Frontiers in Nutrition (Feb 2023)

Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting

  • Valentina De Giorgis,
  • Valentina De Giorgis,
  • Cinzia Ferraris,
  • Mario Leo Brena,
  • Giorgio Farris,
  • Valerio Gentilino,
  • Monica Guglielmetti,
  • Claudia Marazzi,
  • Ludovica Pasca,
  • Ludovica Pasca,
  • Claudia Trentani,
  • Anna Tagliabue,
  • Costanza Varesio,
  • Costanza Varesio

DOI
https://doi.org/10.3389/fnut.2023.1114386
Journal volume & issue
Vol. 10

Abstract

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Ketogenic Dietary Treatments (KDTs) are to date the gold-standard treatment for glucose transporter type 1 (GLUT1) deficiency syndrome. Administration of KDTs is generally per os; however, in some conditions including the acute gastro-enteric post-surgical setting, short-term parenteral (PN) administration might be needed. We report the case of a 14-year-old GLUT1DS patient, following classic KDT for many years, who underwent urgent laparoscopic appendectomy. PN-KDT was required, after 1 day of fasting. No ad hoc PN-KDTs products were available and the patient received infusions of OLIMEL N4 (Baxter). On the sixth day postoperatively enteral nutrition was progressively reintroduced. The outcome was optimal with rapid recovery and no exacerbation of neurological manifestations. Our patient is the first pediatric patient with GLUT1DS in chronic treatment with KDT efficiently treated with exclusive PN for five days. This case reports on real-word management and the ideal recommendations for PN-KDT in an acute surgical setting.

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