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Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific: An investigator-initiated, randomized, crossover study
Hoogeveen, I. J., de Boer, F., Boonstra, W. F., van Der Schaaf, C. J., Steuerwald, U., Sibeijn-Kuiper, A. J., Vegter, R. J. K., van Der Hoeven, J. H., Heiner-Fokkema, M. R., Clarke, K. C., Cox, P. J., Derks, T. G. J. & Jeneson, J. A. L., 7-Sep-2020, In : Journal of Inherited Metabolic Disease. 14 p.Research output: Contribution to journal › Article › Academic › peer-review
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Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific : An investigator-initiated, randomized, crossover study. / Hoogeveen, Irene J.; de Boer, Foekje; Boonstra, Willemijn F.; van Der Schaaf, Caroline J.; Steuerwald, Ulrike; Sibeijn-Kuiper, Anita J.; Vegter, Riemer J. K.; van Der Hoeven, Johannes H.; Heiner-Fokkema, M. Rebecca; Clarke, Kieran C.; Cox, Pete J.; Derks, Terry G. J.; Jeneson, Jeroen A. L.
In: Journal of Inherited Metabolic Disease, 07.09.2020.Research output: Contribution to journal › Article › Academic › peer-review
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TY - JOUR
T1 - Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific
T2 - An investigator-initiated, randomized, crossover study
AU - Hoogeveen, Irene J.
AU - de Boer, Foekje
AU - Boonstra, Willemijn F.
AU - van Der Schaaf, Caroline J.
AU - Steuerwald, Ulrike
AU - Sibeijn-Kuiper, Anita J.
AU - Vegter, Riemer J. K.
AU - van Der Hoeven, Johannes H.
AU - Heiner-Fokkema, M. Rebecca
AU - Clarke, Kieran C.
AU - Cox, Pete J.
AU - Derks, Terry G. J.
AU - Jeneson, Jeroen A. L.
N1 - © 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.
PY - 2020/9/7
Y1 - 2020/9/7
N2 - Glycogen storage disease type IIIa (GSDIIIa) is an inborn error of carbohydrate metabolism caused by a debranching enzyme deficiency. A subgroup of GSDIIIa patients develops severe myopathy. The purpose of this study was to investigate whether acute nutritional ketosis (ANK) in response to ketone-ester (KE) ingestion is effective to deliver oxidative substrate to exercising muscle in GSDIIIa patients. This was an investigator-initiated, researcher-blinded, randomized, crossover study in six adult GSDIIIa patients. Prior to exercise subjects ingested a carbohydrate drink (~66 g, CHO) or a ketone-ester (395 mg/kg, KE) + carbohydrate drink (30 g, KE + CHO). Subjects performed 15-minute cycling exercise on an upright ergometer followed by 10-minute supine cycling in a magnetic resonance (MR) scanner at two submaximal workloads (30% and 60% of individual maximum, respectively). Blood metabolites, indirect calorimetry data, and in vivo 31P-MR spectra from quadriceps muscle were collected during exercise. KE + CHO induced ANK in all six subjects with median peak βHB concentration of 2.6 mmol/L (range: 1.6-3.1). Subjects remained normoglycemic in both study arms, but delta glucose concentration was 2-fold lower in the KE + CHO arm. The respiratory exchange ratio did not increase in the KE + CHO arm when workload was doubled in subjects with overt myopathy. In vivo 31P MR spectra showed a favorable change in quadriceps energetic state during exercise in the KE + CHO arm compared to CHO in subjects with overt myopathy. Effects of ANK during exercise are phenotype-specific in adult GSDIIIa patients. ANK presents a promising therapy in GSDIIIa patients with a severe myopathic phenotype. Trial registration number: ClinicalTrials.gov identifier: NCT03011203.
AB - Glycogen storage disease type IIIa (GSDIIIa) is an inborn error of carbohydrate metabolism caused by a debranching enzyme deficiency. A subgroup of GSDIIIa patients develops severe myopathy. The purpose of this study was to investigate whether acute nutritional ketosis (ANK) in response to ketone-ester (KE) ingestion is effective to deliver oxidative substrate to exercising muscle in GSDIIIa patients. This was an investigator-initiated, researcher-blinded, randomized, crossover study in six adult GSDIIIa patients. Prior to exercise subjects ingested a carbohydrate drink (~66 g, CHO) or a ketone-ester (395 mg/kg, KE) + carbohydrate drink (30 g, KE + CHO). Subjects performed 15-minute cycling exercise on an upright ergometer followed by 10-minute supine cycling in a magnetic resonance (MR) scanner at two submaximal workloads (30% and 60% of individual maximum, respectively). Blood metabolites, indirect calorimetry data, and in vivo 31P-MR spectra from quadriceps muscle were collected during exercise. KE + CHO induced ANK in all six subjects with median peak βHB concentration of 2.6 mmol/L (range: 1.6-3.1). Subjects remained normoglycemic in both study arms, but delta glucose concentration was 2-fold lower in the KE + CHO arm. The respiratory exchange ratio did not increase in the KE + CHO arm when workload was doubled in subjects with overt myopathy. In vivo 31P MR spectra showed a favorable change in quadriceps energetic state during exercise in the KE + CHO arm compared to CHO in subjects with overt myopathy. Effects of ANK during exercise are phenotype-specific in adult GSDIIIa patients. ANK presents a promising therapy in GSDIIIa patients with a severe myopathic phenotype. Trial registration number: ClinicalTrials.gov identifier: NCT03011203.
KW - P-31-MRS
KW - acute nutritional ketosis
KW - exercise
KW - glycogen storage disease
KW - ketone-ester
KW - DEBRANCHER DEFICIENCY
KW - OXIDATIVE-METABOLISM
KW - MUSCLE ULTRASOUND
KW - SKELETAL-MUSCLE
KW - III DIAGNOSIS
KW - KETONE-BODIES
KW - CARBOHYDRATE
KW - MANAGEMENT
KW - MYOPATHY
KW - WORK
U2 - 10.1002/jimd.12302
DO - 10.1002/jimd.12302
M3 - Article
C2 - 33448466
JO - Journal of Inherited Metabolic Disease
JF - Journal of Inherited Metabolic Disease
SN - 0141-8955
ER -
ID: 134616549