isovaleric acidemia

GPTKB entity

Statements (22)
Predicate Object
gptkbp:instanceOf gptkb:metabolic_disorder
gptkb:rare_disease
gptkbp:affects amino acid metabolism
gptkbp:causedBy deficiency of isovaleryl-CoA dehydrogenase
gptkbp:diagnosedBy genetic testing
urine organic acid analysis
gptkbp:firstDescribed 1966
gptkbp:ICD-10_code E71.110
gptkbp:inheritsFrom autosomal recessive
gptkbp:OMIM 243500
gptkbp:prevalence rare
gptkbp:symptom vomiting
seizures
lethargy
developmental delay
distinctive odor of sweaty feet
gptkbp:treatment carnitine supplementation
dietary protein restriction
glycine supplementation
gptkbp:bfsParent gptkb:isovaleric_acid
gptkbp:bfsLayer 7
https://www.w3.org/2000/01/rdf-schema#label isovaleric acidemia