• Home

Home > Medical Reference > Encyclopedia (English)

Toggle: English / Spanish

Bassen-Kornzweig syndrome

• Overview
• Symptoms
• Treatment
• Prevention

Alternative Names:

Abetalipoproteinemia; Acanthocytosis; Apolipoprotein B deficiency

Treatment:

Consult a nutritionist or other medical professional for dietary instruction. Large doses of vitamin supplements containing the fat-soluble vitamins ( vitamin A , vitamin D , vitamin E and vitamin K ) are given. Linoleic acid supplements are also recommended.

To avoid intestinal symptoms, persons with this syndrome should not eat long-chain triglycerides and limit fat intake to 5 - 20 grams per day. The diet should contain no more than 5 daily ounces of lean meat, fish, or poultry. 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 doctor or nutritionist. They should be used with caution because they may cause liver damage.

Expectations (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

Calling your health care provider:

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

References:

Rodriguez-Oquendo A, Kwiterovich Jr PO. Dyslipidemias. In: Fernandes J, Saudubray J-M, van den Berghe G, Walter JH, eds. Inborn metabolic diseases: diagnosis and treatment. Germany: Springer; 2006:400-401.

• Review Date: 6/24/2007
• Reviewed By: Chad Haldeman-Englert, MD, Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed HealthcareNetwork.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process . A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 1997-2007 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.