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Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is the most frequent congenital disorder of mitochondrial fatty acid oxidation (1 out of every 15,000 births: Orphanet 2018 and 2014, BIMDG 2015, Hsu 2008). MCAD protein is an enzyme that enables the use of fatty acids to produce energy during periods of prolonged fasting or physiological stress (infections, diseases).
Its absence or deficiency cause, in situations of fasting or stress, the recurrent appearance of severe hypoglycaemia (without increase in ketones), lethargy, vomiting, convulsions and coma that endanger life (Hegyi 1992) and neurological prognosis if an urgent medical intervention is not produced (BIMDG 2015 and 2008 OMIM, ORPHANET). It usually begins between 3 and 15 months of age, but it can manifest itself in the neonatal period (Ahrens 2016, BIMDG 2015, Hsu 2008, Derks 2006).
MCAD deficiency is included in neonatal screening programmes in many European countries (Orphanet 2018).
During the neonatal period, more than 3 hours should not pass between breastfeeding or bottle-feeding. Some authors have observed a higher risk of crisis in breastfed infants (Ahrens 2016, Wilcken 1993) but others have not (Derks 2006). In any case, neonatal hypoglycaemia should be prevented and treated with frequent feeds, supplemented with expressed breast milk, milk from a milk bank, milk formula or perfusion of intravenous glucose solution according to the clinical situation (BIMDG 2015).
Most experts and clinical guidelines agree that it is compatible with breastfeeding (Dixon 2009, Roe 1986).
Specialized clinical-dietary monitoring is required. Dietary treatment is aimed at strictly avoiding fasting with safe time intervals between feeds for infants and young children, so frequent breastfeeding is desirable.
Apart from avoiding medium chain triglycerides, no other special dietary restriction is required (Orphanet 2018, Matern 2015, BIMDG 2015, Dixon 2009).