Adverse Drug Reaction Classification System

ADR Ontology
ADR Term Fabry's disease
ADR ID BADD_A01596
ADR Hierarchy
03      Congenital, familial and genetic disorders
03.08      Metabolic and nutritional disorders congenital
03.08.02      Lysosomal storage disorders
03.08.02.001      Fabry's disease
14      Metabolism and nutrition disorders
14.14      Inborn errors of metabolism
14.14.02      Lysosomal storage disorders
14.14.02.001      Fabry's disease
17      Nervous system disorders
17.02      Neurological disorders NEC
17.02.06      Paraesthesias and dysaesthesias
17.02.06.013      Fabry's disease
23      Skin and subcutaneous tissue disorders
23.06      Skin vascular abnormalities
23.06.03      Telangiectasia and related conditions
23.06.03.002      Fabry's disease
Description An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders. [MeSH]
MedDRA Code 10016016
MeSH ID D000795; D000795; D000795
ADR Severity Grade (FAERS)
ADR Severity Grade (CTCAE) Not Available
Synonym
Angiokeratoma corporis diffusum universale | Fabry's disease | Fabry crisis | Alpha galactosidase A deficiency | Fabry Disease | alpha-Galactosidase A Deficiency Disease | alpha Galactosidase A Deficiency Disease | Anderson-Fabry Disease | Anderson Fabry Disease | Angiokeratoma Corporis Diffusum | Angiokeratoma Diffuse | Ceramide Trihexosidase Deficiency | Deficiency, Ceramide Trihexosidase | Fabry's Disease | GLA Deficiency | Deficiency, GLA | Hereditary Dystopic Lipidosis | Lipidosis, Hereditary Dystopic | alpha-Galactosidase A Deficiency | Deficiency, alpha-Galactosidase A | alpha Galactosidase A Deficiency | Angiokeratoma, Diffuse | Diffuse Angiokeratoma
ADR Related Proteins
Protein Name UniProt AC TTD Target ID
Not AvailableNot AvailableNot Available
Drugs Leading to the ADR
Drug IDDrug NameADR Frequency (FAERS)ADR Severity Grade (FAERS)
BADD_D00049Agalsidase beta--
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