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Ketogenic diet in neonates: effects, pitfalls, and a paradigm from a preterm newborn affected by pyruvate dehydrogenase deficiency

Journal of Perinatology (2025)Cite this article

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Abstract

The application of ketogenic diet (KD) in newborns is rare and mainly reserved for specific metabolic and seizure disorders. Furthermore, only case reports describe the effects of KD in premature infants. Here, we summarize the recent advances, indications, mechanisms of action and practical issues related to KD. We also provide a paradigm of a preterm male infant born at 34 weeks with pyruvate dehydrogenase deficiency, highlighting the therapeutic challenges and outcomes with the KD. This report underscores the complexities of managing metabolic disorders in neonates and the effects of KD.

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Fig. 1: In red arrows we depict the pathway of the ketone bodies and their link to energy production, while pyruvate is a central intermediate mainly derived from glycolysis pathway as it’s end-product.
Fig. 2: Case description.

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Acknowledgements

We would like to acknowledge the Crystal Charity Ball and the NeuroNICU program at the University of Texas Southwestern Medical Center for supporting this project. Figure 1 was created with BioRender.com and appropriate permission has been obtained (#v25k736).

Funding

No financial support was received for the development of this review.

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Authors and Affiliations

  1. Department of Pediatrics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

    Sokratis Katsoudas

  2. Department of Pediatrics, Division of Genetics and Metabolism, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Luis A. Umana

  3. Department of Radiology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Rebekah Clarke

  4. Department of Pediatrics, Division of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Jennifer Thomas & Michelle Machie

  5. Department of Pediatrics, Division of Neonatal-Perinatal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Dimitrios Angelis

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Contributions

SK wrote the initial draft and critically reviewed the manuscript and approved it as submitted. DA contributed to the concept of the paper, wrote part of the initial and revised drafts of this manuscript, and approved the final manuscript as submitted. LAU, RC, JT, and MM contributed equally to the conceptualization of the paper, reviewed, and revised the manuscript, and approved the final manuscript as submitted.

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Correspondence to Dimitrios Angelis.

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The authors declare no competing interests.

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This study was approved by the University of Texas Southwestern Institutional Review Board, and informed parental consent was waived per IRB guidelines. All methods were performed in accordance with the relevant guidelines and regulations.

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Katsoudas, S., Umana, L.A., Clarke, R. et al. Ketogenic diet in neonates: effects, pitfalls, and a paradigm from a preterm newborn affected by pyruvate dehydrogenase deficiency. J Perinatol (2025). https://doi.org/10.1038/s41372-025-02427-6

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