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MAT deficiency

Beta-ketothiolase deficiency is an uncommon inherited disorder in which the body cannot properly process the amino acid isolecine or the products of lipid breakdown. The condition is inherited in an autosomal recessive pattern and is extremely rare having only been reported in 50 to 60 individuals throughout the world. more...

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The typical age of onset for this disorder is between 6 months and 24 months. The signs and symptoms of beta-ketothiolase deficiency include vomiting, dehydration, trouble breathing, extreme tiredness, and occasionally convulsions. These episodes are called ketoacidotic attacks and can sometimes lead to coma. Attacks occur when compounds called organic acids (which are formed as products of amino acid and fat breakdown) build up to toxic levels in the blood. These attacks are often triggered by an infection, fasting (not eating), or in some cases, other types of stress.

Mutations in the ACAT1 gene cause beta-ketothiolase deficiency. The enzyme made by the ACAT1 gene plays an essential role in breaking down proteins and fats in the diet. Specifically, the enzyme is responsible for processing isoleucine, an amino acid that is part of many proteins. This enzyme also processes ketones, which are produced during the breakdown of fats. If a mutation in the ACAT1 gene reduces or eliminates the activity of this enzyme, the body is unable to process isoleucine and ketones properly. As a result, harmful compounds can build up and cause the blood to become too acidic (ketoacidosis), which impairs tissue function, especially in the central nervous system.

This article incorporates public domain text from The U.S. National Library of Medicine

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Perks for pets: nutritional supplements for Fluffy and Fido
From E: The Environmental Magazine, 11/1/04 by Kathleen M. Mangan

There's little question that Americans love their pets dearly. A recent survey by the American Animal Hospital Association reveals that four out of five pet owners consider their pets to be their children. And just as parents try to provide the best nutritional health for their kids, they're turning to dietary supplements to meet the long-term health needs of their animals.

Although pet food is often advertised as a 100 percent complete and balanced diet, certain beneficial vitamins, fats and nutrients are baked out of the food in processing. As consumers become more aware of the limitations of commercial pet food and more educated on the latest nutritional research, they are fueling a burgeoning supplements market.

According to a study by the American Veterinary Medical Association, one-third of all dogs, cats and horses receive daily dietary supplements. The National Animal Supplement Council estimates the market at $1 billion a year. Numerous companies have jumped into the market with new product offerings.

"Natural supplements for pets are becoming mainstream," says Terri Grow, owner of Pet Sage, a holistic pet product catalog and website. She had to search for natural products to include in her first catalog in 1995, but now she has more than 1,000. Natural and holistic products can effectively treat or prevent common ailments, seasonal allergies, emotional issues and chronic disease. They typically help the body repair itself, so achieving benefits may take four to six weeks.

Fewer Side Effects

Consumers and holistic veterinarians often prefer natural pet supplements and products because they typically have fewer side effects than synthetic formulas and prescription medications. Glucosamine, the most popular of all pet supplements, supports strong and healthy joints naturally, whereas studies have shown that most of the non-steroidal anti-inflammatories prescribed for arthritis can lead to liver damage. A good choice for joint support is the Glyco-Flex line produced by Vetri-Science Laboratories ($44.99 to $74.99).

The side effects of sedatives and calming drugs like Prozac used for destructive, anxious and fearful pets make animals sleepy or lethargic. A different approach by Farnam Pet Products incorporates soothing pheromones into a spray ($34.99) and a room diffuser ($27.99). The canine version of Comfort Zone mimics the pheromone put off by nursing mother dogs to comfort puppies; the feline version mimics the pheromone put off by cats when they rub their cheeks against people and objects. Studies have shown that the product ends 86 percent of destructive dog behavior and 70 percent of excessive barking, while in cats it reduces or eliminates 95 percent of urine-marking and vertical scratching. The product is scent-free.

In another approach to the same problem, Petstages incorporates buckwheat-filled microwavable warming packets into a stuffed pillow (pictured) to calm newly separated pups ($6.99), a saddle-shaped pad to relieve joint stiffness for senior dogs ($5.99), and a stuffed mat to comfort cats ($7.99).

For allergies, veterinarians often prescribe antihistamines that can make a pet drowsy, or steroids like Prednizone that can cause incontinence in the short-term and liver damage in the long-term. An herbal tonic called Detox Blend ($9.99) by Animals' Apawthecary utilizes burdock, dandelion, milk thistle seed, red clover, alfalfa and licorice to treat allergic reactions and liver toxicity. For allergic reactions to flea bites, Grow says her customers report good results with Dr. Goodpets' Flea Relief ($8.99), a homeopathic.

