Fabry disease

Orpha code: 324OMIM code: 301500

Definition

A rare genetic, multisystemic lysosomal disease characterized by specific cutaneous (angiokeratoma), neurological (pain), renal (proteinuria, chronic kidney failure), cardiovascular (cardiomyopathy, arrhythmia), cochleo-vestibular and cerebrovascular manifestations (transient ischemic attacks, strokes). The phenotypic expression depends on age of onset and, in females, the level of X-inactivation.

Disease data
Classification

Disease

Synonyms
Alpha-galactosidase A deficiency
Choroba Andersona i Fabry'ego
FD
Niedobór alfa-galaktozydazy A
Rogowiec naczyniowy rozlany
Rozsiane zmiany o charakterze angiokeratoma
Anderson-Fabry disease
Angiokeratoma corporis diffusum
Diffuse angiokeratoma
FD
ORPHA code
324
OMIM code
301500
ICD10 code
E75.2
ICD11 code
5C56.01

No additional description.

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