Bassen-Kornzweig syndrome
Alternative names:
abetalipoproteinemia
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.
Expectations (prognosis):
The outcome is related to the degree and progression of neurological and visual problems.
Complications:
Calling your health care provider:
Call if your infant or child exhibits symptoms of this disease.
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