In canines, Samoyed Hereditary Glomerulopathy [glo-mer″u-lop´ah-the] (SHG) is an hereditary noninflammatory disease, of the renal glomeruli occuring in the Samoyed breed of dog. The disease has been shown to be a model for hereditary nephritis (HN) in humans[1] in that the disease resembles that of the human disease. Because of this, it is sometimes referred to by the name given to the disease in humans when referring to the conditions in Samoyed dogs. Alternativly, it may also be known as X-linked hereditary nephritis. Genetically, it is a sex-linked, genetically dominant disease, and thus affects male dogs to a greater degree than bitches, since males only have one X chromsome.
Description edit
Samoyed Hereditary Glomerulopathy is caused by a nonsense mutation in codon 1027 of the COL4A5 gene on the X chromosome (glycine to stop codon), which is similar to Alport's syndrome in humans.[2] The disease is simply inherited, X-linked dominant, with males generally having more severe symptoms than females. Clinically, from the age of three to four months, proteinuria in both sexes is seen. In dogs older than this, renal failure in combination with more or less pronounced hearing loss occurs swiftly, and death at the age of 8 to 15 months is expected. In heterozygous females, whereby only one of the two X chromosomes carry the mutation, the disease develops slowly.[3][4]
The disease is specific to the Samoyed in that, the Samoyed, is the only breed of dog to show the more rapid progression to renal failure and death, as well as affecting males to a much more severe degree than females. The Samoyed, however is not the only breed of dog to suffer from life threatening renal diseases. Protienuria has been found consistently in Samoyeds, Doberman Pinschers, and Cocker spaniels.[5][6]
Diagnosis edit
Affected male and carrier female dogs generally begin to show signs of the disease at two to three months of age, with proteinuria. By three to four months of age, symptoms include for affected male dogs: bodily wasting and loss of weight, proteinuria & hypoalbuminemia. Past nine months of age, hypercholesterolemia may be seen.[1] In the final stages of the disease, at around 15 months of age for affected males, symptoms are reported as being renal failure, hearing loss and death. Since the condition is genetically dominant, diagnosis would also include analysis of the health of the sire and dam of the suspected affected progeny if available.
Treatment edit
The disease can be treated only to slow down the development, by use of cyclosporine A[4] and ACE inhibitors, but not stopped or cured.[7]
References edit
- ^ a b Jansen, B.; Valli, V. E.; Thorner, P.; Baumal, R.; Lumsden, J. H. (1987). "Samoyed Hereditary Glomerulopathy: Serial, Clinical and Laboratory (Urine, Serum Biochemistry and Hematology) Studies". Can. J. Vet. Res. 51 (3): 387–393. PMC 1255344. PMID 3651895.
{{cite journal}}: CS1 maint: date and year (link) - ^ B. Jansen u. a.: Mode of inheritance of Samoyed hereditary glomerulopathy: an animal model for hereditary nephritis in humans. In: Journal of Laboratory and Clinical Medicine . 107, Nr. 6, 1986, S. 551–555, PMID 3711721.
- ^ K. Zheng u. a.: Canine X chromosome-linked hereditary nephritis: a genetic model for human X-linked hereditary nephritis resulting from a single base mutation in the gene encoding the alpha 5 chain of collagen type IV. In: Proceedings of the National Academy of Sciences of the United States of America. 91, Nr. 9, 1994, S. 39893993, PMID 8171024
- ^ a b CHEN, DILYS (October 2003). "Cyclosporine A Slows the Progressive Renal Disease of Alport Syndrome (X-Linked Hereditary Nephritis): Results from a Canine Model" (PDF). Journal of the American Society of Nephrology. 14 (3). American Society of Nephrology: 690–698. doi:10.1097/01.ASN.0000046964.15831.16. PMID 12595505. Retrieved 22 May 2011.
{{cite journal}}: Unknown parameter|coauthors=ignored (|author=suggested) (help)CS1 maint: date and year (link) - ^ Wilcock, B. P.; Patterson, J. M. (1979). "Familial glomerulonephritis in Doberman pinscher dogs". Canadian Veterinary Journal. 20 (9): 244–249. PMC 1789598. PMID PMC [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1789598/ 1789598 [[PMC (identifier)|PMC]] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1789598/ 1789598]].
{{cite journal}}: Check|pmid=value (help); External link in|pmid= - ^ Steward, A. P.; MacDougall, D. F. (1984). "Familial nephropathy in the Cocker Spaniel". Journal of Small Animal Practice. 25 (1): 15–24. doi:10.1111/j.1748-5827.1984.tb00475.x. Retrieved 21 May 2011.
{{cite journal}}: Check date values in:|year=/|date=mismatch (help); Unknown parameter|month=ignored (help) - ^ Grodecki, K.M.; Gains, M.J.; Baumal, R.; Osmond, D.H.; Cotter, B.; Valli, V.E.O.; Jacobs, R.M. (1997). "Treatment of X-linked hereditary nephritis in samoyed dogs with angiotensin converting enzyme (ACE) inhibitor". Journal of Comparative Pathology. 117 (3): 209–225. doi:10.1016/S0021-9975(97)80016-3. PMID 9447482.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: date and year (link)
Further reading edit
- Thorner, P (April 1987). "Samoyed hereditary glomerulopathy. Immunohistochemical staining of basement membranes of kidney for laminin, collagen type IV, fibronectin, and Goodpasture antigen, and correlation with electron microscopy of glomerular capillary basement membranes". Laboratory Investigation: A Journal of Technical Methods and Pathology. 56 (4): 435–443. PMID 3550289. Retrieved 21 May 2011.
{{cite journal}}: Unknown parameter|coauthors=ignored (|author=suggested) (help)CS1 maint: date and year (link) - Jansen, B (1986). "Samoyed hereditary glomerulopathy (SHG). Evolution of splitting of glomerular capillary basement membranes". American Journal of Pathology. 125: 536–545. Retrieved 21 May 2011.
{{cite journal}}: Unknown parameter|coauthors=ignored (|author=suggested) (help) - "Hereditary Nephritis-Samoyed Hereditary Glomerulopathy". VetGen. Retrieved 21 May 2011.
- Rawdon, T.G. (May 2001). "Juvenile nephropathy in a Samoyed bitch". The Journal of Small Animal Practice. 42 (5). Anchorage Veterinary Hospital, Acle, Norfolk.: 235–238. doi:10.1111/j.1748-5827.2001.tb02027.x. PMID 11380016. Retrieved 21 May 2011.
{{cite journal}}: CS1 maint: date and year (link) - Zheng, Keqin; Perry, Julie; Harvey, Scott J.; Sado, Yoshikazu; Ninomiya, Yoshifumi; Jefferson, Barbara; Jacobs, Robert; Hudson, Billy G.; Thorner, Paul S. (2005). "Regulation of collagen type IV genes is organ-specific: Evidence from a canine model of Alport syndrome". Kidney International. 68 (5): 2121–2130. doi:10.1111/j.1523-1755.2005.00668.x. PMID 16221211. Retrieved 22 May 2011.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: date and year (link)