Hereditary nephritis in a miniature bull terrier

Hood, J. C.; Aj, Craig

doi:10.1136/vr.135.6.138

Veterinary Record

1994

DOI: 10.1136/vr.135.6.138

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Hereditary nephritis in a miniature bull terrier

J. C. Hood

Craig Aj²

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“…The cystic glomerular capsular change, which was present in these Rottweilers, has been described in Bull Terriers 5,7 and some Doberman Pinschers 2 but has not been reported in people and other dog breeds with Alport syndrome. This lesion has been termed ''cystic glomerular atrophy,'' based on the small size of some glomeruli within the dilated Bowman's space.…”

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confidence: 96%

Histologic and Ultrastructural Studies of Juvenile Onset Renal Disease in Four Rottweiler Dogs

Wakamatsu¹,

Surdyk²,

Carmichael³

et al. 2007

Vet Pathol

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Abstract. Juvenile onset renal disease is described in 2 male and 2 female young Rottweiler dogs. Histologic changes in all dogs were cystic dilatation of Bowman's space, mesangial hypercellularity, and glomerulosclerosis. Three dogs also had glomerular crescents and moderate to severe interstitial fibrosis. Electron microscopy revealed glomerular basement membranes of variable thickness, with extensive splitting or lamellation of the lamina densa. These ultrastructural findings are similar to those found in people and in other breeds of dogs with inherited defects in type IV collagen.

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mentioning

confidence: 96%

Histologic and Ultrastructural Studies of Juvenile Onset Renal Disease in Four Rottweiler Dogs

Wakamatsu¹,

Surdyk²,

Carmichael³

et al. 2007

Vet Pathol

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Congenital Renal Diseases

Lees

1996

Veterinary Clinics of North America: Small Animal Practice

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Bull terrier hereditary nephritis: A model for autosomal dominant Alport syndrome

Hood

Savige

Hendtlass

et al. 1995

Kidney International

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Bull terrier hereditary nephritis is inherited as an autosomal dominant disease and causes renal failure at variable ages in affected dogs. The aims of this study were to compare the clinical, ultrastructural and immunohistochemical features of bull terrier hereditary nephritis with the characteristics of the human forms of Alport syndrome. Many animals with bull terrier hereditary nephritis have hematuria, and some have anterior lenticonus. However, deafness is not associated with the renal disease, and affected dogs do not have the large platelets that are occasionally seen in patients with autosomal Alport syndrome. The glomerular capillary basement membrane (GCBM) in affected bull terriers has an identical ultrastructural appearance to that seen in X-linked Alport syndrome, with lamellations and intramembranous electron-dense deposits. However, both the Goodpasture and the Alport antigens, which represent parts of the alpha 3(IV) and alpha 5(IV) collagen chains, respectively, are present in the GCBM of affected dogs. Bull terrier hereditary nephritis represents an animal model for autosomal dominant Alport syndrome, and can be used to further examine how genetic mutations affect a basement membrane protein and the corresponding membrane structure.

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Hereditary nephritis in a miniature bull terrier

Cited by 3 publications

References 0 publications

Histologic and Ultrastructural Studies of Juvenile Onset Renal Disease in Four Rottweiler Dogs

Histologic and Ultrastructural Studies of Juvenile Onset Renal Disease in Four Rottweiler Dogs

Congenital Renal Diseases

Bull terrier hereditary nephritis: A model for autosomal dominant Alport syndrome

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