Ketoacidosis Due To Monocarboxylate Transporter 1 Deficiency C4015186
A rare genetic condition where the body cannot properly use energy molecules, leading to acid buildup. Affected individuals need lifelong dietary management and close medical supervision.
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 ketoacidotic episodes
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hypoglycemia
(metabolic)Exercise intolerance
(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
Ketogenic diet management
Carefully planned ketogenic diet to provide alternative energy sources given impaired ketone body transport. Requires dietitian coordination and regular metabolic monitoring.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Glucose supplementation during metabolic stress
Increased carbohydrate intake or IV glucose during illness, fasting, or periods of increased metabolic demand to prevent decompensation.
Additional treatment options
Metabolic monitoring and crisis prevention
Regular monitoring of metabolic parameters and development of sick day protocols to prevent metabolic decompensation episodes.
monitoringAvoidance of prolonged fasting
Structured eating schedule with regular meals and snacks to prevent extended periods without carbohydrate intake. Education of family and caregivers on feeding schedules.
lifestyleCoQ10 and mitochondrial support supplementation
Coenzyme Q10 supplementation may support mitochondrial function in some patients with MCT1 deficiency. Evidence is emerging and should be discussed with metabolic specialist.
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 Ketoacidosis Due To Monocarboxylate Transporter 1 Deficiency C4015186?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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