Validity and Reliability of the Turkish Version of the Nijmegen Questionnaire in Asthma.

OBJECTIVE: The Nijmegen Questionnaire (NQ) enables the assessment and identification of symptoms related to respiratory dysfunction and hyperventilation syndrome. The aim was to investigate the validity of the Turkish version of the NQ in asthmatics. MATERIAL AND METHODS: Fifty-four individuals with asthma were included. Spirometry was performed. Dyspnea was assessed using the modified Borg and modified Medical Research Council scales. Breath-holding time was recorded. End-tidal carbon dioxide was measured using a portable capnograph. Oxygen saturation and heart rate were recorded. Asthma Control Test was used to evaluate the asthma control level. Quality of life was assessed using the Asthma Quality of Life Questionnaire and Nottingham Health Profile. Beck Depression Inventory was used to determine depression. RESULTS: Bartlett's test of sphericity (360.749, df 105, P < .001) and Kaiser-Meyer-Olkin criterion (0.752) for 15-item NQ supported a single-factor model with 36.38% of explained variability through principal component analysis and explanatory factor analysis. For 15-item NQ with this single-factor model, Cronbach's alpha was 0.872, and the test-retest reliability was 0.628. There was a significant negative correlation between NQ and Asthma Control Test (r = -0.448), and Asthma Quality of Life Questionnaire (r = -0.743) and a significant positive association with Beck Depression Inventory (r = 0.477), Nottingham Health Profile-energy (r = 0.370), Nottingham Health Profile-pain (r = 0.313), Nottingham Health Profile-sleep (r = 0.294), and Nottingham Health Profile-physical activity scores (r = 0.406) (P < .05). CONCLUSIONS: The 15-item Turkish version of the NQ is valid and reliable in asthmatics. Individuals with uncontrolled asthma have higher NQ scores than those with well-controlled asthma. NQ is associated with asthma control level, asthma-related quality of life, health profile, and depression.

Reduced anaerobic and aerobic performance in children with primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) restricts lifestyle and increases morbidity. The aim of the study was to investigate anaerobic and aerobic performance in children with PCD and their healthy counterparts. Thirty-one children with PCD and 29 age- and sex-matched healthy subjects were studied. Pulmonary function, hand grip strength (HGS), quadriceps strength (QMS), physical activity, anaerobic capacity (muscle power sprint test), and aerobic performance (modified shuttle walk test (MSWT)) were determined. Pulmonary function, HGS, QMS, mean anaerobic power (MAP), and MSWT distance in PCD were significantly lower than those of healthy subjects (p < 0.05). In PCD, the MAP was significantly correlated with age, FEV1, and the mean kcal for 3 days (p < 0.05), and age was its independent predictor (p < 0.05). The MSWT distance was significantly related to gender and weight (p < 0.05), and gender was selected as its independent predictor (p < 0.05). In healthy controls, the MAP was significantly associated with age, gender, FVC, FEV1, HGS, QMS, and the mean kcal for three days (p < 0.05). The MSWT distance was significantly related to weight and body mass index in healthy group (p < 0.05). CONCLUSION: Anaerobic and aerobic performance is impaired in PCD from the early stages. Age determines anaerobic performance. Gender is the determinant of aerobic performance. Whether skeletal muscle characteristics and sex-related changes in body composition affect anaerobic and aerobic capacity in PCD children warrants further study. What is Known: • Exercise performance is determined by anaerobic and aerobic power. • Few studies have shown that PCD patients have lower aerobic performance which is associated with impaired lung function. What is New: • The present research indicated that both anaerobic and aerobic exercise capacity determined using field testing is impaired in PCD from the early stages. • Anaerobic capacity was found to be independently associated with age in PCD. Higher aerobic performance is independently associated with male gender.

Modeling Cystic Fibrosis Using Pluripotent Stem Cell-Derived Human Pancreatic Ductal Epithelial Cells.

UNLABELLED: We established an efficient strategy to direct human pluripotent stem cells, including human embryonic stem cells (hESCs) and an induced pluripotent stem cell (iPSC) line derived from patients with cystic fibrosis, to differentiate into pancreatic ductal epithelial cells (PDECs). After purification, more than 98% of hESC-derived PDECs expressed functional cystic fibrosis transmembrane conductance regulator (CFTR) protein. In addition, iPSC lines were derived from a patient with CF carrying compound frameshift and mRNA splicing mutations and were differentiated to PDECs. PDECs derived from Weill Cornell cystic fibrosis (WCCF)-iPSCs showed defective expression of mature CFTR protein and impaired chloride ion channel activity, recapitulating functional defects of patients with CF at the cellular level. These studies provide a new methodology to derive pure PDECs expressing CFTR and establish a "disease in a dish" platform to identify drug candidates to rescue the pancreatic defects of patients with CF. SIGNIFICANCE: An efficient strategy was established to direct human pluripotent stem cells, including human embryonic stem cells (hESCs) and an induced pluripotent stem cell line derived from patients with cystic fibrosis (CF-iPSCs), to differentiate into pancreatic ductal epithelial cells (PDECs). After purification, more than 98% of hESC-PDECs derived from CF-iPSCs showed defective expression of mature cystic fibrosis transmembrane conductance regulator (CFTR) protein and impaired chloride ion channel activity, recapitulating functional pancreatic defects of patients with CF at the cellular level. These studies provide a new methodology for deriving pure PDECs expressing CFTR, and they establish a "disease-in-a-dish" platform for identifying drug candidates to rescue the pancreatic defects of these patients.