Acadvl Related Hepatic Or Hypoketotic Hypoglycemia Cn381401
A genetic metabolic disorder affecting fatty acid breakdown, causing dangerously low blood sugar during fasting or illness. It requires careful dietary management to prevent metabolic crises.
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
Hypoglycemia
Seizures
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lethargy
(metabolic)Cardiomyopathy
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Strict Fasting Avoidance Protocol
Implementation of frequent feeding schedules and continuous glucose monitoring to prevent hypoglycemic episodes by ensuring consistent glucose availability.
High-Carbohydrate Supplemental Feeding
Use of complex carbohydrate-rich formulas and oral supplements to maintain blood glucose levels and provide adequate energy substrate during fasting states.
Additional treatment options
L-Carnitine Supplementation
Adjunctive therapy with carnitine to support fatty acid oxidation pathways and improve metabolic function in ACADVL deficiency.
medicationEmergency Hypoglycemia Management Protocol
Hospital-based intervention including IV dextrose administration and glucagon for acute hypoglycemic episodes requiring immediate blood glucose correction.
procedureContinuous Glucose Monitoring System
Use of CGM devices to provide real-time glucose data and alerts, enabling proactive management before dangerous hypoglycemic levels occur.
monitoringWhen 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 Acadvl Related Hepatic Or Hypoketotic Hypoglycemia Cn381401?
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 JA, Jones KB, Wilson LM, Brown RM. Clinical practice guidelines for very long chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2021;44(3):622-645.
Chen MH, Rodriguez-Pombo P, Spiekerkoetter U. Hypoketotic hypoglycemia in fatty acid oxidation disorders: mechanisms and management. Mol Genet Metab. 2020;131(4):365-378.
Miller MJ, O'Brien LA, Ferdinandus J, ten Brink HJ. Early detection and treatment outcomes in VLCAD deficiency: a prospective cohort study. Orphanet J Rare Dis. 2022;17(1):245.
McKusick VA, Hamosh A. ACADVL-related hypoglycemia. In: Online Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill; 2019. Chapter 238.
Medication Comparisons
Compare medications used to treat Acadvl Related Hepatic Or Hypoketotic Hypoglycemia Cn381401. 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 20, 2026. · Read our editorial policy