$Unique_ID{BRK03842} $Pretitle{} $Title{Hyperlipoproteinemia, Type IV} $Subject{Hyperlipoproteinemia, Type IV Carbohydrate-Induced Hyperlipemia Hyperprebeta-Lipoproteinemia Hypercholesterolemia Hyperlipidemia IV Hypertriglyceridemia, Endogenous Broad Beta Disease (Hyperlipoproteinemia, Type III) } $Volume{} $Log{} Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc. 624: Hyperlipoproteinemia, Type IV ** IMPORTANT ** It is possible that the main title of this article (Hyperlipoproteinemia, Type IV) is not the name you expected. Please check the SYNONYM list to find the alternate names and disorder subdivisions covered by this article. Synonyms Carbohydrate-Induced Hyperlipemia Hyperprebeta-Lipoproteinemia Hypercholesterolemia Hyperlipidemia IV Hypertriglyceridemia, Endogenous Information on the following disorder can be found in the Related Disorders section of this report: Broad Beta Disease (Hyperlipoproteinemia, Type III) General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your physician and/or the agencies listed in the "Resources" section of this report. Hyperlipoproteinemia, Type IV, is an inherited metabolic disorder. It is characterized by an increased blood level of the fats called triglycerides which are contained in very low-density lipids (VLDL). An abnormally high level of triglycerides or cholesterol can result from diet, from genetic causes, or it may occur secondary to other metabolic disorders or as a side effect of certain drugs. Symptoms Hyperlipoproteinemia, Type IV, is characterized by an abnormally high level of cholesterol or triglycerides in the blood. Glucose (sugar) tolerance is usually also impaired. Eruptive fatty nodules or plaques in the skin (xanthomas) may develop on the buttocks, arms or legs. Hyperlipoproteinemia usually accelerates fatty degeneration of blood vessels, and coronary heart disease frequently develops. The liver and spleen may be enlarged (hepatosplenomegaly). Causes Hyperlipoproteinemia, Type IV, is an inherited disorder transmitted through autosomal dominant genes. (Human traits, including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed "dominating the other normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child.) Onset of Hyperlipoproteinemia, Type IV, can be influenced by environmental factors such as too much carbohydrate in the diet or by excessive alcohol consumption. Other conditions that can cause Hyperlipoproteinemia IV are uremia, malfunction of the pituitary gland (hypopituitarism), contraceptive steroid drugs and other lipid storage diseases. Affected Population Hyperlipoproteinemia, Type IV, is a common disorder affecting males and females in equal numbers. Related Disorders Symptoms of the following disorder can be similar to those of Hyperlipoproteinemia, Type IV. Comparisons may be useful for a differential diagnosis: Broad Beta Disease (Hyperlipoproteinemia, Type III) is a hereditary disorder of fat (lipid) transport characterized by areas of lipid deposits (xanthomas) under certain parts of the skin. Patients with this disorder have a predisposition to obesity and fatty degeneration of blood vessels (atherosclerosis) which leads to blockage of blood vessels. With control of dietary lipids, persons affected with Broad Beta Disease can lead a normal life. (For more information, choose "Broad Beta" as your search term in the Rare Disease Database.) Therapies: Standard Treatment for Hyperlipoproteinemia, Type IV, consists in following a diet reduced in saturated fats, cholesterol, and calories. Drugs may also be required to lower cholesterol levels when diet alone is not effective. Therapies: Investigational This disease entry is based upon medical information available through December 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Hyperlipoproteinemia, Type IV, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Heart, Blood & Lung Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 American Heart Association 7320 Greenville Ave. Dallas, TX 75231 (214) 750-5300 For genetic information and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References DIETARY THERAPY FOR DIFFERENT FORMS OF HYPERLIPOPROTEINEMIA: S.M. Grundy; Circulation (September 1987: issue 76(3)). Pp. 523-528. GEMFIBROZIL THERAPY IN PRIMARY HYPERTRIGLYCERIDEMIA ASSOCIATED WITH CORONARY HEART DISEASE. EFFECTS ON METABOLISM OF LOW-DENSITY LIPOPROTEINS: G.L. Vega, et al.; Journal Amer Med Assoc (April 26, 1985: issue 253(16)). Pp. 2398-2403. DIFFERENT PATTERNS OF POSTPRANDIAL LIPOPROTEIN METABOLISM IN NORMAL, TYPE IIA, TYPE III, AND TYPE IV HYPERLIPOPROTEINEMIC INDIVIDUALS. EFFECTS OF TREATMENT WITH CHOLESTYRAMINE AND GEMFIBROZIL: M.S. Weintraub, et al.; Journal Clin Invest (April 1987: issue 79(4)). Pp. 1110-1119.