Mitochondrial Complex Iii Deficiency Nuclear Type 7 C4014408
A rare inherited condition affecting the mitochondria's energy-producing complexes. Primarily seen in children with variable organ involvement. Treatment centers on supportive measures.
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
Lactic acidosis
Seizures
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Encephalomyopathy
(neurological)Cardiomyopathy
(cardiovascular)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
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Multisystem Surveillance Protocol
Systematic monitoring of affected organ systems including brain imaging, echocardiography, and metabolic testing to identify and address complications promptly.
Ketogenic Diet Consideration
Controlled trial of ketogenic diet in appropriate candidates, as ketone bodies may provide alternative energy substrate for neurons and reduce metabolic stress.
Additional treatment options
Lactic Acidosis Management
Use of sodium bicarbonate or other alkali agents to manage metabolic acidosis, along with dichloroacetate to reduce lactate production.
medicationNeuropsychological Support Services
Cognitive evaluation and support including memory aids, behavioral strategies, and family education to address cognitive decline and neuropsychiatric symptoms.
psychologicalEnergy Conservation Techniques
Teaching of pacing strategies and energy conservation methods to help patients manage fatigue and optimize functional capacity throughout the day.
lifestyleWhen 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 Mitochondrial Complex Iii Deficiency Nuclear Type 7 C4014408?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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