Inherited factor H deficiency and collagen type III glomerulopathy

Vogt, Beth A.; Wyatt, Robert; Burke, Barbara A.; Simonton, Susan C.; Kashtan, Clifford E.

doi:10.1007/bf00858956

Cited by 80 publications

(86 citation statements)

References 11 publications

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“…We described the case of a 12-year-old male, without a significant family history, who presented with hypertension, anemia, renal insufficiency and hypocomplementemia. Inherited factor H deficiency is associated with collagen type III glomerulopathy and results in hypocomplementemia (14). However, factor H was not detected in the present case.…”

Section: Discussioncontrasting

confidence: 63%

Collagen type III glomerulopathy: A case report and review of 20 cases

Dong

Wei

Han

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. Collagen type III glomer ulopathy is a non-immune-mediated glomerular disease, characterized by abnormal accumulation of type III collagen fibrils within the mesangial matrix and subendothelial space. The clinical manifestations of this disease are proteinuria, peripheral edema, hypertension and occasional progression to end-stage renal disease. Collagen type III glomerulopathy is extremely rare, and its etiology and pathogenesis remain elusive. To date, only case reports are available and the majority of these are from Japan. To investigate the idiographic features of collagen type III glomerulopathy in China, we report a case of collagen type III glomerulopathy with two differing renal biopsies and review 20 cases in China. The majority of the Chinese patients were adults. Thirty percent of the patients had nephrotic syndrome, and hypertension was observed in 75% of cases. Elevated creatinine was present in 45% of patients. The pathology of collagen type III glomerulopathy in the Chinese cases was similar to that observed in other ethnicities, although certain cases were IgA-positive by immunofluorescence microscopy, and electron-dense material could be observed in the mesangial area. The onset age, clinical manifestations and pathological features of the disease are not exactly the same in China as worldwide.

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Section: Discussioncontrasting

confidence: 63%

Collagen type III glomerulopathy: A case report and review of 20 cases

Dong

Wei

Han

et al. 2015

Experimental and Therapeutic Medicine

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“…In addition to the previously described recombinant fragments of fH we generated new mutant proteins spanning the middle and carboxy-terminal parts of fH (constructs containing [15][16][17][18] as tools for the fH-C3b interaction studies.…”

Section: Expression Of the New Recombinant Fragments Of Fhmentioning

confidence: 99%

“…basement membranes in kidney glomeruli) (12)(13)(14). This is exemplified by rare cases of fH deficiency in human beings (15)(16)(17)(18) and pigs (19,20) that lead to complement-dependent kidney damage. The ability of AP to discriminate between activating and nonactivating structures depends on the differential binding of fH to C3b on different types of surfaces.…”

Section: Introductionmentioning

confidence: 99%

Each of the Three Binding Sites on Complement Factor H Interacts with a Distinct Site on C3b

Jokiranta¹,

Hellwage²,

Koistinen³

et al. 2000

Journal of Biological Chemistry

197

157

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“…The length of the bar in the lower right represents 20 lm recessive mode of transmission and early disease progression, which suggests a difference in gene penetrance in different populations [10][11][12]. One case with collagenofibrotic glomerulopathy has been associated with factor H deficiency [13]. There is no evidence of a sex predilection.…”

Section: Discussionmentioning

confidence: 99%

Glomerulopathy with distinctive fibrillar deposits but lacking glomerular deposition of type III collagen

et al. 2016

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A 62-year-old woman with nephrotic syndrome underwent a renal biopsy. Under light microscopy, the biopsy findings included lobulation and enlargement of glomeruli, occasional thickening of glomerular capillary walls, and narrowing of the capillary lumen by swollen endothelial cells. Congo red staining was negative for amyloid. No significant intraglomerular fibrin deposition was found by phosphotungstic acid hematoxylin staining. Immunofluorescence microscopy showed no deposition of immunoglobulin G, A, or M; no j or k light chains; and no C3 or C1q. Electron microscopy revealed distinctive subendothelial and mesangial fibrillar deposits, mesangial cell interposition, and swelling and vacuolization of endothelial cells resulting in capillary lumen narrowing. Although some curvilinear fibrillar deposits mimicked the bundles of type III collagen fibers seen in collagenofibrotic glomerulopathy, neither glomerular deposition of type III collagen nor elevation of serum procollagen III peptide was noted. This glomerulopathy does not fulfill any known disease entities with non-amyloid non-immunoglobulinderived organized glomerular deposits.

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Inherited factor H deficiency and collagen type III glomerulopathy

Cited by 80 publications

References 11 publications

Collagen type III glomerulopathy: A case report and review of 20 cases

Collagen type III glomerulopathy: A case report and review of 20 cases

Each of the Three Binding Sites on Complement Factor H Interacts with a Distinct Site on C3b

Glomerulopathy with distinctive fibrillar deposits but lacking glomerular deposition of type III collagen

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