Pepplinkhuizen, S. (2016) Muscle strength in children with pulmonary arterial hypertension : A new parameter for disease severity? thesis, Medicine.
Full text available on request.Abstract
Introduction: Pulmonary arterial hypertension (PAH) is a rare, severe disease of the small pulmonary arteries and has a poor prognosis. Recently, it was shown that muscle strength is decreased in adults with PAH. The aim of this study was to examine the muscle strength in children with PAH and to explore its correlation with disease severity. Methods: This is a prospective study, including children diagnosed with PAH aged 4-18 years. Disease severity was evaluated using the clinical parameters World Health Organization Functional Class (WHO-FC) and 6-minute walking distance (6MWD) and the biochemical parameters NT-proBNP and uric acid. If elevated, the latter two indicate the presence of heart failure. Regarding muscle strength, maximum voluntary isometric contractility (MVIC) (of the elbow extensor and flexor, hip flexor and knee extensor), grip strength and functional strength were measured. These measurements were compared with age and sex matched reference values. Results: Eighteen children were included. There was a slight female predominance (67% females) and median age was 14 years (IQR, 9.9 – 16 yrs). Most children were in WHO-FC II (56%) and mean 6MWD was 430±82.3 meters. Plasma levels of NT-proBNP or uric acid were not severely elevated in this cohort. Muscle strength was significantly lower than the mean reference values. There were various significant correlations (p<0.05) between the muscle strength measurements and the clinical parameters for disease severity: MVIC of the elbow extensor (r=0.688), grip strength (r=0.587) and functional strength (r=0.520) with 6MWD, and functional strength with WHO-FC (r=-0.602). Conclusion: Muscle strength is decreased in children with PAH, also in the absence of heart failure. The decreased muscle strength seems to contribute to exercise intolerance. More research is needed to further investigate the relation between muscle strength and disease severity.
Item Type: | Thesis (Thesis) |
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Supervisor name: | Supervisors: and 1st supervisor: and Berger, R.M.F. MD PhD and 2nd supervisor: and Zijlstra, W.M.H. MD and Department of Pediatric Cardiology, Beatrix Children’s Hospi and University Medical Center Groningen, Groningen |
Faculty: | Medical Sciences |
Date Deposited: | 25 Jun 2020 10:52 |
Last Modified: | 25 Jun 2020 10:52 |
URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/1285 |
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