Ketogenic diet as a glycine lowering therapy in nonketotic hyperglycinemia and impact on brain glycine levels.
Emily ShelkowitzRussell P SanetoWalla Al-HertaniCharlotte M A LuboutNicholas V StenceMark S BrownPatrick LongDiana WalleighJulie A NelsonFrancisco E PerezDennis W W ShawEmma J MichlJohan L K Van HovePublished in: Orphanet journal of rare diseases (2022)
KD is an effective glycine reduction method in NKH, and may provide a more consistent reduction in plasma glycine levels than high-dose benzoate therapy. Both high-dose benzoate therapy and KD equally reduced but did not normalize brain glycine levels even in the setting of low-normal plasma glycine.