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Ency. home > Disease > B > Bassen-Kornzweig syndrome

Bassen-Kornzweig syndrome   

Overview | Symptoms | Treatment | Prevention

Alternative names:

Abetalipoproteinemia; Acanthocytosis; Apolipoprotein B deficiency

Treatment

Consult a nutritionist or other medical professional for dietary teaching. 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. The fat in the diet should come from medium-chain triglycerides since they are absorbed from the gut differently than most other fats.

The diet should contain no more than 5 ounces of lean meat, fish, or poultry per day. Use skim milk instead of whole milk.

Prognosis

The outcome is related to the degree and progression of neurological and visual problems.  Severe forms of the disease lead to irreversible neurologic disease before age 30.

Complications

  • Blindness
  • Mental deterioration
  • Loss of function of peripheral nerves, ataxia

Call Your Health Care Provider If:

Call if your infant or child exhibits symptoms of this disease.

Ency. home > Disease > B > Bassen-Kornzweig syndrome

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