Familial dysbetalipoproteinemia: a potentially fatal disorder

Familial dysbetalipoproteinemia is an inherited disorder in which both cholesterol and triglycerides are elevated in the plasma of the blood, which pre-disposes people to coronary artery disease and peripheral vascular disease. We report two young boys with multiple cutaneous xanthomas and grossly abnormal serum cholesterol and triglycerides. Two of the family members had died of cardiovascular accidents in young age and rest of the family members had deranged lipid profile. Patients were managed with lipid lowering drugs and fat restriction diet. All family members were counseled and advised regular exercise and follow-up