Jaundice Familial Obstructive Of Infancy C1839927
A rare liver condition in newborns where bile ducts are blocked, causing jaundice and liver damage. Surgery or liver transplant may be needed.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Failure to thrive
Generalized symptoms
(hepatobiliary)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Neonatal jaundice
(hepatobiliary)Dark urine
(hepatobiliary)Pale stools
(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
Hepatoportoenterostomy (Kasai procedure)
Surgical bypass procedure to restore bile flow from the liver to the intestine by connecting the hepatic porta to a loop of intestine, most effective when performed before 60 days of age.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Choleretic and supportive therapy
Medical management including ursodeoxycholic acid to promote bile flow, fat-soluble vitamin supplementation, and management of cholestasis symptoms.
Additional treatment options
Specialized nutritional support
Nutritional management with medium-chain triglyceride-enriched formulas to improve fat absorption, address malabsorption, and support normal growth and development.
dietaryLiver function and growth monitoring
Regular assessment of liver function tests, bilirubin levels, nutritional status, and growth parameters to evaluate disease progression and treatment response.
monitoringLiver transplant evaluation
Assessment for liver transplantation candidacy in cases of progressive liver failure, failed Kasai procedure, or complications of portal hypertension.
surgicalWhen 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 Jaundice Familial Obstructive Of Infancy C1839927?
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 20, 2026. · Read our editorial policy