Publication

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 journalArticleAcademicpeer-review

APA

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. (2020). Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific: An investigator-initiated, randomized, crossover study. Journal of Inherited Metabolic Disease. https://doi.org/10.1002/jimd.12302, https://doi.org/10.1002/jimd.12302

Author

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. / Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific : An investigator-initiated, randomized, crossover study. In: Journal of Inherited Metabolic Disease. 2020.

Harvard

Hoogeveen, IJ, de Boer, F, Boonstra, WF, van Der Schaaf, CJ, Steuerwald, U, Sibeijn-Kuiper, AJ, Vegter, RJK, van Der Hoeven, JH, Heiner-Fokkema, MR, Clarke, KC, Cox, PJ, Derks, TGJ & Jeneson, JAL 2020, 'Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific: An investigator-initiated, randomized, crossover study', Journal of Inherited Metabolic Disease. https://doi.org/10.1002/jimd.12302, https://doi.org/10.1002/jimd.12302

Standard

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 journalArticleAcademicpeer-review

Vancouver

Hoogeveen IJ, de Boer F, Boonstra WF, van Der Schaaf CJ, Steuerwald U, Sibeijn-Kuiper AJ et al. Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific: An investigator-initiated, randomized, crossover study. Journal of Inherited Metabolic Disease. 2020 Sep 7. https://doi.org/10.1002/jimd.12302, https://doi.org/10.1002/jimd.12302


BibTeX

@article{bc84bb866b204e80b93d4a9c06728a65,
title = "Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease typeIIIaare phenotype-specific: An investigator-initiated, randomized, crossover study",
abstract = "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.",
keywords = "P-31-MRS, acute nutritional ketosis, exercise, glycogen storage disease, ketone-ester, DEBRANCHER DEFICIENCY, OXIDATIVE-METABOLISM, MUSCLE ULTRASOUND, SKELETAL-MUSCLE, III DIAGNOSIS, KETONE-BODIES, CARBOHYDRATE, MANAGEMENT, MYOPATHY, WORK",
author = "Hoogeveen, {Irene J.} and {de Boer}, Foekje and Boonstra, {Willemijn F.} and {van Der Schaaf}, {Caroline J.} and Ulrike Steuerwald and Sibeijn-Kuiper, {Anita J.} and Vegter, {Riemer J. K.} and {van Der Hoeven}, {Johannes H.} and Heiner-Fokkema, {M. Rebecca} and Clarke, {Kieran C.} and Cox, {Pete J.} and Derks, {Terry G. J.} and Jeneson, {Jeroen A. L.}",
note = "{\textcopyright} 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.",
year = "2020",
month = sep,
day = "7",
doi = "10.1002/jimd.12302",
language = "English",
journal = "Journal of Inherited Metabolic Disease",
issn = "0141-8955",
publisher = "SPRINGER",

}

RIS

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