Inspiratory Muscle Training in Late-Onset Pompe Disease: The Effects on Pulmonary Function Tests, Quality of Life, and Sleep Quality.

INTRODUCTION: Late-onset Pompe disease (LOPD) is characterized by progressive skeletal and respiratory muscle weakness. Little is known about the effect of inspiratory muscle training (IMT) on pulmonary function in subjects with LOPD. The aim of the present study was to investigate the effect of an 8-week IMT program on pulmonary function tests, quality of life, and sleep quality in eight patients with LOPD who were receiving enzyme replacement therapy (ERT). METHODS: Before and after the IMT program, spirometric measurements in sitting and supine positions, and measurements of maximum inspiratory and expiratory pressures, peak cough flow, quality of life (assessed using the Nottingham Health Profile), and sleep quality (assessed using the Pittsburgh sleep quality index) were performed. RESULTS: A significant increase in maximum inspiratory pressure (cmH2O and % predicted) (median [interquartile range]: 30.0 cmH2O [21.5-48] versus 39 cmH2O [31.2-56.5] and 38.3 % [28.1-48.4] versus 50.5 % [37.7-54.9]) was observed after training (p = 0.01). There were no significant changes in the other pulmonary function measurements. With the exception of the social isolation subscore (p = 0.02), quality of life subscores did not change after IMT (p > 0.05). Sleep quality subscores and total scores were similar before and after IMT. CONCLUSION: These results suggest that IMT has a positive effect on maximum inspiratory pressure in subjects with LOPD who are under ERT.

Nebulized Lidocaine as an Alternative Therapy for Reactive Airway Dysfunction Syndrome.

Reactive airway dysfunction syndrome (RADS) is a variant of irritant-induced asthma that develops in subjects without prior bronchoobstructive disease, following high-level exposure to nonimmunogenic irritants. Recommended maintenance treatment for RADS is not different from asthma. But in some cases, severe symptoms may persist despite the bronchodilators and corticosteroids. We describe the first case of a patient with RADS, unresponsive to all medical agents, who was successfully treated with lidocaine.

Effects of respiratory muscle training on pulmonary functions in patients with slowly progressive neuromuscular disease: a randomized controlled trial.

OBJECTIVE: To investigate the effects of inspiratory and expiratory muscle training on pulmonary functions in patients with slowly progressive neuromuscular disease. DESIGN: Prospective randomized controlled double-blinded study. SETTING: Chest diseases clinic of university hospital. SUBJECTS: Twenty-six patients with slowly progressive neuromuscular disease followed for respiratory problems were included in the study. Patients were randomly divided into two groups; experimental (n = 14; age 31.6 ±12.3 years) and sham (n = 12; age 26.5 ±8.6 years) groups. METHODS: Spirometry, peak cough flow, maximal inspiratory pressure, maximal expiratory pressure, and sniff nasal inspiratory pressure were measured before the eighth week of study, and subsequently at end of it. Respiratory muscle training was performed by inspiratory (Threshold Inspiratory Muscle Trainer) and expiratory (Threshold Positive Expiratory Pressure) threshold loading methods. Training intensities were increased according to maximal inspiratory and expiratory pressures in the experimental group, while the lowest loads were used for training in the sham group. Patients performed 15 minutes inspiratory muscle training and 15 minutes expiratory muscle training, twice a day, five days/week, for a total of eight weeks at home. Training intensity was adjusted in the training group once a week. RESULTS: Maximal inspiratory and expiratory pressures (cmH2O, % predicted) (respectively p = 0.002, p = 0.003, p = 0.04, p = 0.03) and sniff nasal inspiratory pressure (p = 0.04) were improved in the experimental group when compared with the sham group. However, there was no improvement in spirometric measurements when groups were compared (p > 0.05). CONCLUSIONS: As a conclusion of our study, we found that respiratory muscle strength improved by inspiratory and expiratory muscle training in patients with slowly progressive neuromuscular disease.

Effects of simvastatin on bleomycin-induced pulmonary fibrosis in female rats.

Statins reduce cholesterol levels by inhibiting 3-hydroxy-3-methylglutaryl coenzyme A reductase and have a major place in the treatment of atherosclerotic disease. Recent studies have shown anti-inflammatory properties of statins. The purpose of this study was to evaluate the anti-inflammatory effect of simvastatin on bleomycin (BLM)-induced pulmonary fibrosis in rats. A total of 31 female Sprague-Dawley rats were divided into four groups: (1) intratracheal (IT) phosphate-buffered saline (PBS) + intraperitoneal (IP) PBS (n=7); (2) IT BLM + IP PBS (n=8); (3) IT BLM + low dose (LD) simvastatin (1 mg/kg daily, n=8); (4) IT BLM + high dose (HD) simvastatin (5 mg/kg daily, n=8). Simvastatin was administered IP for 15 days, beginning 1 day prior to IT BLM. The effect of simvastatin on pulmonary fibrosis was studied by measurements of IL-13, PDGF, IFN-γ, TGF-p1 levels in bronchoalveolar lavage (BAL) fluid and lung tissue hydroxyproline (HPL) content and by histopathological examination (Ashcroft score). BLM caused significant change in BAL fluid cytokine levels and increased both HPL content and histopathological score (p<0.001 for all). While LD simvastatin had no effect on cytokine levels, HD significantly reduced IL-13 (15.12 ±7.08 pg/ml vs. 4.43±2.34 pg/mL; p<0.05) and TGF-ß1 levels (269.25 ±65.42 pg/mL vs. 131.75±32.65 pg/mL; p<0.05). Neither HD nor LD simvastatin attenuated HPL content or Ashcroft score. In conclusion, this study showed that LD simvastatin had no effect on a BLM-induced pulmonary fibrosis model, while the high dose caused partial improvement in profibrotic cytokine levels.

Effects of simvastatin on bleomycin-induced pulmonary fibrosis in female rats

Statins reduce cholesterol levels by inhibiting 3-hydroxy-3-methylglutaryl coenzyme A reductase and have a major place in the treatment of atherosclerotic disease. Recent studies have shown anti-inflammatory properties of statins. The purpose of this study was to evaluate the anti-inflammatory effect of simvastatin on bleomycin (BLM)-induced pulmonary fibrosis in rats. A total of 31 female Sprague-Dawley rats were divided into four groups: (1) intratracheal (IT) phosphate-buffered saline (PBS) + intraperitoneal (IP) PBS (n=7); (2) IT BLM + IP PBS (n=8); (3) IT BLM + low dose (LD) simvastatin (1 mg/kg daily, n=8); (4) IT BLM + high dose (HD) simvastatin (5 mg/kg daily, n=8). Simvastatin was administered IP for 15 days, beginning 1 day prior to IT BLM. The effect of simvastatin on pulmonary fibrosis was studied by measurements of IL-13, PDGF, IFN-γ, TGF-p1 levels in bronchoalveolar lavage (BAL) fluid and lung tissue hydroxyproline (HPL) content and by histopathological examination (Ashcroft score). BLM caused significant change in BAL fluid cytokine levels and increased both HPL content and histopathological score (p<0.001 for all). While LD simvastatin had no effect on cytokine levels, HD significantly reduced IL-13 (15.12 ±7.08 pg/ml vs. 4.43±2.34 pg/mL; p<0.05) and TGF-β1 levels (269.25 ±65.42 pg/mL vs. 131.75±32.65 pg/mL; p<0.05). Neither HD nor LD simvastatin attenuated HPL content or Ashcroft score. In conclusion, this study showed that LD simvastatin had no effect on a BLM-induced pulmonary fibrosis model, while the high dose caused partial improvement in profibrotic cytokine levels.