kammoun K, Dammak N, Hachicha J, Boudawara T, Jarraya F, Hmida MB
Pan Afr Med J 2021;38:218. Epub 2021 Feb 25 doi: 10.11604/pamj.2021.38.218.21059. PMID: 34046124Free PMC Article
Kaczmarczyk K, Musiał J, Soja J, Kuźniewski M, Gala-Błądzińska A, Białas M, Okoń K
Pol J Pathol 2015 Jun;66(2):149-53. doi: 10.5114/pjp.2015.53011. PMID: 26247528
Harmankaya O, Baştürk T, Oztürk Y, Karabiber N, Obek A
Int Urol Nephrol 2001;33(3):583-7. doi: 10.1023/a:1019546617485. PMID: 12230299
Naiker IP, Ramsaroop R, Somers SR, Randeree IG, Naicker S, Seedat YK
Am J Kidney Dis 1996 Mar;27(3):316-20. doi: 10.1016/s0272-6386(96)90352-2. PMID: 8604698

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