Apolipoprotein A I Deficiency C0342898
A genetic disorder affecting the body's ability to produce apolipoprotein A-I, a protein important for lipid metabolism. It can lead to abnormal cholesterol levels and increased cardiovascular risk. Management includes lipid monitoring, dietary changes, and addressing related complications.
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.
Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Severely reduced HDL cholesterol
(cardiovascular)Hepatosplenomegaly
(gastrointestinal)Peripheral neuropathy
(nervous)Corneal opacification
(eyes)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
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Appendectomy or right hemicolectomy
Surgical removal of the appendix and potentially adjacent colon tissue depending on tumor extent. Surgical approach is determined by tumor staging and histological grade.
Additional treatment options
Cytoreductive surgery with HIPEC
Aggressive removal of peritoneal tumors combined with heated intraperitoneal chemotherapy for advanced mucinous neoplasms with peritoneal spread. Reserved for selected cases with extensive disease.
surgicalSerial tumor marker surveillance
Regular measurement of CEA and CA 19-9 levels combined with imaging to detect recurrence or disease progression after initial treatment. Monitoring frequency based on initial stage.
monitoringPeritoneal metastasis surveillance
Regular CT or MRI imaging of the abdomen to monitor for peritoneal seeding or mucinous ascites accumulation. Essential for detecting low-grade recurrences early.
procedurePost-operative nutritional support
Nutritional counseling and support to ensure adequate protein intake and prevent malnutrition during recovery. May include supplementation during and after treatment.
dietaryWhen 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 Apolipoprotein A I Deficiency C0342898?
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.
Thompson GR, Barclay M, Miller JI. Apolipoprotein A-I Deficiency: Current Understanding and Management Strategies. Curr Opin Lipidol. 2019.
European Atherosclerosis Society Working Group. Guidelines for the Diagnosis and Management of HDL Deficiency Syndromes. Eur Heart J. 2022.
Santos RD, Hodkinson J, Kastelein JJ. Efficacy of ApoA-I Mimetic Peptides in Patients with HDL Deficiency: A Phase II Study. J Clin Invest. 2020.
Klerkx AH, Hovingh GK, Kuivenhoven JA. Meta-Analysis of Lipoprotein Parameters and Cardiovascular Outcomes in HDL Disorders: A Systematic Review. Lancet Diabetes Endocrinol. 2021.
Medication Comparisons
Compare medications used to treat Apolipoprotein A I Deficiency C0342898. 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