Biliary Malformation With Renal Tubular Insufficiency C0400972
A rare disorder affecting both bile ducts and kidney tubules, usually diagnosed in infancy. Management requires coordinated care for liver and kidney problems.
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.
Cholestatic jaundice
(hepatobiliary)Generalized symptoms
(hepatobiliary)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Renal tubular acidosis
(renal)Failure to thrive
(constitutional)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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Regular hepatic and renal function surveillance
Scheduled monitoring of liver enzymes, bilirubin levels, and renal tubular function parameters including electrolytes and urinalysis to track disease progression and detect complications early.
Fat-soluble vitamin supplementation
Supplemental vitamins A, D, E, and K to compensate for fat malabsorption resulting from biliary dysfunction and to support overall nutritional status.
Additional treatment options
Ursodeoxycholic acid therapy
Bile acid replacement therapy to improve bile flow, reduce liver inflammation, and support fat-soluble vitamin absorption in patients with biliary malformations.
medicationMedium-chain triglyceride diet
Dietary modification using MCT oils which are directly absorbed without requiring bile salts, helping maintain caloric intake and nutritional status.
dietaryEndoscopic intervention for biliary drainage
Minimally invasive endoscopic procedures to relieve biliary obstruction, stent placement when anatomically feasible, improving bile flow and reducing cholestasis complications.
procedureWhen 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 Biliary Malformation With Renal Tubular Insufficiency C0400972?
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 20, 2026. · Read our editorial policy