Hyperlipoproteinemia type 5
Overview
An inherited disorder where the body's ability to break down lipids (triglycerides and cholesterol) is impaired resulting in increased blood lipid levels of chylomicrons and VLDL's
Symptoms
* Recurrent abdominal pain * Increased VLDL level * Increased chylomicron level * Eruptive xanthoma * Enlarged liver
Causes
* Liver cirrhosis * Alcoholic liver cirrhosis * Hemochromatosis * Liver abscess * Liver cysts (see Liver symptoms) * Hydatid cyst of the liver * Polycystic liver disease * Hepatic vein thrombosis * Budd-Chiari syndrome * Fatty liver
Treatment
The most effective treatment for type V is weight reduction and long-term maintenance of a low-fat diet. Alcoholic beverages must be avoided. Niacin, clofibrate, gemfibrozil, and a 20- to 40-g/day medium-chain triglyceride diet may prove helpful. The prognosis is uncertain because of the risk of pancreatitis. Increased fat intake may cause recurrent bouts of illness, possibly leading to pseudocyst formation, hemorrhage, and death.