A Case of Collagenof ibrotic Glomerulopathy Associated with Hepatic Perisinusoidal Fibrosis

Mizuiri, Sonoo; Hasegawa, Akira; Kikuchi, Arata; Amagasaki, Y; Nakamura, Noriko; Sakaguchi, Hiroshi

doi:10.1159/000187180

Cited by 29 publications

(28 citation statements)

References 9 publications

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“…The hepatic collagen fibrils were not frayed and curled as the accumulated glomerular collagen; rather, the fibrils appeared as normal straight collagen, well organized in bundles. In comparison, the 2 reported human cases of Col3GP with hepatic fibrosis 13,27 showed curled collagen fibrils in the liver, similar to those of the glomeruli, in 1 case, and less spiraled and frayed in the liver than in the glomeruli in the other case. Liver involvement might suggest the possibility that Col3GP is a systemic disease; however, the observed liver pathology could also be secondary to the clinical progression of the renal failure in the Col3GP-affected cases.…”

Section: Discussionmentioning

confidence: 75%

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Glomerular Collagen V Codeposition and Hepatic Perisinusoidal Collagen III Accumulation in Canine Collagen Type III Glomerulopathy

et al. 2014

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Collagen type III glomerulopathy, also known as collagenofibrotic glomerulopathy, is a rare renal disease of unknown pathogenesis. The disease occurs in humans and animals and is characterized by massive glomerular accumulations of collagen type III. In the present study, we describe a Drever dog litter affected by an early onset variant of this glomerular disease, where 4 of 9 puppies developed renal failure within 50 days of age. Necropsy specimens of kidney from the 4 affected cases were studied by light microscopy, electron microscopy, and immunohistochemistry, and characteristic lesions compatible with a diagnosis of collagen type III glomerulopathy were found. In addition, 2 cases showed atypical epithelium in the collecting ducts of the medulla, so-called adenomatoid change. Immunohistochemistry of renal specimens from collagen type III glomerulopathy-affected dogs (n ¼ 10) originating from two different dog strains, the Drever dogs and a mixed-breed strain, demonstrated that the deposited glomerular collagen is composed of a mixture of collagen III and collagen V. The distribution of the collagen V corresponded to the localization of collagen III; however, differences in staining intensity showed that collagen type III is the dominating component. Immunohistochemistry for collagen III (n ¼ 9) and a transmission electron microscopic study (n ¼ 1) showed hepatic perisinusoidal collagen type III deposition in affected cases from both dog strains. This is the first report documenting glomerular accumulations of collagen type V and perisinusoidal liver collagen III deposition in canine collagen type III glomerulopathy.

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Section: Discussionmentioning

confidence: 75%

“…Two human patients with concurrent pathologic collagen type III fibrils in the liver have been reported. 13,27 Previous case reports in animals have not reported the occurrence of extrarenal abnormal collagen accumulations.…”

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confidence: 99%

Glomerular Collagen V Codeposition and Hepatic Perisinusoidal Collagen III Accumulation in Canine Collagen Type III Glomerulopathy

et al. 2014

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“…'Primary glomerular fibrosis' was used by Ikeda et al 15 in 1990 after identifying the fibrils as collagen type III. Since then 'collagenofibrotic glomerulonephropathy' , 'collagenofibrotic glomerulopathy' 16,17 and 'Col3GP' 2,18 have been in use. We have chosen to apply the term Col3GP because it describes the anatomical location of the primary pathology and precisely accounts for the characteristics of the glomerulopathy.…”

Section: Discussionmentioning

confidence: 99%

A canine autosomal recessive model of collagen type III glomerulopathy

Rørtveit

Lingaas

Bønsdorff

et al. 2012

Laboratory Investigation

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Collagen type III glomerulopathy (Col3GP) is a rare renal disease characterized by massive glomerular accumulations of collagen type III. The disease occurs in both humans and animals, and has been presumed to be heritable with an autosomal recessive inheritance pattern. The pathogenesis is unknown. We describe herein a condition of canine autosomal recessive Col3GP. This spontaneously occurring canine disease was incidentally diagnosed in six mongrel dogs. We then established and studied a pedigree segregating the disease to confirm the genetic nature and inheritance of canine Col3GP. Twenty-nine percent of offspring (14/48) were affected, strongly supporting a simple autosomal recessive inheritance pattern. Kidney specimens were studied by light microscopy, electron microscopy (EM), immunohistochemistry and in situ hybridization. Characteristic findings of Col3GP previously reported in both humans and animals were demonstrated, including massive glomerular collagen type III deposition, and evidence of local mesangial collagen type III synthesis was found. We propose that canine Col3GP may serve as an animal model of human Col3GP. Our initial studies, using simple segregation analysis, showed that the Col3A1 gene was not involved in the disease. This is the first animal model of Col3GP, and further studies of this phenotype in dogs may have the potential to provide information on the pathogenesis and genetics of the disease in both animals and humans, and may thus contribute to the development of treatment regimes.

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“…l4 In humans, however, neither hereditary factors nor specific background disorders have been suggested. 10,15 To our knowledge this is the first case of feline CFGN-like renal glomerular fibrosis.…”

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confidence: 83%

Renal Glomerular Fibrosis in a Cat

Nakamura

Shibata²,

Shirota³

et al. 1996

Vet Pathol

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Renal glomerular fibrosis was observed in a 1-year-old spayed female Japanese domestic cat that showed clinically advanced renal failure. In the glomeruli, increased homogeneous materials were stained strongly with aniline blue by Masson's trichrome and positive for anti-type III collagen antibody by immunohistochemical staining, causing mesangial sclerosis and capillary collapse. By electron microscopy, randomly arranged fibrils: were observed in the expanded subendothelial and mesangial areas, and the fibrils showed periodicity characteristic of collagen fibers in longitudinal sections. These findings of glomerular lesions closely resemble those of human “collagenofibrotic glomerulonephropathy,” which has recently been described as a new type of glomerulonephropathy.

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A Case of Collagenof ibrotic Glomerulopathy Associated with Hepatic Perisinusoidal Fibrosis

Cited by 29 publications

References 9 publications

Glomerular Collagen V Codeposition and Hepatic Perisinusoidal Collagen III Accumulation in Canine Collagen Type III Glomerulopathy

Glomerular Collagen V Codeposition and Hepatic Perisinusoidal Collagen III Accumulation in Canine Collagen Type III Glomerulopathy

A canine autosomal recessive model of collagen type III glomerulopathy

Renal Glomerular Fibrosis in a Cat

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