Nutritional ketosis improves exercise metabolism in patients with very long-chain acyl-CoA dehydrogenase deficiency.

A maladaptive shift from fat to carbohydrate (CHO) oxidation during exercise is thought to underlie myopathy and exercise-induced rhabdomyolysis in patients with fatty acid oxidation (FAO) disorders. We hypothesised that ingestion of a ketone ester (KE) drink prior to exercise could serve as an alternative oxidative substrate supply to boost muscular ATP homeostasis. To establish a rational basis for therapeutic use of KE supplementation in FAO, we tested this hypothesis in patients deficient in Very Long-Chain acyl-CoA Dehydrogenase (VLCAD). Five patients (range 17-45 y; 4 M/1F) patients were included in an investigator-initiated, randomised, blinded, placebo-controlled, 2-way cross-over study. Patients drank either a KE + CHO mix or an isocaloric CHO equivalent and performed 35 minutes upright cycling followed by 10 minutes supine cycling inside a Magnetic Resonance scanner at individual maximal FAO work rate (fatmax; approximately 40% VO2 max). The protocol was repeated after a 1-week interval with the alternate drink. Primary outcome measures were quadriceps phosphocreatine (PCr), Pi and pH dynamics during exercise and recovery assayed by in vivo 31 P-MR spectroscopy. Secondary outcomes included plasma and muscle metabolites and respiratory gas exchange recordings. Ingestion of KE rapidly induced mild ketosis and increased muscle BHB content. During exercise at FATMAX, VLCADD-specific plasma acylcarnitine levels, quadriceps glycolytic intermediate levels and in vivo Pi/PCr ratio were all lower in KE + CHO than CHO. These results provide a rational basis for future clinical trials of synthetic ketone ester supplementation therapy in patients with FAO disorders. Trial registration: ClinicalTrials.gov. Protocol ID: NCT03531554; METC2014.492; ABR51222.042.14.

Physiological demands of therapeutic horseback riding in children with moderate to severe motor impairments: an exploratory study.

PURPOSE: To examine energy expenditure at rest and during a single therapeutic horseback riding (THR) session in children with moderate to severe motor impairments. METHODS: Heart rate (HR), oxygen uptake (.VO2), and minute ventilation (.VE) were measured continuously during a 10-minute rest period and during a typical THR session. RESULTS: Seven children (4 males, mean age 12.3 ± 3.5 years) completed the protocol. Significant increases from rest were seen for mean HR, .VO2, .VE, and energy expenditure. Based on .VO2, 43.3 ± 24.3% of the THR session consisted of sedentary, 44.4 ± 13.4% of light, and 12.3 ± 21.8% of moderate to vigorous activity intensity, with large interindividual differences. CONCLUSIONS: The physiological demands of THR in children with moderate to severe motor impairments are moderate. However, considering the short duration of maintaining moderate to vigorous exercise activity during THR in combination with the low training frequency, group data indicate that it is unlikely that THR will improve cardiopulmonary fitness in these children.

Clinimetric evaluation of measurement tools used in hand therapy to assess activity and participation.

STUDY DESIGN: Systematic review. INTRODUCTION: A number of measurement tools with strong clinimetric properties address activities and participation in hand-injured persons. PURPOSE OF THE STUDY: To evaluate clinimetric quality of measurement tools assessing activities and participation in patients with hand injuries. METHODS: The electronic databases Medline, Cochrane library, EMBASE, PEDro, Cumulative Index to Nursing and Allied Health Literature (ClNAHL), and Allied and Complementary Medicine Database (AMED) were searched for appropriate literature. Descriptive data of included tools were given, and their clinimetric quality was scored with specific criteria. RESULTS: The literature search identified 696 publications, referring to 15 measurement tools that met the inclusion criteria. For most of the included tests, applicability was good, whereas information on clinimetric properties was often lacking, especially for the performance tests. Overall, the Disabilities of the Arm, Shoulder, and Hand (DASH) was the most extensively studied tool with positive ratings for all criteria, closely followed by the Michigan Hand Outcomes Questionnaire (MHQ). Of the performance tests, the Functional Dexterity Test (FDT) received the best ratings. CONCLUSIONS: Optimal measurement tool selection depends highly on the purpose of measurement and the type of hand injury. LEVEL OF EVIDENCE: 2a-.