Acute bilirubin encephalopathy

Orpha code: 529799OMIM code:

Definition

A rare neurologic disease characterized by lethargy, hypotonia, poor feeding, opisthotonus, and a typical high-pitched cry due to bilirubin accumulation in the globus pallidus, sub-thalamic nuclei, and other brain regions, resulting from severe neonatal unconjugated hyperbilirubinemia. Onset of symptoms is typically within the first three to five days of life. Additional features include fever, apnea, seizures, and coma. Especially respiratory failure or refractory seizures may lead to a fatal outcome.

Disease data
Classification

Clinical syndrome

Synonyms
ABE
Ostra żółtaczka jąder podstawy mózgu
Acute kernicterus
ORPHA code
529799
OMIM code
-
ICD10 code
P57.8
ICD11 code
-

No additional description.

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