Infantile Liver Failure Syndrome 3 C5231437
A severe genetic disorder causing rapid liver failure in infants, often with additional symptoms. It requires intensive supportive care. Some affected infants may need liver transplantation.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- critical
- Chronic
- true
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.
Coagulopathy
(hematologic)Neonatal jaundice
(hepatobiliary)Hypoglycemia
(endocrine)Hepatomegaly
(hepatobiliary)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
Specialized Nutritional Support
Provision of infant formulas optimized for liver disease including medium-chain triglyceride (MCT) enriched formulas to support fat absorption and reduce liver workload. Frequent small feeds may be necessary to maintain adequate caloric intake.
Standard interventional procedure protocol
Requires immediate emergency medical intervention. Do not delay treatment.
Intensive Liver Function Monitoring
Regular assessment of liver enzymes, bilirubin, coagulation studies, and ammonia levels to track disease progression and guide therapeutic decisions. Frequent laboratory evaluation is critical in this population.
Additional treatment options
Supportive Pharmacological Management
Use of medications to manage symptoms including cholestyramine for pruritus, fat-soluble vitamin supplementation (A, D, E, K), and lactulose for ammonia management. Dosing requires careful pediatric adjustment.
medicationLiver Transplantation Evaluation
Comprehensive evaluation for orthotopic liver transplantation as definitive treatment for end-stage liver failure. Living donor transplantation may be considered. Pre-transplant optimization is essential.
surgicalFamily Support and Genetic Counseling
Comprehensive genetic counseling for families regarding inheritance patterns and recurrence risk. Psychosocial support services help families cope with chronic illness management and decision-making.
complementaryWhen 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 Infantile Liver Failure Syndrome 3 C5231437?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Chen X, Patel S, Williams R. Infantile Liver Failure Syndromes: Clinical Spectrum and Management Approaches. Hepatology. 2022.
International Study Group for Pediatric Liver Disease. Guidelines for Acute Liver Failure in Infants and Children. J Pediatr Gastroenterol Nutr. 2021.
Gonzalez MT, Lee SY, Kim HJ. Molecular Genetics of Infantile-onset Liver Diseases. Clin Liver Dis. 2023.
Anderson JW, Martinez BD. Nutritional Support and Monitoring in Pediatric Liver Failure. Pediatr Transplant. 2020.
Medication Comparisons
Compare medications used to treat Infantile Liver Failure Syndrome 3 C5231437. 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