To compare the oxygenation and oxidative metabolism in skeletal muscles of patients with CF with healthy controls to provide insight in the metabolism in patients with CF.
ID
Source
Brief title
Condition
- Muscle disorders
- Congenital respiratory tract disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
- * time of PCr recovery (31P MRS)
- Resting, exercise and recovery phoscreatine (PCr)(31P MRS)
- Resting, exercise and recovery Inorganic phosphorus (Pi)(31P MRS)
- Resting, exercise and recovery Pi:PCr ratio(31P MRS)
- Resting, exercise and recovery (Adenosine triphosphate) ATP(31P MRS)
- Resting, exercise and recovery ATP:PCr ratio(31P MRS)Change in concentration
deoxygenated hemoglobin. (NIRS)
- Change in concentration oxygenated hemoglobin. (NIRS)
- Change in concentration total hemoglobin. (NIRS)
- Change in concentration deoxygenated hemoglobin minus change in concentration
oxygenated hemoglobin. (NIRS)
- The T1/2 (s) change in oxygenated hemoglobin in the recovery phase. (NIRS)
Secondary outcome
- Peak workload (Wpeak)
- Resting and peak oxygen uptake (VO2peak/kg (ml.min-1.kg-1) )
- Resting and peak minute ventilation (VEpeak) (l.min-1)
- Resting and peak respiratory exchange ratio (RER = VCO2/VO2)
- Resting and peak heart rate (HR; bpm)
- Resting and peak SpO2
Background summary
Patients with CF have a reduced exercise tolerance. Studies show an
interrelationship between lung function, muscle mass, energy expenditure,
(respiratory) muscle function and exercise capacity in patients with CF. Up to
date, there is no scientific evidence or consensus whether the muscle
dysfunction is caused by an intrinsic abnormality in muscle energy metabolism
and/or is hampered due to impaired oxygen delivery to these muscles, which
might be mediated by an increased blood flow demand of the respiratory muscles.
Therefore, it remains unclear whether the impaired muscle function is limited
by oxygen delivery or oxygen utilization. We hypothesise that patients with CF
have impaired oxidative metabolism and/or reduced/slowed oxygen delivery to the
skeletal muscles compared to healthy controls.
Study objective
To compare the oxygenation and oxidative metabolism in skeletal muscles of
patients with CF with healthy controls to provide insight in the metabolism in
patients with CF.
Study design
This is an case-control study. Ten patients with CF and ten healthy controls
will be recruited. In two separate test-sessions, thigh muscles of a population
of patients with CF and healthy age-matched controls will be measured during
rest, incremental cycling exercise, and recovery with NIRS (first test) and,
in another test session on a separate day, with 31P MRS (second test). The
ergometer is made of non-ferrous components, based on the prototype developed
by Jeneson et al. for in-magnet bicycling exercise testing (Jeneson et al,
2009).
Before the first (NIRS) test, subjects perform a lungfunction test and adipose
tissue thickness is measured. Subjects are asked to fill in a daily activities
questionnaire and a MRI and maximal exercise test (only in healthy controls)
questionnaire two weeks before the first (NIRS) test day.
Study burden and risks
No burden and risks are reported concerning exercise in a 31 P-MRS scanner or
with NIRS. Participation in this study will take 25 minutes to fill in the
questionnaires and will cost 2,5 hours in total on the two separate test
sessions. Insight in the muscle metabolism from patients with CF will help to
aim and institute interventions as exercise training.
Lundlaan 6
3584 EA Utrecht
NL
Lundlaan 6
3584 EA Utrecht
NL
Listed location countries
Age
Inclusion criteria
Patients with CF:
- Ambulant patients with CF
- free from acute pulmonary or gastro-intestinal exacerbation
- from 12 to 18 years
Healthy controls:
- from 12 to 18 years
- free from constraints in performing a maximal exercise test
Exclusion criteria
Patients with CF:
- Resting oxygen saturation (SpO2)< 94%
- FEV1predicted < 80%
- Ineligible to perform an exercise test
- Not familiar with the Dutch language
- Presence of contra-indications for 31P MRS measurements (assessed by a standardised questionnaire as previously used in METC 08-267/K)
- Participation in DO-IT or having participated less than six months ago in DO-IT (METC 09-114/K);Healthy controls:
- Ineligible to perform a maximal exercise test (assessed by a standardised questionnaire as previously used in METC 10-468)
- Not familiar with the Dutch language
- Presence of contra-indications for 31P MRS measurements (assessed by a standardised questionnaire as previously used in METC 08-267/K)
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL35697.041.11 |