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Little information is available about the effects of inspiratory muscle training in patients with cystic fibrosis (CF). In this study the effects of inspiratory-threshold loading in patients with CF on strength and endurance of the inspiratory muscles, pulmonary function, exercise capacity, dyspnoea and fatigue were evaluated. Sixteen patients were assigned to one of two groups using the minimization method: eight patients in the training group and eight patients in the control group. The training was performed using an inspiratory-threshold loading device. Patients were instructed to use the threshold trainer 20 min a day, 5 days a week for 6 weeks. Patients in the training group trained at inspiratory threshold loads up to 40% of maximal static inspiratory pressure (Pimax) and patients in the control group got 'sham' training at a load of 10% of Pimax. No significant differences were found among the two groups in gender, age, weight, height, pulmonary function, exercise capacity, inspiratory-muscle strength and inspiratory-muscle endurance before starting the training programme. Mean (SD) age in the control group was 19 (5.5) years, mean (SD) age in the training group was 17 (5.2) years. Mean FEV1 in both groups was 70% predicted, mean inspiratory-muscle strength in both groups was above 100% predicted. All patients except one, assigned to the training group, completed the programme. After 6 weeks of training, mean inspiratory-muscle endurance (% Pimax) in the control group increased from 50% to 54% (P = 0.197); in the training group mean inspiratory muscle endurance (% Pimax) increased from 49% to 66% (P = 0.003). Statistical analysis showed that the change in inspiratory-muscle endurance (% Pimax) in the training group was significantly higher than in the control group (P = 0.012). After training, in the training group there was a tendency of improvement in Pimax with an increase from 105 to 123% predicted, which just fell short of statistical significance (P = 0.064). After training no significant differences were found in changes from baseline in pulmonary function, exercise capacity, dyspnoea and fatigue. It is concluded that low-intensity inspiratory-threshold loading at 40% of Pimax was sufficient to elicit an increased inspiratory-muscle endurance in patients with CF.
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PMID:Inspiratory muscle training in patients with cystic fibrosis. 1120 14

Conditions leading to chronic pulmonary insufficiency can affect infants and children. These can lead to growth failure and delayed development. Among the most common and severe of these are bronchopulmonary dysplasia (BPD) and cystic fibrosis. In addition to the respiratory consequences of these diseases, there is ample evidence that they lead to decreased growth as a result of decreased energy intake and increased energy expenditure. Furthermore, there is evidence that infants with BPD may also have delayed development, independent of the effects of their prematurity. Enhancing the long-term outlook for these conditions may therefore require consideration of both improved pulmonary management and aggressive nutritional management to limit growth failure and potentially enhance developmental outcome. Specific micronutrient supplementation, such as antioxidant therapy, may also enhance pulmonary and nutritional status.
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PMID:Chronic pulmonary insufficiency in children and its effects on growth and development. 1123 90

Dyspnea is a common problem among patients with interstitial fibrosis, lung cancer, cystic fibrosis, and chronic obstructive pulmonary disease. The slow but steady progression of such diseases, often punctuated by acute exacerbations or secondary illnesses, can lead to decision-making dilemmas among patients and their caregivers, such as when to accept mechanical ventilation, when to forgoe aggressive therapies, and when to make formal end-of-life care plans. Two cases, a 74-year-old woman with dyspnea secondary to emphysema and a 65-year-old woman with recurrent lung cancer and severe exertional fatigue and dyspnea, illustrate how dyspneic patients approaching the end of life can be evaluated and treated. Four management strategies for dyspnea are discussed: reducing ventilatory impedance, reducing ventilatory demand, improving respiratory muscle function, and altering central perception. Physicians should encourage end-stage lung disease patients and their families to discuss issues such as hospitalization and mechanical ventilation, to prepare advance directives, and to participate in a plan to manage their dyspnea.
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PMID:Perspectives on care at the close of life. Management of dyspnea in patients with far-advanced lung disease: "once I lose it, it's kind of hard to catch it... ". 1198 May 26

