Simona Bertoli1, Claudia Trentani2, Cinzia Ferraris2, Valentina De Giorgis3, Pierangelo Veggiotti3, Anna Tagliabue4. 1. International Centre for the Assessment of Nutritional Status (ICANS), Department of Food Environmental and Nutritional Sciences (DeFENS), University of Milan, Milan, Italy. 2. Human Nutrition and Eating Disorder Research Center, Department of Public Health, Experimental and Forensic Medicine University of Pavia, Pavia, Italy. 3. Department of Neuroscience, IRCCS "C. Mondino" Foundation, Pavia, Italy. 4. Human Nutrition and Eating Disorder Research Center, Department of Public Health, Experimental and Forensic Medicine University of Pavia, Pavia, Italy. Electronic address: Anna.tagliabue@unipv.it.
Abstract
OBJECTIVE: The only known treatment of glucose transporter 1 deficiency syndrome (GLUT-1 DS) is a ketogenic diet (KD), which provides the brain with an alternative fuel. Studies in children with intractable epilepsy have shown that a prolonged KD can induce a progressive loss of bone mineral content associated with poor bone health status, probably as a consequence of a chronic acidic environment. The aim of this study is to determine the long-term effects of a KD on body composition and bone mineral status of patients with GLUT-1 DS, is currently unknown. METHODS: In this case series, we report the changes in body composition and bone mineral status observed in three adult patients with GLUT-1 DS who have been treated with a KD for more than 5 y. RESULTS: A long-term KD did not produce appreciable changes in weight and body composition of adults with GLUT-1 DS. Moreover, we found no evidence of potential adverse effects of a KD on bone health. In summary, this case series contributes to a small but growing body of literature that investigated the potential long-term effects of a KD on bone health. CONCLUSIONS: Our data suggest that maintaining a KD for more than 5 y does not pose any major negative effects on body composition, bone mineral content, and bone mineral density in adults with GLUT-1 DS, a finding that is at variance with previous reports focusing on children with intractable epilepsy. Further studies with larger sizes are needed to confirm and expand our findings.
OBJECTIVE: The only known treatment of glucose transporter 1 deficiency syndrome (GLUT-1 DS) is a ketogenic diet (KD), which provides the brain with an alternative fuel. Studies in children with intractable epilepsy have shown that a prolonged KD can induce a progressive loss of bone mineral content associated with poor bone health status, probably as a consequence of a chronic acidic environment. The aim of this study is to determine the long-term effects of a KD on body composition and bone mineral status of patients with GLUT-1 DS, is currently unknown. METHODS: In this case series, we report the changes in body composition and bone mineral status observed in three adult patients with GLUT-1 DS who have been treated with a KD for more than 5 y. RESULTS: A long-term KD did not produce appreciable changes in weight and body composition of adults with GLUT-1 DS. Moreover, we found no evidence of potential adverse effects of a KD on bone health. In summary, this case series contributes to a small but growing body of literature that investigated the potential long-term effects of a KD on bone health. CONCLUSIONS: Our data suggest that maintaining a KD for more than 5 y does not pose any major negative effects on body composition, bone mineral content, and bone mineral density in adults with GLUT-1 DS, a finding that is at variance with previous reports focusing on children with intractable epilepsy. Further studies with larger sizes are needed to confirm and expand our findings.
Authors: M Caprio; M Infante; E Moriconi; A Armani; A Fabbri; G Mantovani; S Mariani; C Lubrano; E Poggiogalle; S Migliaccio; L M Donini; S Basciani; A Cignarelli; E Conte; G Ceccarini; F Bogazzi; L Cimino; R A Condorelli; S La Vignera; A E Calogero; A Gambineri; L Vignozzi; F Prodam; G Aimaretti; G Linsalata; S Buralli; F Monzani; A Aversa; R Vettor; F Santini; P Vitti; L Gnessi; U Pagotto; F Giorgino; A Colao; A Lenzi Journal: J Endocrinol Invest Date: 2019-05-20 Impact factor: 4.256
Authors: Mackenzie C Cervenka; Susan Wood; Manny Bagary; Antoaneta Balabanov; Eduard Bercovici; Mesha-Gay Brown; Orrin Devinsky; Cherubino Di Lorenzo; Colin P Doherty; Elizabeth Felton; Laura A Healy; Pavel Klein; Magnhild Kverneland; Danielle Lambrechts; Jennifer Langer; Janak Nathan; Jude Munn; Patty Nguyen; Matthew Phillips; Kelly Roehl; Adrianna Tanner; Clare Williams; Beth Zupec-Kania Journal: Neurol Clin Pract Date: 2021-10
Authors: Salvador Vargas-Molina; Leandro Carbone; Ramón Romance; Jorge L Petro; Brad J Schoenfeld; Richard B Kreider; Diego A Bonilla; Javier Benítez-Porres Journal: Eur J Appl Physiol Date: 2021-05-18 Impact factor: 3.078