Disorder Of Ketone Body Transport C5681184
A metabolic disorder affecting how ketone bodies are transported in the body, potentially causing energy problems and low blood sugar. Management includes dietary changes and monitoring.
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
Ketoacidosis
Hyperammonemia
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Neurodevelopmental delay
(metabolic)Growth retardation
(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
Controlled Carbohydrate Diet
Carefully managed dietary approach limiting excessive ketone body production while maintaining adequate glucose availability through consistent carbohydrate intake throughout the day.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Metabolic Stress Avoidance
Avoidance of prolonged fasting, strenuous exercise, and catabolic stress through careful planning of activity and nutrition to prevent ketone body accumulation crises.
Additional treatment options
Glucose Loading During Crises
Oral or intravenous glucose administration to suppress ketogenesis and provide alternative fuel during metabolic stress, preventing dangerous ketone body accumulation.
medicationKetone Body Level Monitoring
Regular blood or urine testing to monitor ketone body levels, particularly during illness or times of metabolic stress, allowing for early intervention when levels rise.
monitoringMetabolic Disorder Support Supplements
Nutritional supplementation including B-complex vitamins, magnesium, and other cofactors that support mitochondrial function and may help optimize ketone body metabolism.
complementaryWhen 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 Disorder Of Ketone Body Transport C5681184?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Disorder Of Ketone Body Transport C5681184. 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