Since the introduction of neonatal screening for branched-chain organic acidurias, it has been noted that isolated 3-methylcrotonyl-CoA carboxylase deficiency is probably one of the most frequent organic acidurias. Only a few cases with severe clinical presentation have been described. Profound hypoglycaemia is an uncommon but life-threatening complication.,
Journal of Inherited Metabolic Disease
Department of Pediatrics

Oude Luttikhuis, H. G. M., Touati, G., Rabier, D., Williams, M., Jakobs, C., & Saudubray, J. (2005). Severe hypoglycaemia in isolated 3-methylcrotonyl-CoA carboxylase deficiency; a rare, severe clinical presentation. Journal of Inherited Metabolic Disease, 28(6), 1136–1138. doi:10.1007/s10545-005-4545-1