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$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.