Hyperlipoproteinemia Type I C0023817
Also called familial chylomicronemia syndrome, this prevents the body from breaking down dietary fats properly. It causes very high triglycerides, abdominal pain, and risk of pancreatitis. Treatment involves a very low-fat diet.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Recurrent pancreatitis
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lipemia retinalis
(ophthalmic)Eruptive xanthomas
(dermatologic)Hepatomegaly
(hepatobiliary)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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Extremely low-fat diet
A very restrictive low-fat diet is the primary treatment, limiting fat intake to less than 10-15% of total calories. This prevents accumulation of chylomicrons that cause the characteristic symptoms.
Fibrate medications
Fibrates enhance lipoprotein lipase activity to help clear triglycerides from the blood. They serve as an adjunct to dietary management in controlling lipid levels.
Additional treatment options
Triglyceride level surveillance
Regular monitoring of fasting triglyceride levels helps track disease control and response to treatment. Levels above 1000 mg/dL indicate poor control and increased pancreatitis risk.
monitoringOmega-3 fatty acid supplementation
Marine-derived omega-3 fatty acids may provide modest triglyceride-lowering effects as a complementary approach to dietary management. They should not replace primary dietary restrictions.
complementaryPlasmapheresis for acute hypertriglyceridemia
Emergency plasmapheresis rapidly lowers extremely elevated triglycerides when acute pancreatitis is imminent. This intervention can be life-saving in crisis situations.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Hyperlipoproteinemia Type I C0023817?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Hyperlipoproteinemia Type I C0023817. 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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy