Does fatty acid oxidation flux predict the clinical severity of VLCAD deficiency?
Our results demonstrate a very strong correlation between LC-FAO flux in fibroblasts and the clinical severity of VLCADD. LC-FAO flux measurements may thus predict whether patients are likely to develop symptoms.
Normal clinical and radiologic examination predicted absence of a significant cardiac abnormality on echocardiography in 100% of cases. We conclude that routine echocardiography before embarking on surgical intervention may not always be necessary but should be reserved for infants with abnormal clinical and/or radiologic findings.