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Bassen-Kornzweig Syndrome

Bassen-Kornzweig Syndrome is an inherited disorder that affects how fats are made and used in the body. The body needs fats for healthy nerves, muscles, and digestion. Like oil and water, fats can't travel in the blood around the body by themselves-they just don't mix in. Fats attach to special proteins called lipoproteins to travel to wherever they're needed.

Because of a genetic disorder, people with Bassen-Kornzweig Syndrome don't produce a critical "building block" (a special protein) for making lipoproteins. Without enough lipoproteins, fats can't be digested properly or travel around the body to where they're needed, which leads to serious health problems.

Symptoms of Bassen-Kornzweig Syndrome include

* Failure to thrive in infancy
* Fatty stools that appear pale in color
* Frothy stools
* Abnormally foul-smelling stools
* Protruding abdomen
* Developmental delay
* Poor muscle coordination that usually develops after age 10
* Muscle weakness
* Slurred speech
* Curvature of spine
* Progressive decreased vision
* Balance and coordination difficulties

Treatment of Bassen-Kornzweig Syndrome

Large doses of vitamin supplements containing the fat-soluble vitamins (vitamin A, vitamin D, vitamin E and vitamin K) are given. To avoid intestinal symptoms, avoid eating long-chain triglycerides. Thus, the diet should contain no more than 5 ounces of lean meat, fish, or poultry per day. Use skim milk instead of whole milk. Since a certain amount of fat is needed for normal growth and development in all people, medium chain triglycerides are alternatively used as the major source of fat in the diet. These are absorbed from the gut differently than other fats, and thus avoid the intestinal symptoms. Medium chain triglycerides are taken as a dietary supplement, typically under the supervision of a physician or nutritionist.

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