Inherited Disease in the Staffordshire Bull Terrier
As with other breeds of dog the Staffordshire bull terrier can suffer from inherited disease. The AHT has been researching the genetic basis of two such diseases over the last few years, Hereditary Cataract (HC) and L-2-Hydroxyglutaric Aciduria (L-2-HGA) and is pleased to announce the development of DNA Diagnostic Tests for both these conditions are now available at the AHT.L-2-Hydroxyglutaric aciduria
L-2-HGA (L-2-hydroxyglutaric aciduria) in Staffordshire Bull Terriers is a neurometabolic disorder characterised by elevated levels of L-2-hydroxyglutaric acid in urine, plasma and cerebrospinal fluid. L-2-hydroxyglutarate is normally metabolised to a-ketoglutarate but in affected dogs it is not, and builds up in the body with devastating results. L-2-HGA affects the central nervous system, with clinical signs usually apparent between 6 months and one year (although they can appear later). Symptoms include epileptic seizures, "wobbly" gait, tremors, muscle stiffness as a result of exercise or excitement and altered behavior.Hereditary Cataract
Hereditary cataract in the Staffordshire bull terrier was first reported in the United Kingdom in 1976. The condition is not congenital, so puppies are born with normal eyes, but cataracts begin to appear at a few weeks to months in age, progressing to total cataracts by 2 to 3 years of age. This cataract is always bilateral, symmetrical in the two eyes, and progressive until total with resultant blindness.DNA Diagnostic Tests
After several years of research into the genetic basis of these two inherited conditions the AHT is pleased to announce the identification of the mutations that cause both diseases.How Breeders Can Benefit From the DNA Diagnostic Tests
Owners can now submit a DNA sample from any dog for testing and find out whether the dog is clear, a carrier or affected with either, or both, HC and L-2-HGA. The owner will receive a certificate that states whether the dog is Clear, Carrier or Affected, with the following explanations: CLEAR: the dog has 2 copies of the normal gene and will neither develop HC / L-2-HGA, nor pass a copy of the HC / L-2-HGA mutation to any of its offspring. CARRIER: the dog has one copy of the normal gene and one copy of the mutant gene that causes HC / L-2-HGA. It will not develop HC / L-2-HGA but will pass on the HC / L-2-HGA mutation to 50% (on average) of its offspring. AFFECTED: the dog has two copies of the HC / L-2-HGA mutation and is affected with HC / L-2-HGA and will develop HC / L-2-HGA during its lifetime. Further Deatails and DNA testing forms can be downloaded from the AHT web site http://www.aht.org.uk.PHPV & PPSC
PHPV – PERSITENT HYPERPLASTIC PRIMARY VITREOUS
The mode of inheritance of PHPV is not so clear, but it is known that it is a congenital condition (present at birth) and that it is not progressive. This means that if a puppy is born with PHPV it can be detected by ophthalmic screening from 6 weeks of age and if it is affected, whatever the condition of the problem at that stage it will not change throughout the dogs life.Either of the above conditions can be operated on, but it is a serious operation and can be traumatic and very expensive. It is not always covered by insurance due to the hereditary nature.
Even though the genetic test is now available for Hereditary Cataracts it is still important to screen for PHPV.