Aciduria: 3-methylglutaconic type 5
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(AGM type 5, DCMA syndrome)
Very rare. Form of 3-methylglutaconic aciduria due to a mutation of the DNAJC19 gene (3q16.33).
Characterized by:
- a dilated cardiomyopathy
- cerebellar ataxia
- growth retardation
- testicular dysgenesis
Anesthetic implications:
go to mitochondrial cytopathies ; preoperative echocardiography
References:
Updated: December 2019