Spontaneous Collagenofibrotic Glomerulonephropathy in a Young Cynomolgus Monkey

Fujisawa-Imura, Kae; Takasu, Nobuo; Tsuchiya, Naho; Matsushima, Shuuichi; Inagaki, Haruhisa; Torii, Mikinori

doi:10.1293/tox.17.279

Cited by 7 publications

(7 citation statements)

References 6 publications

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“…The clinical, histopathological, immunohistochemical and electron microscopic findings of the glomerular lesions in the present case were consistent with those of CFGN in both humans [2,6] and animals [1,5,9,11,19]. On light microscopy, adhesion of glomerular tufts to the Bowman's capsules and cellular crescent formation indicated severe injury of the glomerular basement membrane (GBM) and podocytes, as seen in the Shiba Inu dog case [9].…”

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confidence: 79%

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Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

Kobayashi

Yasuno

Ogihara

et al. 2009

The Journal of Veterinary Medical Science

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ABSTRACT. This report describes the morphological and immunohistochemical findings of a case of apparent collagenofibrotic glomerulonephropathy in a 7-month-old dog. Clinical examination showed moderate protenuria with elevated blood urea nitrogen and creatinine. Histopathological examination of the glomerular capillary walls and mesangial areas revealed diffuse and global accumulation of eosinophilic homogeneous or fine fibrous materials, which were immunohistochemically positive for type III collagen. On electron microscopy, the randomly crossed fibrils had transverse bands with a periodicity of approximately 60 nm. The clinical, histopathological, immunohistochemical and electron microscopic findings of the present dog were consistent with those of the human, childhood form of collagenofibrotic glomerulonephropathy.

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confidence: 79%

“…In animals, glomerulonephropathy similar to CFGN has been reported in dogs [9,10], a cat [11], pigs [18,19] and cynomolgus monkeys [1,5]. There have been two reports of canine CFGN.…”

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confidence: 98%

Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

Kobayashi

Yasuno

Ogihara

et al. 2009

The Journal of Veterinary Medical Science

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“…Col3GP has also been reported to occur in pigs [ 5 ], dogs [ 6 - 9 ], monkeys [ 10 , 11 ] and a cat [ 12 ], with clinical signs like proteinuria and hypoproteinemia, and a variable degree of renal failure. To the authors’ knowledge blood pressure and serum PIIINP levels in Col3GP affected animals have not been reported.…”

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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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“…Collagen type III glomerulopathy (Col3GP), also known as collagenofibrotic glomerulopathy, is a rare renal disease with unknown pathogenesis that occurs in humans and animals. In veterinary medicine, the disease is reported to occur in monkeys, 2,5 dogs, [9][10][11]21,22 pigs, 25 and a cat. 15 Col3GP is characterized by massive glomerular accumulations of collagen type III.…”

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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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Spontaneous Collagenofibrotic Glomerulonephropathy in a Young Cynomolgus Monkey

Cited by 7 publications

References 6 publications

Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

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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