Congenital Renal Glomerular Fibrosis in a Case of Swine Chondrodysplasia.

Pathological Characterization of Collagenofibrotic Glomerulonephropathy in a Young Dog

“…This indicates that abnormal type III collagen deposition induces injury of the GBM and podocytes. Electron microscopy also revealed an unusual array of accumulated fibrils with the characteristics of collagen fibrils as seen in other reports on CFGN [2,18]. Clinically acute, progressive disease manifested by protenuria and young age at onset might be comparable to the human childhood type of CFGN.…”

“…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.…”

“…In addition, high levels of serum PIIINP in human CFGN indicate the systemic nature of this condition. However, there have been no reports on systemic abnormalities associated with CFGN in animals, except in a congenital swine case [18]. In contrast, nail-patella syndrome (NPS) is a known systemic disease with abnormal type III collagen deposition.…”

Spontaneous Collagenofibrotic Glomerulonephropathy in a Young Cynomolgus Monkey

“…Arakawa et al 3 and Mizuiri et al 4 reported a case of collagenofibrotic glomerulopathy, and thereafter these type III collagen accumulative glomerular diseases occasionally mixed with type I collagen were classified as collagenofibrotic glomerulonephropathy under WHO classification 5 . Primary glomerular fibrosis was also reported in dogs 6 , pigs 7,8 and cat 9 at about the same time, but had not been reported in nonhuman primates and rodents, that are often used as experimental animals, to the best of our knowledge. We encountered a case of a young male cynomolgus monkey diagnosed as having collagenofibrotic glomerulonephropathy by histopathologic examination, and report here the clinical findings and light and electron microscopic features.…”

Diagnosis of Collagen Type III Glomerulopathy Using Picrosirius Red and PASH/Masson’s Trichrome Stain

“…Collagen type III glomerulopathy (Col3GP), also known as collagenofibrotic glomerulopathy, is a rare renal disease described in humans and several animal species. 1,2,4 –6,8–10,12 –17 Individuals with Col3GP accumulate abundant type III collagen and, to a lesser extent, fibronectin and type V collagen in the mesangium and subendothelial space, adjacent to the glomerular basement membrane. This differs from the normal glomerular extracellular matrix, which contains type IV collagen, fibronectin, and other components, but lacks type III collagen.…”