Journal of applied physiology
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The validity and safety of using supramaximal verification (Smax) to confirm a maximal effort during cardiopulmonary exercise testing (CPET) in people with cystic fibrosis (CF) and/or those with severe disease has been questioned. Therefore, this study aimed to investigate these concerns in children, adolescents, and adults with mild-to-severe CF lung disease. Retrospective analysis of 17 pediatric and 28 adult participants with CF [age range: 9.2-62.9 y; forced expiratory volume in 1 s: 66.7% (range: 29.9%-102.3%); 30 men] who completed a routine ramp-incremental cycling test to determine peak oxygen uptake (V̇o2peak) was studied. ⋯ Given the clinical importance of aerobic fitness in people with CF, incorporating Smax is recommended to provide a safe and valid measure of V̇o2max in children, adolescents, and adults who span the spectrum of CF disease severity. NEW & NOTEWORTHY Incorporating supramaximal verification into cardiopulmonary exercise testing protocols did not increase the frequency of adverse events or perceived discomfort versus a single-phase incremental exercise test in people with mild-to-severe cystic fibrosis. Furthermore, a valid measure of maximal oxygen uptake (V̇o2max) was obtained from 85.7% of pediatric and 96.4% of adult exercise tests, whereas peak oxygen uptake underestimated aerobic fitness in comparison with V̇o2max in 21.4% of cases (by up to 24.4%).
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Neurogenic orthostatic hypotension (NOH) is a cardinal feature of autonomic dysfunction. The cortical autonomic network (CAN) is a network of brain regions associated with autonomic function. Therefore, our objective was to investigate whether impairment of CAN structures is involved in the pathophysiology of NOH. ⋯ Our result reveal that patients with autonomic dysfunction caused by postganglionic sympathetic impairment also have impaired activation of structures within the cortical autonomic network. Impaired activation is evident during a test of sympathetic, but not parasympathetic, activation. Impaired cortical autonomic networks associated with sympathetic activation may be involved in the pathophysiology of NOH.