Hereditary X-linked Nephritis

Kidney failure, although generally thought to be a condition associated with old age, can be seen at any age and is not uncommon in dogs under five years old. Juvenile kidney diseases have been reported in many breeds of dogs. However, there are few instances where a genetic basis has been conclusively demonstrated. In cats, juvenile kidney diseases (nephropathies) are much rarer (as are most inherited problems), because the vast majority of cats are bred from totally unrelated parents. Some renal diseases have been shown to have a familial basis in dogs and cats.

X-linked hereditary nephritis in the Samoyed is similar to Alport syndrome in humans. In the Dalmatian breed, the mode of inheritance is autosomal dominant. The age at onset of kidney failure is 18 months. Both males and females may be affected equally often and equally severely.

Hereditary nephritis is a group of inherited conditions characterized initially by presence of blood. With time, nephritis slowly progresses to renal insufficiency, poor function of the kidneys that may be due to a reduction in blood-flow to the kidneys caused by renal artery disease. At this stage, the kidneys perform below the normal level in the ability to remove wastes, concentrate urine, maintain electrolyte balance (sodium and potassium), control blood pressure, and metabolize calcium. Renal insufficiency usually develops by five months of age and progresses to uremia and death by eight to ten months. No visual or hearing defects are usually seen in affected male or female dogs. The most severe form of renal insufficiency is kidney failure. Affected male dogs have excessive amount of protein in the urine, with or without blood.



Angiotensin converting enzyme (ACE) inhibitors have been shown to slow the progression of renal disease in animals and human patients. Treatment improves weight gain and, overall, treated dogs survive 1×36 times longer than affected untreated dogs.

References

  1. Peter D. Vize, Adrian S. Woolf, Jonathan B. L. Bard. The Kidney
  2. K.M. Grodeckia, M.J. Gainsa, R. Baumal, D.H. Osmond, B. Cotter, V.E.O. Valli and R.M. Jacobsa. Treatment of X-linked hereditary nephritis in samoyed dogs with angiotensin converting enzyme (ACE) inhibitor
  3. Cox ML, Lees GE, Kashtan CE, Murphy KE. Genetic cause of X-linked Alport syndrome in a family of domestic dogs
  4. Jennifer C Hood, Judy Savige, Anne Hendtlass, Mary M Kleppel, Clive R Huxtable and Wayne F Robinson. Bull terrier hereditary nephritis: A model for autosomal dominant Alport syndrome
  5. Jennifer C. Hood, Clive Huxtable, Ichiro Naito, Carole Smith, Roger Sinclair and Judy Savige. A novel model of autosomal dominant Alport syndrome in Dalmatian dogs
  6. Allison Gleadhill, PhD. Juvenile nephropathies in dogs and cats




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