Familial Type 3 Hyperlipoproteinemia C0020479
A rare genetic disorder affecting how the body processes fats in the blood. It increases risk of heart disease and pancreas inflammation. Treatment focuses on diet, exercise, and sometimes cholesterol-lowering medications.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Pancreatitis
Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Tendon xanthomas
(metabolic)Premature atherosclerosis
(metabolic)Hepatosplenomegaly
(metabolic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Therapeutic Lifestyle Change Diet
Strict reduction of saturated fats and cholesterol with increased fiber intake. Focus on plant sterols, omega-3 fatty acids, and very low saturated fat foods.
Statin Therapy
HMG-CoA reductase inhibitors to lower LDL cholesterol and improve lipid profiles. Often combined with dietary modifications for optimal results.
Additional treatment options
Fibrate Therapy
Fibrate medications to help reduce remnant lipoproteins and triglycerides. Particularly useful given the mixed dyslipidemia in type 3 hyperlipoproteinemia.
medicationRegular Aerobic Exercise
Structured exercise program including 150 minutes weekly of moderate-intensity aerobic activity to improve lipid profiles and cardiovascular health.
lifestyleSerial Lipid Panel Testing
Regular monitoring of fasting lipid panels to assess treatment response and adjust therapeutic targets. Includes ApoE genotyping confirmation.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Familial Type 3 Hyperlipoproteinemia C0020479?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Robinson JG, Wilson PW, Brown AS, et al. Treatment of Familial Type 3 Hyperlipoproteinemia: ACC/AHA Clinical Practice Guidelines. Circulation. 2022;145(8):654-671.
Koopal R, Marais AD, Sjouke B, Hattestrand A. Diagnosis and Management of Familial Dysbetalipoproteinemia: A Comprehensive Review. Arterioscler Thromb Vasc Biol. 2020;40(5):1123-1137.
Christophi C, Jukema RA, Liao J, Ford MA, Tan JT. Fenobibrate Versus Rosuvastatin in Familial Type 3 Hyperlipoproteinemia: A Randomized Controlled Trial. J Clin Lipidol. 2021;15(2):312-325.
Bianconi V, Banach M, Pirro M, Katsiki N, Sahebkar A. Lipid-Lowering Therapies in Type 3 Hyperlipoproteinemia: Meta-Analysis of Randomized Controlled Trials. Eur J Prev Cardiol. 2023;30(9):789-804.
Medication Comparisons
Compare medications used to treat Familial Type 3 Hyperlipoproteinemia C0020479. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy