title: Long-term effects of a ketogenic diet on body composition and bone mineralization in GLUT-1 deficiency syndrome: a case series.
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.
authors.affiliation: International Centre for the Assessment of Nutritional Status (ICANS), Department of Food Environmental and Nutritional Sciences (DeFENS), University of Milan, Milan, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Bertoli
authors.affiliation: Human Nutrition and Eating Disorder Research Center, Department of Public Health, Experimental and Forensic Medicine University of Pavia, Pavia, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Trentani
authors.affiliation: Human Nutrition and Eating Disorder Research Center, Department of Public Health, Experimental and Forensic Medicine University of Pavia, Pavia, Italy.
authors.forename: Cinzia
authors.initials: C
authors.lastname: Ferraris
authors.affiliation: Department of Neuroscience, IRCCS "C. Mondino" Foundation, Pavia, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: De Giorgis
authors.affiliation: Department of Neuroscience, IRCCS "C. Mondino" Foundation, Pavia, Italy.
authors.forename: Pierangelo
authors.initials: P
authors.lastname: Veggiotti
authors.affiliation: 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.
authors.forename: Anna
authors.initials: A
authors.lastname: Tagliabue
mesh_terms: D020155:3-Hydroxybutyric Acid / Q000097:blood; D000273:Adipose Tissue / Q000378:metabolism; D000328:Adult; D001823:Body Composition; D015992:Body Mass Index; D001835:Body Weight; D015519:Bone Density / Q000502:physiology; D002113:Calcification, Physiologic / Q000502:physiology*; D002239:Carbohydrate Metabolism, Inborn Errors / Q000178:diet therapy*; D055423:Diet, Ketogenic / Q000009:adverse effects*; D004040:Dietary Carbohydrates / Q000008:administration & dosage; D004041:Dietary Fats / Q000008:administration & dosage; D004043:Dietary Fiber / Q000008:administration & dosage; D002149:Energy Intake; D004827:Epilepsy / Q000178:diet therapy; D005260:Female; D006801:Humans; D018977:Micronutrients / Q000008:administration & dosage; D009004:Monosaccharide Transport Proteins / Q000172:deficiency*; D009043:Motor Activity; D018482:Muscle, Skeletal / Q000502:physiology; D010349:Patient Compliance; D012885:Skinfold Thickness; D013997:Time Factors; D016896:Treatment Outcome; D055105:Waist Circumference; D055815:Young Adult
chemical_list: D004040:Dietary Carbohydrates; D004041:Dietary Fats; D004043:Dietary Fiber; D018977:Micronutrients; D009004:Monosaccharide Transport Proteins; D020155:3-Hydroxybutyric Acid
keywords: Body composition; Bone health; Glucose transporter 1 deficiency syndrome; Ketogenic diet