Cerebrotendinous xanthomatosis

Orpha code: 909OMIM code: 213700

Definition

Cerebrotendinous xanthomatosis (CTX) is an anomaly of bile acid synthesis (see this term) characterized by neonatal cholestasis, childhood-onset cataract, adolescent to young adult-onset tendon xanthomata, and brain xanthomata with adult-onset neurologic dysfunction.

Disease data
Classification

Disease

Synonyms
CTX
CTX
Niedobór 27-hydroksylazy sterolowej
Sterol 27-hydroxylase deficiency
ORPHA code
909
OMIM code
213700
ICD10 code
E75.5
ICD11 code
5C52.11

No additional description.

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