Hyperlipoproteinemia Type 1d C4014767
A rare genetic disorder causing severely elevated triglycerides due to impaired fat clearance from the blood. It increases risk of pancreatitis and requires a strict fat-restricted 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
Abdominal pain
Eruptive xanthomas
(dermatologic)Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
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
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Very low-fat diet
Strict restriction of dietary fat intake is the cornerstone of managing type 1d hyperlipoproteinemia. This approach reduces the accumulation of chylomicrons and prevents severe hypertriglyceridemia.
Fibrate therapy
Fibrate medications help lower triglyceride levels by activating lipoprotein lipase, an enzyme that breaks down triglycerides. They are used when dietary management alone is insufficient.
Additional treatment options
Alcohol and sugar avoidance
Complete avoidance of alcohol and simple sugars is essential as both can dramatically increase triglyceride levels. Patient education on reading food labels and identifying hidden sources of these substances is important.
lifestyleRegular lipid panel monitoring
Frequent blood tests to monitor triglyceride levels help assess treatment effectiveness and guide dietary and medication adjustments. Monitoring frequency may decrease once levels are stable.
monitoringTherapeutic plasmapheresis
In severe acute cases with extremely high triglyceride levels posing risk of pancreatitis, plasmapheresis can rapidly remove lipoproteins from the blood. This is typically reserved for emergency 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 1d C4014767?
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 1d C4014767. 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 27, 2026. · Read our editorial policy