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Mesangial microfibril deposition

MedGen UID:
1778692
•Concept ID:
C5539672
•
Finding
HPO: HP:0033546

Definition

Unbranched noncollagenous microfibrils within the mesangial matrix, composed of proteins not present within the glomerular basement membrane (GBM), most notably fibrillin-1, that attach to mesangial cells and GBM structural proteins. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMesangial microfibril deposition

Recent clinical studies

Etiology

Fibrillary glomerulonephritis in a 9-year-old girl.
Chung WY, Lee SY, Joo JE
Nephron 1995;69(1):79-82. doi: 10.1159/000188364. PMID: 7891802
Glomerulonephritis with organized deposits: a new clinicopathological entity? Light-, electron-microscopic and immunofluorescence study of 12 cases.
Mazzucco G, Casanova S, Donini U, Rollino C, Zucchelli P, Piccoli G, Monga G
Am J Nephrol 1990;10(1):21-30. doi: 10.1159/000168049. PMID: 2111635
Glomerular matrix proteins in nodular glomerulosclerosis in association with light chain deposition disease and diabetes mellitus.
Bruneval P, Foidart JM, Nochy D, Camilleri JP, Bariety J
Hum Pathol 1985 May;16(5):477-84. doi: 10.1016/s0046-8177(85)80086-1. PMID: 3921452

Diagnosis

A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9.
Hattori K, Shimizu R, Tanaka S, Terashima T, Hiramatsu M, Shimomura T, Ito T, Morinaga T, Terasaki M, Shimizu A, Morozumi K, Tamai H
CEN Case Rep 2022 Nov;11(4):412-416. Epub 2022 Feb 23 doi: 10.1007/s13730-022-00693-3. PMID: 35199316Free PMC Article
Fibrillary glomerulonephritis in a 9-year-old girl.
Chung WY, Lee SY, Joo JE
Nephron 1995;69(1):79-82. doi: 10.1159/000188364. PMID: 7891802
Nonamyloidotic fibrillar glomerulopathy and recurrent gestational anasarca.
Bakir AA, Ainis H, Rhee HL
Am J Nephrol 1990;10(4):333-8. doi: 10.1159/000168129. PMID: 2240062
Glomerulonephritis with organized deposits: a new clinicopathological entity? Light-, electron-microscopic and immunofluorescence study of 12 cases.
Mazzucco G, Casanova S, Donini U, Rollino C, Zucchelli P, Piccoli G, Monga G
Am J Nephrol 1990;10(1):21-30. doi: 10.1159/000168049. PMID: 2111635
Glomerular matrix proteins in nodular glomerulosclerosis in association with light chain deposition disease and diabetes mellitus.
Bruneval P, Foidart JM, Nochy D, Camilleri JP, Bariety J
Hum Pathol 1985 May;16(5):477-84. doi: 10.1016/s0046-8177(85)80086-1. PMID: 3921452

Therapy

A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9.
Hattori K, Shimizu R, Tanaka S, Terashima T, Hiramatsu M, Shimomura T, Ito T, Morinaga T, Terasaki M, Shimizu A, Morozumi K, Tamai H
CEN Case Rep 2022 Nov;11(4):412-416. Epub 2022 Feb 23 doi: 10.1007/s13730-022-00693-3. PMID: 35199316Free PMC Article

Prognosis

Glomerulonephritis with organized deposits: a new clinicopathological entity? Light-, electron-microscopic and immunofluorescence study of 12 cases.
Mazzucco G, Casanova S, Donini U, Rollino C, Zucchelli P, Piccoli G, Monga G
Am J Nephrol 1990;10(1):21-30. doi: 10.1159/000168049. PMID: 2111635
Glomerulonephropathy with amyloid-stain-negative microfibrillar glomerular deposits.
Kobayashi Y, Fujii K, Kurokawa A, Kamiyama M, Miyazawa H
Nephron 1988;48(1):33-9. doi: 10.1159/000184865. PMID: 3277075

Clinical prediction guides

Demonstration of collagen type VI and alpha-smooth muscle actin in renal fibrotic injury in man.
Groma V
Nephrol Dial Transplant 1998 Feb;13(2):305-12. doi: 10.1093/oxfordjournals.ndt.a027823. PMID: 9509439
Immunotactoid glomerulopathy and cutaneous vasculitis.
Orfila C, Meeus F, Bernadet P, Lepert JC, Suc JM
Am J Nephrol 1991;11(1):67-72. doi: 10.1159/000168276. PMID: 1904680

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