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Hereditary xanthinuria is a rare autosomal recessive disorder of purine metabolism caused by mutations in xanthine dehydrogenase (Type I) or molybdenum cofactor sulfarase (Type II) genes, and causes xanthine uroliths. Breeds with hereditary xanthinuria include Toy Manchester Terrier, Cavalier King Charles Spaniel, English Cocker Spaniel, Dachshund, Chihuahua, and mixed breed dogs. Xanthine uroliths can also develop as an adverse consequence of xanthine dehydrogenase inhibitors (allopurinol), especially when given in higher doses and without dietary purine/protein reduction. Xanthine uroliths associated with allopurinol administration can be rapidly dissolved by stopping allopurinol and feeding a low-purine, urine-alkalinizing diet.