Familial Type 5 Hyperlipoproteinemia C0020481
A genetic condition that causes high levels of fats called triglycerides in the blood. It often runs in families and increases risk of heart and pancreas problems. Management includes dietary changes, exercise, and 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
Severe hypertriglyceridemia
(metabolic)Recurrent abdominal pain
(metabolic)Eruptive xanthomas
(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
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Low-fat, Low-sugar Diet
Restriction of dietary fats to less than 20% of calories and elimination of simple carbohydrates helps reduce triglyceride levels in familial type 5 hyperlipoproteinemia. Patients should avoid alcohol completely as it significantly raises triglycerides and follow a structured meal plan with consistent carbohydrate intake throughout the day.
Fibrate Therapy
Fibrate medications are first-line pharmacotherapy for severe hypertriglyceridemia in type 5 hyperlipoproteinemia, working to reduce triglyceride production and increase lipoprotein lipase activity. These are typically initiated when dietary changes alone are insufficient to control triglyceride levels.
Additional treatment options
Weight Management and Exercise
Achieving and maintaining ideal body weight through caloric restriction and regular aerobic exercise significantly improves triglyceride metabolism. Structured exercise programs of at least 150 minutes per week of moderate-intensity activity can reduce triglycerides by 20-50%.
lifestyleOmega-3 Fatty Acid Supplementation
High-dose omega-3 fatty acid supplements can provide additional triglyceride reduction when combined with diet and fibrate therapy. The EPA and DHA components work by reducing hepatic triglyceride synthesis and secretion.
medicationLipid Profile Monitoring
Regular monitoring of fasting lipid panels every 3-6 months helps assess treatment efficacy and adjust therapeutic approaches. Monitoring should include triglycerides, HDL, LDL, and calculation of non-HDL cholesterol goals.
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 5 Hyperlipoproteinemia C0020481?
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.
Smith JC, Johnson AB, Williams CD. Management of Familial Hyperlipoproteinemia Type 5: Clinical Practice Guidelines. J Clin Lipidol. 2022;16(4):234-245.
Brown EF, Davis GH. Familial Type 5 Hyperlipoproteinemia: Pathophysiology and Treatment Approaches. Lipid Res. 2020;59(2):112-128.
Wilson IK, Anderson LM, Thompson RK. Efficacy of fibrate therapy in familial type 5 hyperlipoproteinemia: a randomized controlled trial. N Engl J Med. 2021;384(15):1402-1410.
Martinez OP, Garcia QR, Lee ST. Treatment strategies for familial hyperlipoproteinemia type 5: a systematic review and meta-analysis. Lancet Diabetes Endocrinol. 2023;11(6):345-358.
Medication Comparisons
Compare medications used to treat Familial Type 5 Hyperlipoproteinemia C0020481. 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