Genetics Northern California

Familial Hypoalphalipoproteinemia

Familial hypoalphalipoproteinemia (HA) is a relatively common genetic condition that causes the body to produce low levels of HDL or high-density lipoprotein (known as “good” cholesterol). Low HDL can increase the risk of developing cardiovascular disease, including an added risk for heart attack and stroke.

High-density lipoprotein (HDL)
HDL is made by the liver and the intestines. It is often called “good” cholesterol because higher levels help lower the risk for cardiovascular disease. HDL moves cholesterol to the liver so it can be removed from the body. HDL also helps the body by reducing inflammation and protecting the walls of the blood vessels from normal wear and tear.
Lifestyle factors such as smoking, being overweight, and not exercising can all lower HDL levels. However, in familial HA, low levels of HDL can be due to gene changes that affect the production and breakdown of HDL. Familial HDL deficiency is generally inherited in a dominant manner, meaning that there is a 50% chance for a parent to pass on the condition to any child.

Diagnosis
A diagnosis of HA is made by measuring the amount of HDL in the blood.
Reference Range-HDL Cholesterol.gif

REC05d019Management
Improving HDL levels mainly focuses on lifestyle changes but can sometimes include medications: Low HDL? Here's What You Can Do
Here are some lifestyle strategies that can help improve HDL levels:
    • Focus on healthy fats – see: How Fats Compare
        o Avoid trans fatty acids -  Click here to learn more about trans fats (American Heart Association website)
        o Substitute monounsaturated fats for saturated and trans fats
    • Reduce refined carbohydrate foods (like pasta, white rice, crackers, bread, and noodles)
    •  Avoid the use of alcohol and cigarettes
    •  Exercise regularly, especially aerobic exercise
    •  Weight loss, especially weight around the waist


Medications that can help to increase HDL levels: 
    •  Niacin
    •  Statins (for example: Lipitor, Crestor, Zocor) can increase or decrease HDL. Individual response may vary, so blood tests are recommended.

The Lipid Program providers carefully evaluate whether medication may be beneficial. If medications are indicated, the staff will make recommendations as to the appropriate age to start and how ongoing treatment should be managed. The Program specialists work with parents and other family members to optimize their care and adjust lifestyle choices for the benefit of the whole family.

Created by: Suzanne Kordesh, MPH, RD, Pediatric Lipid Clinic Coordinator 
Reviewed by: Dr. Leslie Manace Brenman, MD MPhil, Sub-Chief, Kaiser Oakland Genetics 

Last Updated: January 2016