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Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is an autosomal recessive congenital disorder of mitochondrial oxidation of very long chain fatty acids. It results from autosomal recessive inheritance, with a frequency of 1 in 30,000 to 50,000 births (Leslie 2018, OMIM 2017, GARD 2015, ORPHANET 2014, Arnold 2009).
The impossibility of using Long Chain Triglycerides (LCT) to produce energy during periods of prolonged fasting or physiological stress (infections, diseases) causes low blood sugar levels (hypoglycemia, not ketotic), lack of energy and muscle weakness. Very long chain fatty acids are an important source of energy for the heart and muscles (GARD 2015).
Presentation can be early, even neonatal, with high incidence of cardiomyopathy, pericardial effusion, arrhythmias and high mortality, but many patients remain asymptomatic and are diagnosed during childhood via non-ketotic hypoglycemia crisis or in adulthood due to cardiac and skeletal muscle involvement with rhabdomyolysis and myoglobinuria following exercise or fasting (Leslie 2018, OMIM 2017, NORD 2016, GARD 2015, Pervaiz 2011, Arnold 2009, Boles 2002).
Very few patients have been observed worldwide (ORPHANET 2014, Spiekerkoetter 2009). Many neonatal screening programmes include VLCADD detection.
Breastmilk has practically the same level of medium chain triglycerides (MCT) as formulas enriched with MCT, but it has a higher concentration of LCT and less MCT than formulas highly enriched with MCT (Arnold 2009).
There is no consensus on whether breastfeeding is always possible (Potter 2012, Pervaiz 2011, Arnold 2009). Most experts recommended breastfeeding supplemented with a formula enriched with MCT in asymptomatic infants and as long as cardiac function remains normal (Arnold 2009).
Dietary treatment consists of avoiding prolonged periods of fasting through regular meals, a low-fat diet (especially LCT), and one which is rich in carbohydrates and supplemented with MCT (Leslie 2018, GARD 2015, OFPHANET 2014, Potter 2012, Pervaiz 2011, Arnold 2009), which manages to reverse cardiological symptoms (Spiekerkoetter 2003, Cox 1998, Brown 1996).
MOTHER AFFECTED by VLCADD: When the mother is affected, the disease usually improves during pregnancy, but delivery and postpartum are periods of increased energy expenditure and risk of rhabdomyolysis and myoglobinuria. Dietary measures and greater vigilance are prescribed (Leslie 2018, Yamamoto 2015, Mendez 2010). During breastfeeding a daily increase of 1,000 calories is recommended (Mendez 2010)