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. 2020 Jan 6;22(5):908–916. doi: 10.1038/s41436-019-0739-z

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© The Author(s) 2020

Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, and provide a link to the Creative Commons license. You do not have permission under this license to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.

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Fig. 1 — a Proportion of multiple acyl-CoA dehydrogenase deficiency (MADD)(-like) patients with organ based indication and efficacy of D,L-3-hydroxybutyrate treatment, with the numbers presented in the columns. Clinical improvement regarding liver symptoms included hyperammonemia, hypoglycemia, and/or metabolic acidosis; clinical improvement regarding muscle symptoms included exercise intolerance, hypotonia, myopathy, and/or rhabdomyolysis. b Kaplan–Meier curve of the survival in type II (n = 9) and type III (n = 14) MADD(-like) patients treated with D,L-3-HB compared with the survival in historical controls from literature (type I [n = 16], type II [n = 26] and type III [n = 156] MADD) who were not reported to have been treated with D,L-3-HB. c Kaplan–Meier plot that demonstrates the cumulative proportion of MADD(-like) patients with reported clinical improvement upon initiation of D,L-3-HB treatment over time. Calculated from a total of 21 patients with sufficient data. HC historical control. x = censored patient.