Supplements Enhance Medications

Nutraceuticals are not necessarily a substitute for prescription drugs since they are often used with conventional medications for a synergistic effect. Complementary use of a natural product can allow a reduced drug dose, which is helpful if the drug has side effects. In cases of chronic disease like cancer, trying both conventional and alternative medicine gives pets more of a fighting chance. CAS Options by Genesis Ltd. supports the animal's immune system with three types of medicinal mushrooms (maitake, reishi and shiitake), Chinese herbs, vitamins and antioxidants. It also activates production of natural killer cells that eliminate viruses and cancer cells, supports heart and kidney function, detoxifies the liver and enhances energy.

Sometimes nutraceuticals are the only treatment option. Conventional medicine has nothing to offer animals suffering from kidney disease, one of the top causes of death among cats and dogs. The kidneys clean urinary toxins, and when they fail, poisonous toxins in the pet's body make it progressively ill until it stops eating and drinking. A new product debuting at the end of the year, Kibow Biotics made by Kibow Biotech, provides naturally occurring probiotics that eat the toxins left behind by malfunctioning kidneys. By eliminating the toxins, the animal can enjoy a better quality of life for a longer period of time.

Consumers may choose natural products because the ingredients seem safer for the animal. For example, many hairball remedies are petroleum-based, whereas Vet Basis Healthy Breath & Body for Cats is a gel of canola oil, hydrogenated vegetable oil and cod liver oil ($11.99). It also includes live probiotics, Omega-3 and Omega-6 fatty acids, taurine and vitamins that aid digestion and breath odor.

Consumer Awareness

Increasing awareness and education about pet nutritional needs has fostered the growth of certain supplements like essential fatty acids (EFA), primarily Omega-3 and Omega-6. The body cannot produce EFA, and a deficiency can produce hair loss, skin conditions, liver and kidney degeneration, and immune dysfunction. EFA Capsules by Animal Essentials ($19.99) include fish oils as well as plant-derived oils (borage seed, flax seed, wheat germ oils), lecithin, spirulina, vitamin E and sea bed trace minerals.

When people use natural products themselves, they often realize the treatments might benefit their pets too. For instance, many people take probiotics when they are taking antibiotics to replenish the beneficial bacteria in their digestive system wiped out by the antibiotics. Probiotics are also effective against diarrhea, some food allergies, skin infections, yeast infections and irritable bowel syndrome. Gentle Digest by Ark Naturals ($9.99) includes microencapsulated lactobacillus spores, which are resistant to heat and stomach acid, plus chicory, a prebiotic or food for the lactobacilli to encourage their growth.

Many natural products are simply easier to use. For example, clean teeth are essential to long-term health because mouth infections can spread to body organs. But brushing a dog's or cat's teeth can be a struggle, and dental cleaning by the veterinarian requires anesthesia, which can be risky for older or ill animals. An alternative is Wysong Dentatreat ($6.99), a powder to sprinkle on food made of dental-active cheeses, probiotic cultures, enzymes, calcium and other ingredients to discourage bacteria growth, and prevent tooth decay and gingivitis.

Ensuring Product Quality

Some pet supplements are available only through veterinarians, but a majority of natural products and supplements can be had through pet stores, natural food stores and specialty catalogs. Bill Bookout, president of the National Animal Supplement Council, recommends using products formulated for animals rather than humans to ensure proper dosage and to avoid ingredients that may be toxic to pets.

Since the FDA prevents label statements about therapeutic uses and ingredient grades, and doesn't regulate product quality, consumers must become educated about holistic health care, seek recommendations from natural product suppliers or a holistic veterinarian, and trust their chosen brand. Check to see if the product ingredients are human-grade or certified organic; if the product has a lot number and expiration date to ensure tracking and quality control; and if it is produced according to USP (U.S. Pharmacopeia) Guidelines. To further ensure quality, look for seals of approval from the National Animal Supplement Council, Consumer Lab and the National Nutritional Foods Association's Good Manufacturing Practices program. CONTACT: Academy of Veterinary Homeopathy, (866)652-1590, www. theavh.org; American Academy of Veterinary Acupuncture, (973)379-1100, www.aava.org; American Holistic Veterinary Medical Association, (410)569-0795,www. ahvma.org; American Veterinary Chiropractic Association, (918) 784-2231, www.animal chiropractic.org; International Veterinary Acupuncture Society, (970) 266-0666, www.ivas. org; National Animal Supplement Council, (760)751-3360, www.nasc.cc; Pet Sage, (800)738-4584, www.petsage.com; Veterinary Botanical Medicine Association, www.vbma.org.

KATHLEEN M. MANGAN, the former "Natural Front" columnist for Dog Fancy, specializes in holistic pet subjects.

COPYRIGHT 2004 Earth Action Network, Inc.
COPYRIGHT 2004 Gale Group

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