Energy Supplements to Improve Exercise Tolerance in Metabolic Myopathies
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 6
- 试验地点
- 2
- 主要终点
- maximal work capacity
研究概览
简要总结
Patients suffering from the metabolic myopathy Glycogen Storage Disease type IIIa (GSDIIIa) have a problem releasing sugar stored in cells that is needed for energy production. This causes several systemic impairments, but only recently have the exercise-related symptoms in the muscles been examined. A previous study showed signs that intravenous infusion of glucose relieves some of these symptoms. The purpose of this study is to investigate in a randomized and placebo-controlled fashion whether oral ingestion of sugar can alleviate muscular symptoms in patients with GSDIIIa.
详细描述
It has recently been documented how patients with GSDIIIa have a moderate to severely reduced exercise capacity, and that exercise induces muscle pain and cramps. These symptoms are caused by the inability to mobilize skeletal muscle glycogen and are most likely the consequence of a severe energy deficiency within muscles. The study changed the phenotype of GSDIIIa, to include exercise-induced symptoms, which is a typical presentation in other metabolic myopathies. It also documented that exercise capacity was significantly improved while exercise-induced muscular symptoms were relieved by an intravenous glucose infusion. Based on these findings, this study wishes to investigate if oral ingestion of sucrose has the same effects on work capacity on a larger number of patients, in a randomized, placebo-controlled, cross-over setup. Ingestion of sucrose has the potential to be an effective, cheap and easily accessible dietary treatment of muscular symptoms in GSDIIIa.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Crossover
- 主要目的
- Treatment
- 盲法
- Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor)
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •Genetically and/or biochemically verified GSDIIIa.
- •18 years or older.
排除标准
- •Clinically significant cardiac or pulmonary disease.
- •Pregnancy or lactation.
- •Severe mental disorders or participants that are in other ways unable to understand the purpose of the trials.
- •Subjects where the investigator assess that it is not possible or very difficult to place an intravenous catheters.
- •Other conditions of the joints or skeletal muscle such as arthritis or sprains. If the condition is expected to resolve before the study inclusion period is stopped, the subject may be included at a later time.
- •Moderate to severe muscle weakness, where the participants are not expected to complete 10 minutes of cycle-ergometry exercise at 70 % of VO2peak.
- •Verified diabetes.
- •Participation in other clinical trials that may interfere with the results.
- •Medications that may interfere with the results or increase the risk of bleeding.
- •Blood-clotting or bleeding disorders.
- •Blood donation one month or less prior to inclusion.
研究组 & 干预措施
FAXE Kondi Free - a sugarfree soft-drink
100 ml FAXE Kondi Free (0 grams of carbohydrates per 100 ml) is ingested every ten minutes during exercise plus 400 ml before exercise start.
干预措施: Faxe Kondi Free (Dietary Supplement)
FAXE Kondi - a sugary soft-drink
100 ml FAXE Kondi (10 grams of carbohydrates per 100 ml) is ingested every ten minutes during exercise plus 400 ml before exercise start.
干预措施: FAXE Kondi (Dietary Supplement)
结局指标
主要结局
maximal work capacity
时间窗: After up to 1 hour of bicycling on the 2nd and 4th day.
Area Under the Curve (AUC) = resistance times duration of workout
次要结局
- Peak oxygen consumption(After up to 1 hour of cycling on the 2nd and 4th day.)
- Peak workload(After up to 1 hour of cycling on the 2nd and 4th day.)
- Peak respiratory exchange ratio(After up to 1 hour of cycling on the 2nd and 4th day.)
- p-lactate(measured at rest and max on day 1, and before first dose of soft drink, before exercise and every 10 minutes during exercise at day 2 and 4.)
- Heart rate(Continously during the cycle test (max. 1 hour) on the 2nd and 4th day)
- Borg score(Measured periodically during the cycle test (max. 1 hour) on the 2nd and 4th day)
研究者
Astrid Emilie Buch
BSc
Rigshospitalet, Denmark