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Combined Oxidative Phosphorylation Defect Type 4 C1857682

Published: July 22, 2026Updated: August 20, 2026

A rare mitochondrial disorder affecting the body's energy production at the cellular level. Can cause problems with muscles, nerves, brain, and heart. Treatment focuses on managing specific symptoms and supportive care.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

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.

Fatigue or weight changes

(metabolic)

Significant symptoms that substantially affect daily functioning.

Frequency: Common at all stages

Lactic acidosis

(metabolic)

Muscle weakness

(metabolic)

Cardiomyopathy

(metabolic)

Sensorineural hearing loss

(metabolic)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

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.]

Medication Comparisons

Compare medications used to treat Combined Oxidative Phosphorylation Defect Type 4 C1857682. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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