Congenital dicarboxylic acidurias

1 Glutaric aciduria type IF neonatal multiple acyl-CoA dehydrogenase deficiency 

This report was followed by the description of a second patient by Goodman et al. (1980). This patient (male), whose female sibling died at 4 days of age with Reye s syndrome , presented with neonatal acidosis and hypotonia associated 

Concentration (mmol per g of creatinine) (in four random urines)  

Long-chain fatty acids Medium-chain fatty acids 

The dissimilarity of these patients from those with apparent multiple acyl-CoA dehydrogenase deficiency (Section 14.2.1) and other dicarboxylic acidurias is noticeable and the primary defect remains unknown. 

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Dicarboxylic aciduria (congenital medium-chain-length dicarboxylic aciduria) ... 

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