Long-term ketone body therapy of severe multiple acyl-CoA dehydrogenase deficiency (MADD) – A case report

MADD is the most severe disorder of mitochondrial fatty acid ß-oxidation. Treatment of this disorder is difficult since the functional loss of the ETF makes energy supply from fatty acids impossible. Acetyl-CoA, provided by exogenous ketone bodies like NaßHB, is the only treatment option in severe cases. Short-term therapy attempts have shown positive resul ts. There are no reports about long-term application of ketone body salts in patients with severe MADD.

Source: Nutrition - October 20, 2018 Category: Nutrition Authors: Tobias Fischer, Ulrike Och, Thorsten Marquardt Source Type: research