Familial juvenile glomerulonephropathy

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Familial juvenile glomerulonephropathy is a relatively common autosomal-recessive genetic renal disease of dogs characterized by juvenile-onset glomerulonephritis and chronic renal disease.

This disease is reported in several breeds of dogs and the mode of inheritance and spectrum of pathological lesions vary amongst breeds.

Progressive juvenile glomerulonephropathy has been reported in the French Mastiff[1], Brittany Spaniel and Bull Terrier[2]. In the Brittany Spaniel, concurrent Complement-3 deficiency is also common.

Affected dogs usually are young (under 2 years of age) and present with dehydration, polydipsia and polyuria.

Hematological examination reveals varying degrees of azotemia and hyperkalemia.

Urinalysis invariably reveals proteinuria and sometimes haematuria.

Definitive diagnosis requires histopathological analysis of renal biopsies. Usually there is varying degrees of cystic glomerular atrophy, glomerular hypercellularity, and capillary wall thickening without immune complex deposition (when tested with immunohistochemistry or immunofluorescence), as well as ultrastructural abnormalities in the glomerular basement membrane, characteristic of 'end-stage' kidneys.

A differential diagnosis would include other congenital renal diseases such as X-linked hereditary nephropathy in Cocker Spaniels and Samoyed dogs, and other causes of chronic renal disease.

Survival rates are poor, with most patients succumbing to renal failure within 6 - 12 months of diagnosis, without renal transplantation.

References

  1. Lavoué R et al (2012) Progressive juvenile glomerulonephropathy in 16 related French Mastiff (Bordeaux) dogs. J Vet Intern Med 24(2):314-322
  2. Hood JC et al (1990) Hereditary nephritis in the bull terrier: evidence for inheritance by an autosomal dominant gene. Vet Rec 126(18):456-459