Definition:
Very long-chain acyl-coenzyme A dehydrogenase deficiency or VLCAD for brevity is a disorder of the fatty acid oxidation disorder that hinders the normal conversion of certain fats to energy by the body especially during period without food intake.
Diagnosis:
Confirmation of diagnosis of VLCAD deficiency may be obtained by assaying enzymes activity in cultured skin fibroblasts leukocytes.
Treatment:
Management involves avoiding fasting, maintaining high carbohydrate intake and treating episodes of acute diseasewith glucose-containing intravenous fluids. Medium chain triglycerides whose oxidation does not involve VLCAD can be administered to provide calories but should never ve used until the diagnosis of VLCAD deficiency is certain.
Symptoms and Signs:
Symptoms include low blood sugar or hypoglycemia, lethargy or lack of energy and weakness of the muscle. Symptoms that begin later in childhood, adolescence or even in adulthood normally involve heart problems. It is also characterized by fasting induced hypokelotic hypoglycemia, sncephalopathy and mild heap tomegaly and exercise-induced muscle pain, rhabdomyolysis and elevated creatine kinase levels (Colid, D. (2003). Rudolph’s Pediatric, MacGraw-Hill Professional, p.630).
Causes:
Very long-chain acyl-coenzyme A dehydrogenase deficiency or VLCAD is said to be caused by myopathy and cardiomyopathy (Hoffman,G. (2001). Inherited Metabolic Disease, p.256).
