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

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) adminis...

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Autores principales: De Giorgis, Valentina, Ferraris, Cinzia, Brena, Mario Leo, Farris, Giorgio, Gentilino, Valerio, Guglielmetti, Monica, Marazzi, Claudia, Pasca, Ludovica, Trentani, Claudia, Tagliabue, Anna, Varesio, Costanza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9975382/
https://www.ncbi.nlm.nih.gov/pubmed/36875855
http://dx.doi.org/10.3389/fnut.2023.1114386
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author De Giorgis, Valentina
Ferraris, Cinzia
Brena, Mario Leo
Farris, Giorgio
Gentilino, Valerio
Guglielmetti, Monica
Marazzi, Claudia
Pasca, Ludovica
Trentani, Claudia
Tagliabue, Anna
Varesio, Costanza
author_facet De Giorgis, Valentina
Ferraris, Cinzia
Brena, Mario Leo
Farris, Giorgio
Gentilino, Valerio
Guglielmetti, Monica
Marazzi, Claudia
Pasca, Ludovica
Trentani, Claudia
Tagliabue, Anna
Varesio, Costanza
author_sort De Giorgis, Valentina
collection PubMed
description 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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spelling pubmed-99753822023-03-02 Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting De Giorgis, Valentina Ferraris, Cinzia Brena, Mario Leo Farris, Giorgio Gentilino, Valerio Guglielmetti, Monica Marazzi, Claudia Pasca, Ludovica Trentani, Claudia Tagliabue, Anna Varesio, Costanza Front Nutr Nutrition 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. Frontiers Media S.A. 2023-02-15 /pmc/articles/PMC9975382/ /pubmed/36875855 http://dx.doi.org/10.3389/fnut.2023.1114386 Text en Copyright © 2023 De Giorgis, Ferraris, Brena, Farris, Gentilino, Guglielmetti, Marazzi, Pasca, Trentani, Tagliabue and Varesio. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Nutrition
De Giorgis, Valentina
Ferraris, Cinzia
Brena, Mario Leo
Farris, Giorgio
Gentilino, Valerio
Guglielmetti, Monica
Marazzi, Claudia
Pasca, Ludovica
Trentani, Claudia
Tagliabue, Anna
Varesio, Costanza
Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting
title Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting
title_full Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting
title_fullStr Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting
title_full_unstemmed Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting
title_short Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting
title_sort classic ketogenic diet in parenteral nutrition in a glut1ds patient: doing more with less in an acute surgical setting
topic Nutrition
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9975382/
https://www.ncbi.nlm.nih.gov/pubmed/36875855
http://dx.doi.org/10.3389/fnut.2023.1114386
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