Acute Infantile Liver Failure Due To Synthesis Defect Of Mtdna Encoded Proteins C3278664
A rare genetic condition causing sudden liver failure in infants due to defective protein synthesis. Requires specialized medical care and can be life-threatening.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- critical
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Jaundice
Coagulopathy
Hypoglycemia
Lethargy
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
Liver transplantation
Definitive surgical treatment involving replacement of the failing liver with a donor organ. This is considered in infants with progressive liver failure due to mitochondrial dysfunction when other supportive measures fail.
Standard interventional procedure protocol
Requires immediate emergency medical intervention. Do not delay treatment.
ICU supportive care and continuous monitoring
Intensive care unit admission with close monitoring of vital signs, liver function tests, coagulation parameters, and neurological status. Includes IV fluid management and temperature regulation.
Additional treatment options
Coenzyme Q10 supplementation
Mitochondrial cofactor supplementation to support electron transport chain function. Often used as supportive therapy in mitochondrial disorders affecting energy metabolism.
medicationSpecialized nutritional support
Parenteral or enteral nutrition tailored to meet metabolic demands while reducing hepatic workload. May include medium-chain triglyceride supplementation and restriction of long-chain fatty acids.
dietaryExchange transfusion
Procedural intervention to remove toxins and replace deficient clotting factors in acute liver failure. Used as a bridge to more definitive treatment in severe cases.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Acute Infantile Liver Failure Due To Synthesis Defect Of Mtdna Encoded Proteins C3278664?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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 20, 2026. · Read our editorial policy