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| [[Liver_Flashcards_-_Pathology|Liver Pathology Flashcards]] | | [[Liver_Flashcards_-_Pathology|Liver Pathology Flashcards]] |
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| + | (LSD) |
| + | * Occurs in many breeds of dogs and cats. |
| + | * Genetic defects give deficiencies of specific enzymes of degradation. |
| + | ** Disease only occurs in animals homozygous at the gene pair controlling enzyme activity. |
| + | *** The genes are co-dominant. |
| + | ** Enzyme activity is extremely low or entirely absent. |
| + | * Large amounts of waste products accumulate within cells. |
| + | ** Nervous system cells are predisposed. |
| + | ** Cells become engorged. |
| + | ** Normal function is prevented. |
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| + | * [http://w3.vet.cornell.edu/nst/nst.asp?Fun=Display&imgID=20531 Image 1, courtesy of Cornell Veterinary Medicine] <BR> |
| + | * [http://w3.vet.cornell.edu/nst/nst.asp?Fun=Display&imgID=20530 Image 2, courtesy of Cornell Veterinary Medicine] |
| + | * [http://w3.vet.cornell.edu/nst/nst.asp?Fun=Display&imgID=20185 Image 3, courtesy of Cornell Veterinary Medicine] |
| | | |
| + | ===Clinical Signs=== |
| | | |
| | | |
| + | * Nervous system cells are predisposed to the disease, and so progressive neurological signs predominate. |
| + | ** Develop between 2 weeks and 12 months of age. |
| + | ** The disease is ultimately fatal. |
| + | * For example, Siamese cats show: |
| + | ** Ganglisidosis |
| + | ** Mucopolysaccharidosis |
| + | |
| + | ===Diagnosis=== |
| + | |
| + | * Clinical signs are considered. |
| + | * Urine enzyme assays may be helpful. |
| + | * Post-mortem diagnosis is possible. |
| + | * More recently, gene tests have been available. |
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| ==References== | | ==References== |
| + | |
| + | |
| + | [[Category:Central Nervous System - Degenerative Pathology]] |
| [[Category:Liver - Storage Diseases]] | | [[Category:Liver - Storage Diseases]] |
| [[Category:To_Do_-_Clinical]] | | [[Category:To_Do_-_Clinical]] |