TY - JOUR
T1 - Long-term effects of a ketogenic diet on body composition and bone mineralization in GLUT-1 deficiency syndrome
T2 - A case series
AU - Bertoli, Simona
AU - Trentani, Claudia
AU - Ferraris, Cinzia
AU - De Giorgis, Valentina
AU - Veggiotti, Pierangelo
AU - Tagliabue, Anna
PY - 2014
Y1 - 2014
N2 - 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 bonemineral content associated with poor bone health status, probably as a consequence ofachronic acidic environment. The aim of this study is to determine the long-term effects of aKD 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.
AB - 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 bonemineral content associated with poor bone health status, probably as a consequence ofachronic acidic environment. The aim of this study is to determine the long-term effects of aKD 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.
KW - Body composition
KW - Bone health
KW - Glucose transporter 1 deficiency syndrome
KW - Ketogenic diet
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U2 - 10.1016/j.nut.2014.01.005
DO - 10.1016/j.nut.2014.01.005
M3 - Article
C2 - 24800673
AN - SCOPUS:84899718300
VL - 30
SP - 726
EP - 728
JO - Nutrition International
JF - Nutrition International
SN - 0899-9007
IS - 6
ER -