Congenital heart disease can increase or decrease pulmonary blood flow, pulmonary vascular resistance (PVR) or pulmonary artery pressure (PAP). PAP is the product of PVR and pulmonary minute volume (Qp), such that pulmonary hypertension (PHT) may develop as a result of an increase in either PVR or Qp or both. Given that the pulmonary vascular bed is a low pressure system with high flow, any increase in resistance would generate PHT. The normal value of PVR is 2 Woods units (mm Hg/l/min). Increased PAP is due to hypoxic lesions of the endothelium, which release proteolytic enzymes that alter the balance of metabolites of arachidonic acid, regulators of pulmonary vasomotor tone. Hypoxia and acidosis cause intense pulmonary vasoconstriction (hypoxic vasoconstrictor reflex). An increase of PVR is due to a combination of vasoconstrictive processes and remodeling, with hypertrophy of the pulmonary artery. Structural lesions are related to hypertrophy of the endothelium, the transformation of fibroblasts to myocytes and the decrease of the alveolar/arteriolar ratio with the formation of new vessels.PHT may be primary or secondary to another disease. Primary PHT is a rare genetic disease. The most common secondary forms of PHT in pediatrics are due to persistence of neonatal anatomy (neonatal PHT), to heart diseases with left-right shunt (CIV, DAP, etc.), to diseases of the pulmonary parenchyma (interstitial viral infection, mucoviscidosis), and complications of heart surgery. All congenital heart diseases can lead to PHT if not treated promptly. Clinical signs of PHT are highly non-specific: dyspnea, fatigue, syncopes, exercise intolerance, precordialgia, cyanosis and edema. The best approaches to diagnosis and prognosis are echocardiography and cardiac catheterization with vasodilators. Anesthetics that do not alter PVR should be used in such patients, who are sensitive to changes in pulmonary ventilation, to changes in cardiac output and to anesthetics. The treatment of PHT during intra and postoperative pediatric surgery is based on the use of high inspirated oxygen concentration (100%), an adequate sedation and the use of vasodilators (prostaglandin I2, nitric oxide, sodium nitroprusiate and milrinone).
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PMID:[Pulmonary hypertension in pediatric heart surgery]. 1179 1

It was hypothesized that adult cystic fibrosis (CF) patients with severe lung disease have impaired daytime function related to nocturnal hypoxaemia and sleep disruption. Nineteen CF patients (forced expiratory volume in one second 28+/-7% predicted) and 10 healthy subjects completed sleep diaries, overnight polysomnography (PSG), and assessment of daytime sleepiness and neurocognitive function. CF patients tended to report more awakenings (0.7+/-0.5 versus 0.3+/-0.2 x h(-1), p=0.08), and PSG revealed reduced sleep efficiency (71+/-25 versus 93+/-4%, p=0.004) and a higher frequency of awakenings (4.2+/-2.7 versus 2.4+/-1.4 x h(-1), p=0.06). Mean arterial oxygen saturation during sleep was lower in CF patients (84.4+/-6.8 versus 94.3+/-1.5%, p<0.0001) and was associated with reduced sleep efficiency (regression coefficient (r)=0.57, p=0.014). CF patients had short sleep latency on the multiple sleep latency test (6.7+/-3 min). The CF group reported lower levels of activation and happiness and greater levels of fatigue (p<0.01), which correlated with indices of sleep loss, such as sleep efficiency (r=0.47, p=10.05). Objective neurocognitive performance was also impaired in CF patients, reflected by lower throughput for simple addition/subtraction, serial reaction and colour-word conflict. The authors concluded that adult cystic fibrosis patients with severe lung disease have impaired neurocognitive function and daytime sleepiness, which is partly related to chronic sleep loss and nocturnal hypoxaemia.
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PMID:Sleep quality and daytime function in adults with cystic fibrosis and severe lung disease. 1193 30

Appropriate, regular exercise is of benefit to patients with cystic fibrosis (CF). As with other segments of the population, it has been difficult to devise exercise programs to which most patients will adhere for long periods of time. In healthy children, factors that are related to positive exercise compliance include social support, perceptions of competency and self-esteem, enjoyment of activity, and availability of a variety of activities. In patients with CF, complications associated with the disease, e.g., time required for other treatment and fatigue, make compliance with recommended exercise activities more difficult. Factors likely to increase compliance in this population include explicit and continued encouragement and support from the family and healthcare team, and the introduction of behavior-changing strategies.
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PMID:Factors that influence adherence to exercise and their effectiveness: application to cystic fibrosis. 1211

