High protein prescription in methylmalonic and propionic acidemia patients and its negative association with long-term outcome

Methylmalonic acidemia (MMA) and propionic acidemia (PA) are inborn errors of metabolism. While survival of MMA and PA patients has improved in recent decades, long-term outcome is still unsatisfactory. A protein restricted diet is the mainstay for treatment. Additional amino acid mixtures (AAM) can be prescribed if natural protein is insufficient. It is unknown if dietary treatment can have an impact on outcome.

Source: Clinical Nutrition - December 24, 2020 Category: Nutrition Authors: F. Molema, H.A. Haijes, M.C. Janssen, A.M. Bosch, F.J. van Spronsen, M.F. Mulder, N.M. Verhoeven-Duif, J.J.M. Jans, A.T. van der Ploeg, M.A. Wagenmakers, M.E. Rubio-Gozalbo, M.C.G.J. Brouwers, M.C. de Vries, S. Fuchs, J.G. Langendonk, D. Rizopoulos, P.M. Tags: Original article Source Type: research