Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

Kobayashi, Ryosuke; Yasuno, Kyohei; Ogihara, Kikumi; Yamaki, Munetaka; Kagawa, Yumiko; Kamiie, Junichi; Shirota, Kinji

doi:10.1292/jvms.71.1137

Cited by 8 publications

(15 citation statements)

References 20 publications

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“…Glomerular C3 deposits were not detected in another described canine case where C3 was investigated, while glomerular IgM and IgG deposits were occasionally seen. 6 In humans, immunofluorescence for IgG is mainly negative, and staining for C3 and IgM is negative or shows focal and segmental staining, often along the capillary walls, 10,[15][16][17]20,31 coinciding with our findings. The deposits are described as non-specific 2 and not indicative of an immune-mediated process, 1 as also confirmed by lack of immune complexes by EM.…”

Section: Discussionsupporting

confidence: 83%

“…Immunohistochemistry also revealed that some of the mesangial cells in the glomeruli of affected cases were a-SMA positive, as previously seen in other dogs with Col3GP 5,6 and in a human case. 20 In another animal case report, however, a-SMA was not detected in affected glomeruli.…”

Section: Discussionsupporting

confidence: 81%

“…The typical clinical features are proteinuria, hypertension and a variable degree of slowly progressive renal impairment, and the disease tends to have a more aggressive course in children than in adults. 2 In veterinary medicine Col3GP is reported to occur in various animal species, including monkeys, 3,4 dogs, [5][6][7] pigs 8 and a cat. 9 The pathogenic mechanisms of Col3GP are obscure, and it is not clarified whether it is a primary renal disease in which the collagen is produced in the glomeruli itself or a systemic disease where the collagen or its precursor is derived from an extrarenal source.…”

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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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Section: Discussionsupporting

confidence: 83%

Section: Discussionsupporting

confidence: 81%

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confidence: 99%

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A canine autosomal recessive model of collagen type III glomerulopathy

Rørtveit

Lingaas

Bønsdorff

et al. 2012

Laboratory Investigation

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“…In a previous litter from the same dam and sire, two of the puppies had died at a young age, strongly supporting a hereditary disease [ 9 ]. The two other case reports describe a seven month old mongrel dog with growth retardation and depression [ 8 ], and a Shiba Inu that presented with anorexia at three years of age [ 7 ]. Both cases had a mortal outcome and at necropsy the dogs had pathological changes characteristic of Col3GP.…”

Section: Discussionmentioning

confidence: 99%

A clinical study of canine collagen type III glomerulopathy

et al. 2013

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BackgroundCollagen type III glomerulopathy (Col3GP), also known as collagenofibrotic glomerulonephropathy, is a rare renal disease with unknown pathogenesis that occurs in animals and humans. We recently described a naturally occurring canine autosomal recessive model of Col3GP, and the aim of the present work was to study the clinical features of canine Col3GP and compare with the human phenotype. In humans two different clinical syndromes with different age at onset (child- or adulthood) have been observed. In children a more aggressive course with familial occurrence is described, characterized by progressively increasing proteinuria, nephrotic syndrome, hypertension and chronic renal failure. A markedly increased serum level of the aminoterminal propeptide of type III procollagen (PIIINP) is considered a useful marker for the disease. Since Col3GP and concurrent hypocomplementemia have been observed in humans, we also aimed to investigate if hypocomplementemia was present in Col3GP affected dogs. A litter consisting of seven puppies, four Col3GP affected and three healthy unaffected, was observed from the day of birth until the affected puppies developed a mild or moderate renal azotemia.ResultsDuring the period of observation growth retardation, increasing blood pressure, progressive proteinuria, azotemia, hypoalbuminemia, hypercholesterolemia and increased serum PIIINP were observed in all the affected dogs. Hypocomplementemia was not detected. Affected dogs were euthanized between 109 and 144 days of age, and pathological examinations revealed ascites and massive glomerular accumulations of collagen type III, consistent with Col3GP.ConclusionsDogs with Col3GP develop juvenile chronic renal failure, preceded by nephrotic syndrome, elevated serum PIIINP and hypertension, thus have similar clinical features as the juvenile Col3GP in humans. Further studies of this naturally occurring canine phenotype may provide more information on the pathogenesis and genetics of Col3GP in both animals and humans.

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“…21 The clinical features in dogs are similar to those of affected children. 7 With one exception, 9 all described canine cases 10,11,21,22 developed clinical signs of chronic renal failure within a year of birth.…”

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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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Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

Cited by 8 publications

References 20 publications

A canine autosomal recessive model of collagen type III glomerulopathy

A canine autosomal recessive model of collagen type III glomerulopathy

A clinical study of canine collagen type III glomerulopathy

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

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