Malnutrition is a common complication of chronic diseases in children and may lead to growth impairment (stunting). Malnutrition in cystic fibrosis (CF) results from increased energy expenditure, decreased energy intakes, malabsorption of ingested nutrients because of pancreatic insufficiency and chronic inflammation. Malnutrition and high levels of inflammatory cytokines affect IGF-1 production through interrelated mechanisms. Nutritional support was shown to improve both nutritional status and outcome in CF. However, some nutrients have a direct effect on the disease. n-3 fatty acids supplementation is able to correct lipid abnormalities resulting from a primary mechanism. Moreover, n-3 fatty acids have a direct effect on the inflammatory response, decreasing eicosanoid synthesis and modulating nuclear transcriptional factors nuclear factor kappaB and peroxisome proliferator-activated receptors gamma. Nutritional support may be considered part of the care of the CF patient together with antibiotics, pancreatic enzymes and physiotherapy, influencing significantly the evolution of the disease.
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PMID:Nutrition and growth in cystic fibrosis. 1237 8

It is unclear whether a relationship between physical activity (PA) and maximal oxygen uptake (V'(O2,max)) exists in cystic fibrosis (CF) and, if so, whether the relationship reflects a direct effect or is mediated by the effects of confounding variables, such as pulmonary or muscle function. The objective of the present study was to determine the relationship between PA and V'(O2,max) in CF while adjusting for possible influences of confounding factors. In total, 36 female and 35 male patients with CF from Germany and Switzerland (aged 12-40 yrs, forced expiratory volume in one second (FEV1) 25-107% predicted) were studied. A Wingate test was employed to measure muscle power. PA was monitored for 7 days and expressed in two ways: 1) average daily accelerometer count (ADAC) and 2) time spent in moderate-to-vigorous PA (MVPA). V'(O2,max) was determined during an incremental cycle exercise test to volitional fatigue. PA was positively related to V'(O2,max). In a multiple linear regression analysis, height, sex, FEV1, muscle power and ADAC (additionally explained variance 2.5%) or time spent in MVPA (additionally explained variance 3.7%) were identified as independent predictors of V'(O2,max). In conclusion, high levels of physical activity in addition to good muscular and pulmonary functions are associated with a high aerobic capacity in cystic fibrosis.
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PMID:Physical activity is independently related to aerobic capacity in cystic fibrosis. 1701 24

Bronchiectasis is generally classified into cystic fibrosis and non-cystic fibrosis bronchiectasis. This review article describes non-cystic fibrosis bronchiectasis in adults. Bronchiectasis can be considered a heterogeneous condition characterized by irreversible airway dilatation with chronic bronchial infection/inflammation. It remains a common condition and is a major cause of respiratory morbidity. Many factors are associated with bronchiectasis, but most commonly patients will have idiopathic disease. Important clinical findings include chronic productive cough, rhinosinusitis, fatigue and bi-basal crackles. Patients have usually had symptoms for many years. Diagnosis is confirmed by high-resolution computed tomography scanning using standardized criteria. Spirometry shows moderate airflow obstruction and there is a high prevalence of bronchial hyperreactivity. The most common pathogens are non-typeable Haemophilus influenzae and Pseudomonas aeruginosa. There may be considerable overlap with other chronic airway diseases. Treatment regimens are still not well defined. Patients tend to have ongoing symptoms and decline in respiratory function despite treatment.
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PMID:Bronchiectasis. 1731 49

Symptom burden is a key component of health-related quality of life in patients with cystic fibrosis (CF). To examine symptom prevalence and characteristics of adults with CF, we administered the Memorial Symptom Assessment Scale (MSAS), a previously validated measure of symptom burden, to CF patients enrolled in the Project on Adult Care in CF. The mean age of the 303 respondents (response rate 91%) was 32.8 years (range, 19-64); 58% were female, and their mean baseline pulmonary function (FEV(1) % predicted) was 69% (SD 28%). The median number of symptoms reported was 10, and there was no difference in the number of symptoms reported based on age, gender, or FEV(1). The most prevalent symptoms were cough (94%), shortness of breath (77%), and lack of energy (77%). Lack of energy and irritability caused the highest level of distress. MSAS symptom subscales were only moderately correlated with symptom status domains from existing CF health-related quality of life measures. Factor analysis led to the development of three distinct MSAS CF-symptom subscales, each with high internal validity. These findings show that adults with CF have a high symptom burden, particularly with respiratory and psychological symptoms, and that the new MSAS CF-specific subscales are a reliable measure of symptom distress in the CF population.
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PMID:Self-reported physical and psychological symptom burden in adults with cystic fibrosis. 1821 97


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