Hacettepe University
Ankara, State, 06100, Turkey (Türkiye)
NCT Number: NCT06449456
Congenital Adrenal Hyperplesia (CAH) is a group of automosal recessive disorders that develop due to a deficiency of one of the five enyzmes necessary for cortisol synthesis in the adrenal cortex. Research indicates a predisposition in children and adolescents with CAH towards adverse metabolic changes such as obesity, hypertension, insulin resistance and increased intima-media thickness. This study aims to compare the extent to which children and adolescents with CAH are effected in terms of respiratory and peripheral muscle strength, exercise capasity, physical fitness and physical activity levels compared o their matched healthy individuals.
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Notify Me8 year–18 year
All sexes
Observational
Ankara, State, 06100, Turkey (Türkiye)
Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive diseases caused by the deficiency of one of the five enzymes necessary for cortisol synthesis in the adrenal cortex. Dysfunction in cortisol synthesis, as seen in other enzyme deficiencies, leads to an increase in adrenocorticotropic hormone (ACTH) secretion, resulting in hypertension and hypokalemia. Additionally, patients with CAH exhibit excess androgens alongside cortisol deficiency. İn CAH treatment, glucocorticoids are used to suppress androgen production in the adrenal cortex. Deficiency in glucocorticoids and mineralocorticoids, as well as excess androgens, can contribute to the development of adverse metabolic and cardiovascular anomalies.
Ongoing research has revealed a predisposition for adverse metabolic changes in children and adolescents with CAH, including obesity, hypertension, insulin resistance, and increased intima-media thickness, which can increase the risk of developing cardiovascular disease in adulthood. Furthermore, subclinical left ventricular diastolic dysfunction has been observed in adolescents with CAH. İn these patients, due to the deficiency of epinephrine there is an increased tendency for hypoglycemia under stress conditions.
These metabolic changes can lead to impaired exercise performance in patients with CAH, consequently affecting their quality of life.
A study involving twenty classic CAH adolescents and twenty healthy counterparts demonstrated that classic CAH patients exhibited reduced exercise capacity with lower peak workload and higher peak systolic blood pressure during exercise. However, there is a lack of research in the literature comparing respiratory and peripheral muscle strength, daily life activities, physical fitness, and levels of physical activity in children and adolescents with CAH to their healthy peers.
Healthy volunteers accepted: Yes
Only the study team can determine whether someone qualifies for participation.
Inclusion criteria
for CAH Group:
Exclusion criteria
for CAH Groups:
Inclusion criteria
for Healthy Group:
Exclusion criteria
for Healthy Group:
Time frame: 1 day
Respiratory muscle strength will be measured using a portable, electronic mouth pressure measurement device (Micro Medical MicroMPM, UK). The best of three measurements for Maximal inspiratory pressure (MIP) and Maximal expiratory pressure (MEP) will be selected. Real MIP and MEP values and the percentages of expected values based on age and gender will be used in the analyses.
Time frame: 1 day
Peripheral muscles'strength will be assessed using the K-Force hand dynamometer.
Time frame: 1 day
It will be evaluated with the Fitnessgram test battery.
Time frame: 1 day
It will be measured using the 6-Minute Walk Test.
Time frame: 1 day
The Physical Activity Level will be assessed using the Physical Activity Questionnaire for Adolescents (PAQ-A) and the Physical Activity Questionnaire for Children (PAQ-C). The scores for PAQ-A and PAQ-C range from 9 to 45, with higher scores indicating better physical activity levels. The results of both questionnaires will be presented separately. The PAQ-A score will be reported as a distinct outcome measure for adolescents, and the PAQ-C score will be reported as a distinct Outcome Measure for children. This approach ensures a clear evaluation of physical activity levels in both age groups.
Time frame: 1 day
Jamar hand dynamometer (Jamar®, California, USA) will be used to measure grip strength.
Time frame: 1 day
Anthropometric measurements will include separate measurements of waist and hip circumference, and calculations of the waist/hip ratio and waist/height ratio.
Time frame: 1 day
Body composition will be evaluated using bioelectric impedance analysis (Body Composition Analyzer MC-780MA). Measurements will include fat mass, fat-free mass, and water percentage. Each of these measurements will be reported as separate outcome measures. Fat mass will be reported in kilograms, fat-free mass in kilograms, and water percentage as a percentage of total body weight.
Time frame: 1 day
Quality of life will be assessed using the Pediatric Outcome Data Collection Instrument (PODCI) child and adolescent form. Scores for all dimensions range from 0 to 100, with 100 indicating the highest level of function or participation.
Time frame: 1 day
HRV will be assessed by measuring it with the Polar H10 device before, after, and during the recovery phase of the 6-minute walk test, specifically in the first and second minutes.
Hacettepe University
Other
A Comparison of Exercise Capacity, Muscle Functions, Physical Fitness, and Physical Activity Levels in Children With Congenital Adrenal Hyperplasia and Healthy Controls
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