PMID- 32773572
OWN - NLM
STAT- MEDLINE
DCOM- 20210219
LR  - 20210219
IS  - 1752-2978 (Electronic)
IS  - 1752-296X (Linking)
VI  - 27
IP  - 5
DP  - 2020 Oct
TI  - The potential of a ketogenic diet to minimize effects of the metabolic fault in 
      glycogen storage disease V and VII.
PG  - 283-290
LID - 10.1097/MED.0000000000000567 [doi]
AB  - PURPOSE OF REVIEW: To explore the potential of a low carbohydrate ketogenic diet 
      (LCKD) to counter physical activity intolerance, pain and muscle damage for 
      glycogen storage disease (GSD) V and VII, and highlight the realistic possibility 
      that nutrition could be key. RECENT FINDINGS: Carbohydrate (CHO) ingestion during 
      physical activity in GSDV and a LCKD for GSDVII is common. For the latter, a 
      long-term study demonstrated improvement in physiological markers while on a 
      LCKD. This included improvement in aerobic power and activity tolerance. In GSDV, 
      preliminary research on a LCKD suggest a diet of 75% fat, 15% protein, 10% CHO, 
      is best for improved function and compliance. Ketones provide immediate fuel for 
      acute physical activity, and have an epigenetic role, improving ketone and lipid 
      use. Evidence from elite athletes found a LCKD can increase fat oxidation and is 
      optimal at 70% VO2max. This suggests the need to also improve conditioning via 
      exercise to maximize the benefit of a LCKD. SUMMARY: A high CHO diet in GSDV and 
      VII comes with a restricted physical activity capacity alongside significant 
      pain, muscle damage and risk of renal failure. Mounting evidence suggests a LCKD 
      is efficacious for both disorders providing an immediate fuel source which may 
      negate the need for a 'warm-up' prior to every activity and restore 'normal' 
      function.
FAU - Reason, Stacey L
AU  - Reason SL
AD  - International Association for Muscle Glycogen Storage Disease, San Francisco, 
      California, USA.
FAU - Godfrey, Richard J
AU  - Godfrey RJ
AD  - Centre for Human Performance, Exercise and Rehabilitation, Brunel University, 
      London, UK.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - England
TA  - Curr Opin Endocrinol Diabetes Obes
JT  - Current opinion in endocrinology, diabetes, and obesity
JID - 101308636
SB  - IM
MH  - Diet, Carbohydrate-Restricted
MH  - *Diet, Ketogenic
MH  - Exercise Tolerance/physiology
MH  - Glycogen Storage Disease Type V/complications/*diet therapy/metabolism
MH  - Glycogen Storage Disease Type VII/complications/*diet therapy/metabolism
MH  - Humans
MH  - Lung Volume Measurements
MH  - Muscle, Skeletal/metabolism
MH  - Oxidation-Reduction
EDAT- 2020/08/11 06:00
MHDA- 2021/02/20 06:00
CRDT- 2020/08/11 06:00
PHST- 2020/08/11 06:00 [pubmed]
PHST- 2021/02/20 06:00 [medline]
PHST- 2020/08/11 06:00 [entrez]
AID - 01266029-202010000-00007 [pii]
AID - 10.1097/MED.0000000000000567 [doi]
PST - ppublish
SO  - Curr Opin Endocrinol Diabetes Obes. 2020 Oct;27(5):283-290. doi: 
      10.1097/MED.0000000000000567.