Chronic bilirubin encephalopathy

Orpha code: 529808OMIM code:

Definition

A rare neurologic disease characterized by the chronic consequences of bilirubin toxicity in the globus pallidus, sub-thalamic nuclei, and other brain regions, after exposure to high levels of unconjugated bilirubin in the neonatal period. Symptoms begin after the acute phase of bilirubin encephalopathy in the first year of life, evolve slowly over several years, and include mild to severe extrapyramidal disturbances (especially dystonia and athetosis), auditory neuropathy spectrum disorder, and oculomotor and dental abnormalities.

Disease data
Classification

Clinical syndrome

Synonyms
BIND
Bilirubin-induced neurological dysfunction
CBE
KSD
Kernicterus spectrum disorder
BIND
Bilirubin-induced neurological dysfunction
CBE
KSD
Kernicterus spectrum disorder
ORPHA code
529808
OMIM code
-
ICD10 code
P57.8
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl