Dysmyelination With Jaundice C1857143
A neurological condition affecting myelin development along with liver dysfunction causing jaundice. Typically presents in infancy. Requires supportive management addressing both neurological and liver concerns.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Seizures
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Neonatal jaundice
(neurologic)Hypotonia
(neurologic)Developmental delay
(neurologic)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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Phototherapy for neonatal jaundice
Blue light therapy to convert bilirubin into water-soluble forms for excretion. Standard first-line treatment for hyperbilirubinemia in dysmyelination disorders.
Exchange transfusion
Blood exchange procedure to rapidly reduce bilirubin levels and remove circulating toxins. Reserved for severe cases or when phototherapy fails.
Additional treatment options
Specialized metabolic formula supplementation
Nutritional support with formulas designed for metabolic disorders, potentially including medium-chain triglycerides and reduced long-chain fats to support liver function.
dietarySerial bilirubin and liver function monitoring
Regular laboratory assessment of bilirubin levels and hepatic function to guide treatment intensity and detect complications early.
monitoringUrsodeoxycholic acid therapy
Bile acid supplementation to support liver function and improve bile flow. May help manage cholestasis component of the disorder.
medicationWhen 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 Dysmyelination With Jaundice C1857143?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy