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Forced Vital Capacity (forced + vital_capacity)
Selected AbstractsDecline of neuroadrenergic bronchial innervation and respiratory function in type 1 diabetes mellitus: a longitudinal studyDIABETES/METABOLISM: RESEARCH AND REVIEWS, Issue 4 2007Raffaele Antonelli Incalzi Abstract Background and aim Type 1 diabetes mellitus complicated by autonomic neuropathy (AN) is characterized by depressed cholinergic bronchomotor tone and neuroadrenergic denervation of the lung. We explored the effects of AN on the rate of decline of pulmonary sympathetic innervation and respiratory function during a 5-year follow-up. Methods Twenty diabetic patients, 11 with AN, were enrolled in 1998 and then followed-up until 2003. During follow-up, glycosylated haemoglobin (HbA1c) was measured every 3 months. In 1998 and 2003 the patients underwent respiratory function tests and a ventilatory scintigraphic study of neuroadrenergic bronchial innervation using 123I-MIBG. Results During follow-up 4 patients, all with AN, were lost, and 1 developed AN. Forced vital capacity (FVC), and diffusing capacity of the lung for carbon monoxide (DLCO) showed comparable rates of decrease in patients with and without AN. The yearly decline of forced expiratory volume in 1 s (FEV1) was about double the physiologic rate, in both AN and AN-free patients. The MIBG clearance significantly increased both in patients with AN (T1/2: 118.88 ± 30.14 min at baseline and 92.10 ± 24.52 min at the end of follow-up) and without AN (135.14 ± 17.09 min and 92.68 ± 13.52 min, respectively), indicating a rapidly progressive neuroadrenergic denervation. The rate of the neuroadrenergic denervation was inversely related to the severity of autonomic dysfunction at baseline (Spearman's rho , 0.62, p = 0.017). Neither respiratory function indexes nor MIBG clearance changes correlated with the overall HbA1c values. Conclusions Neuroadrenergic denervation of the lung parallels the decline of respiratory function indexes in diabetic patients both with and without AN and seems to be independent from the quality of glycemic control. Copyright © 2006 John Wiley & Sons, Ltd. [source] Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis,ARTHRITIS & RHEUMATISM, Issue 7 2010J. R. Seibold Objective Endothelin is implicated as a participatory pathway in systemic sclerosis (SSc). We tested this hypothesis in a 12-month trial of bosentan, a nonselective endothelin receptor antagonist, as a therapy for SSc-related interstitial lung disease (ILD). Method Patients with SSc and significant ILD were recruited to this prospective, double-blind, randomized, placebo-controlled, parallel group study. The inclusion criteria were designed to select a cohort enriched for patients with active and progressive disease. Exclusion factors included significant pulmonary hypertension. Patients with a diffusing capacity for carbon monoxide of <80% predicted and a 6-minute walk distance of 150,500 meters or a 6-minute walk distance of ,500 meters with a decrease in oxygen saturation received bosentan or placebo. The primary efficacy end point was a change in the 6-minute walk distance from baseline up to month 12. Secondary end points included time to death or worsening results of pulmonary function tests (PFTs). The safety and tolerability of bosentan were also assessed. Results Among the 163 patients, 77 were randomized to receive bosentan, and 86 were randomized to receive placebo. No significant difference between treatment groups was observed for change in the 6-minute walk distance up to month 12. No deaths occurred in this study group. Forced vital capacity and diffusing capacity for carbon monoxide remained stable in the majority of patients in both groups. Significant worsening of PFT results occurred in 25.6% of patients receiving placebo and 22.5% of those receiving bosentan (P not significant). Conclusion No improvement in exercise capacity was observed in the bosentan-treated group compared with the placebo group, and no significant treatment effect was observed for the other end points. Although many outcome variables were stable, bosentan did not reduce the frequency of clinically important worsening. These data do not support the use of endothelin receptor antagonists as therapy for ILD secondary to SSc. [source] Pulmonary function tests and diaphragmatic compound muscle action potential in patients with sporadic amyotrophic lateral sclerosisACTA NEUROLOGICA SCANDINAVICA, Issue 6 2010T. N. Sathyaprabha Sathyaprabha TN, Pradhan C, Nalini A, Thennarasu K, Raju TR. Pulmonary function tests and diaphragmatic compound muscle action potential in patients with sporadic amyotrophic lateral sclerosis. Acta Neurol Scand: 2010: 121: 400,405. © 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard. Background,,, Respiratory failure is the primary cause of death in patients with amyotrophic lateral sclerosis (ALS). Diaphragmatic compound muscle action potentials (DCMAP) are valid parameters to assess the respiratory muscle innervation. Aim,,, In this study we propose to establish evidence of pulmonary dysfunction in patients with ALS and its relation to DCMAP parameters among patients with sporadic ALS. Materials and methods,,, Twenty nine patients (M-20, F-9) diagnosed to have sporadic ALS by El. Escorial criteria, without symptoms of pulmonary dysfunction, and able to perform the PFT satisfactorily, were studied. Thirty controls (M-20, F-10) were selected from patient's relatives. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), peak expiratory flow rate (PEFR) and maximum voluntary ventilation (MVV) were measured by spirometry. Maximum expiratory pressure (MEP) was measured by digital peak pressure monitor. Right phrenic nerve conductions (DCMAP) were performed and the latencies and amplitude of diaphragmatic com-pound action potential (DCMAP) was recorded in controls and ALS patients. Results,,, The mean age of patients was 51.41 ± 10.72 years (37,82) and control was 53.57 ± 8.85 years (30,68). None of the patients had symptoms or clinical evidence of respiratory dysfunction. The FVC, FEV1, PEFR, MVV, MIP and MEP were significantly (P < 0.001) reduced in ALS. The mean DCMAP amplitude was reduced among patients (610 ± 506.231 ,v) as compared to controls (1303.33 ± 584.56, P < 0.001) and mean latency was increased in patients (9.73 ± 2.57 ms) compared to controls (7.69 ± 0.87, P = 0.001). There was significant negative correlation between PFTs and latencies of DCMAP. Amplitude of DCMAP did not correlate with PFTs. Conclusion,,, There is significant negative correlation between DCMAP latencies and PFTs suggesting early loss of myelinated fibres and diaphragmatic dysfunction. DCMAP latencies may be a good indicator of early respiratory muscle involvement and also of disease progression in ALS. [source] Association of findings in flow-volume spirometry with high-resolution computed tomography signs in asbestos-exposed male workersCLINICAL PHYSIOLOGY AND FUNCTIONAL IMAGING, Issue 1 2009Päivi Piirilä Summary Introduction:, Disorders of pulmonary tissue and pleura are visualized by findings in high-resolution computed tomography (HRCT), and the impairment caused by these findings is assessed by pulmonary function tests. Our aim was to determine how some commonly used spirometric variables are related to certain HRCT signs, in order to find out which HRCT signs are associated with restrictive and which with obstructive ventilatory impairment. Methods:, Altogether 590 asbestos-exposed workers, 95% of whom were smokers or ex-smokers, were studied with HRCT; 19 pathological signs were scored. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), FEV1/FVC ratio, forced expiratory flow at 50% of FVC (MEF50) and total lung capacity (TLC) were measured, and their relationship with HRCT signs was examined with bivariate correlations and multiple regression analysis. Results:, FVC and TLC were negatively correlated with fibrosis score, parenchymal bands, extent of pleural thickenings and positively with widened retrosternal space. FEV1/FVC ratio was negatively correlated with emphysema types and widened retrosternal space and positively with parenchymal bands and subpleural nodules. Thickened bronchial walls did not separate between restrictive and obstructive ventilatory function. Conclusions:, HRCT signs showed distinctive patterns in restrictive and obstructive ventilatory impairment. These results can be used to help to analyse the lung function of patients simultaneously exposed to asbestos and smoking, when this relationship requires elucidation. In addition, the results may be helpful in explaining some radiological findings. [source] A clinical pharmacological study of the potential beneficial effects of a propolis food product as an adjuvant in asthmatic patientsFUNDAMENTAL & CLINICAL PHARMACOLOGY, Issue 1 2003M. T. Khayyal Abstract The aqueous extract of propolis has been formulated as a nutritional food product and administered, as an adjuvant to therapy, to patients with mild to moderate asthma daily for 2 months in the framework of a comparative clinical study in parallel with a placebo preparation. The diagnosis of asthma was made according to the criteria of patient classification of the National Institutes of Health and Global Initiative for Asthma Management. At inclusion, the pulmonary forced expiratory volume in the first second (FEV1) as a percentage of the forced vital capacity (FVC) was more than 80% in mild persistent cases, and between 60 and 80% in moderate persistent cases, showing an increase in the degree of reversibility of >,15% in FEV1. All patients were on oral theophylline as controller therapy, none was receiving oral or inhaled corticosteroids, none had other comorbidities necessitating medical treatment, and all were from a middle-class community and had suffered from asthma for the last 2,5 years. Twenty-four patients received the placebo, with one drop-out during the study, while 22 received the propolis extract, with no drop-outs. The age range of the patients was 19,52 years; 36 were male and 10 female. The number of nocturnal attacks was recorded on a weekly basis, while pulmonary function tests were performed on all patients at the beginning of the trial, 1 month later and at the termination of the trial. Immunological parameters, including various cytokines and eicosanoids known to play a role in asthma, were measured in all patients at the beginning of the trial and 2 months later. Analysis of the results at the end of the clinical study revealed that patients receiving propolis showed a marked reduction in the incidence and severity of nocturnal attacks and improvement of ventilatory functions. The number of nocturnal attacks dropped from an average of 2.5 attacks per week to only 1. The improvement in pulmonary functions was manifested as a nearly 19% increase in FVC, a 29.5% increase in FEV1, a 30% increase in peak expiratory flow rate (PEFR), and a 41% increase in the forced expiratory flow rate between 25 and 75% of the vital capacity (FEF25-75). The clinical improvement was associated with decreases by 52, 65, 44 and 30%, respectively, of initial values for the pro-inflammatory cytokines tumor necrosis factor (TNF)-,, ICAM-1, interleukin (IL)-6 and IL-8, and a 3-fold increase in the ,protective' cytokine IL-10. The levels of prostaglandins E2 and F2, and leukotriene D4 were decreased significantly to 36, 39, and 28%, respectively, of initial values. Patients on the placebo preparation showed no significant improvement in ventilatory functions or in the levels of mediators. The findings suggest that the aqueous propolis extract tested is potentially effective as an adjuvant to therapy in asthmatic patients. The benefits may be related to the presence in the extract of caffeic acid derivatives and other active constituents. [source] How do COPD and healthy-lung patients tolerate the reduced volume ventilation strategy during OLV ventilation.ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 9 2010P. MICHELET Background: Although a strategy of tidal volume (Vt) reduction during the one-lung ventilation (OLV) period is advised in thoracic surgery, the influence of the pre-operative respiratory status on the tolerance of this strategy remains unknown. Therefore, the aim of this study was to compare the pulmonary function between chronic obstructive pulmonary disease (COPD) and healthy-lung patients during the operative and the post-operative period. Methods: Forty-eight patients undergoing a planned lobectomy for cancer and presenting either a healthy lung function (n=24) or a moderate COPD stage (n=24) were ventilated without external positive end-expiratory pressure (PEEP) and received 9 ml/kg Vt during the two-lung ventilation (TLV) period, secondary reduced to 6 ml/kg during the OLV period. Lung function was assessed by peroperative gas exchange, venous admixture, respiratory mechanical parameters and post-operative spirometric measurements. Results: Although the PaO2 was superior in the healthy-lung group during the TLV, once the OLV was established, no difference was observed between the two groups. Moreover, the PaO2/FiO2 was proportionally more impaired in the healthy-lung group compared with the COPD group (50 ± 13 vs. 72 ± 19% of the baseline values after exclusion and 32 ± 15 vs. 51 ± 25% after the thoracotomy, P<0.05 for each) as well as the venous admixture. In the post-operative period, a higher decrease was observed in the healthy-lung group for the forced vital capacity and the forced expiratory volume. Conclusions: Reducing Vt to 6 ml/kg without the adjunction of external PEEP during OLV is associated with better preservation of lung function in the case of moderate COPD than in the case of healthy-lung status. [source] Reduced lung function predicts increased fatality in future cardiac events.JOURNAL OF INTERNAL MEDICINE, Issue 6 2006A population-based study Abstract. Objective., Moderately reduced lung function in apparently healthy subjects has been associated with incidence of coronary events. However, whether lung function is related to the fatality of the future events is unknown. This study explored whether reduced forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) in initially healthy men is related to the fatality of the future coronary events. Design., Prospective cohort study. Setting., Population-based study from Malmö, Sweden. Subjects., A total of 5452 healthy men, 28,61 years of age. Main outcome measures., Incidence of first coronary events was monitored over a mean follow-up of 19 years. The fatality of the future events was studied in relation to FEV and FVC. Results., A total of 589 men suffered a coronary event during follow-up, 165 of them were fatal during the first day. After risk factors adjustment, low FEV or FVC were associated with incidence of coronary events (fatal or nonfatal) and this relationship was most pronounced for the fatal events. Amongst men who subsequently had a coronary event, the case-fatality rates were higher in men with low FEV or FVC. Adjusted for risk factors, the odds ratio for death during the first day was 1.00 (reference), 1.63 (95% CI: 0.9,3.1), 1.86 (1.0,3.5) and 2.06 (1.1,3.9), respectively, for men with FVC in the 4th, 3rd, 2nd, and lowest quartiles (trend: P < 0.05). FEV showed similar relationships with the fatality rates. Conclusion., Apparently healthy men with moderately reduced lung function have higher fatality in future coronary events, with a higher proportion of coronary heart disease deaths and less nonfatal myocardial infarction. [source] Comparison of perioperative spirometric data following spinal or general anaesthesia in normal-weight and overweight gynaecological patientsACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 7 2005B. S. Von Ungern-Sternberg Background:, There is limited data comparing the impact of spinal anaesthesia (SA) and general anaesthesia (GA) on perioperative lung function. Here we assessed the differences of these two anaesthetic techniques on perioperative lung volumes in normal-weight (BMI < 25) and overweight (BMI 25,30) patients using spirometry. Methods:, We prospectively studied 84 consenting patients having operations in the vaginal region receiving either GA (n = 41) or SA (n = 43). Both groups (GA and SA) were further divided into two subgroups each (normal-weight vs. overweight). We measured vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), midexpiratory (MEF25-75) and peak expiratory flow rates (PEFR) at the preoperative assessment (baseline), after premedication, after effective SA, and 20 min, 1 h, 2 h and 3 h after the operation (last measurement after patient mobilization). Results:, Premedication was associated with a small but significant decrease in lung volumes in direct correlation with BMI (,5%). Spinal anaesthesia resulted in a significant reduction in lung volumes in overweight as opposed to normal-weight patients. Postoperatively, lung volumes were significantly more reduced following GA than SA as indicated by differences in mean VC (SD) of ,12 (6)% vs. ,6 (5)% 20 min after the end of the operation in the normal-weight and ,18 (5)% vs. ,10 (5)% in the overweight patients. There was a significant impact of BMI on postoperative respiratory function, which was significantly more important in the GA group than in the SA group, and recovery of lung volumes was more rapid in the normal-weight patients than in the overweight patients, particularly in the SA group. Conclusion:, In gynaecological patients undergoing vaginal surgery, the impact of anaesthesia on postoperative lung function as assessed by spirometry was significantly less after SA than GA, particularly in overweight patients. [source] Comparison of roflumilast, an oral anti-inflammatory, with beclomethasone dipropionate in the treatment of persistent asthmaALLERGY, Issue 1 2006J. Bousquet Background:, Roflumilast is an oral, once-daily phosphodiesterase 4 inhibitor with anti-inflammatory activity in development for the treatment of asthma. Roflumilast was compared with inhaled beclomethasone dipropionate (BDP) in patients with asthma. Methods:, In a double blind, double-dummy, randomized, noninferiority study, 499 patients (forced expiratory volume in 1 s [FEV1] = 50,85% predicted) received roflumilast 500 ,g once daily or BDP 200 ,g twice daily (400 ,g/day) for 12 weeks. Lung function and adverse events were monitored. Results:, Roflumilast and BDP significantly improved FEV1 by 12% (270 ± 30 ml) and 14% (320 ± 30 ml), respectively (P < 0.0001 vs baseline). Roflumilast and BDP also significantly improved forced vital capacity (FVC) (P < 0.0001 vs baseline). There were no significant differences between roflumilast and BDP with regard to improvement in FEV1 and FVC. Roflumilast and BDP showed small improvements in median asthma symptom scores (,0.82 and ,1.00, respectively) and reduced rescue medication use (,1.00 and ,1.15 median puffs/day, respectively; P < 0.0001 vs baseline). These small differences between roflumilast and BDP were not considered clinically relevant. Both agents were well tolerated. Conclusions:, Once daily, oral roflumilast 500 ,g was comparable with inhaled twice-daily BDP (400 ,g/day) in improving pulmonary function and asthma symptoms, and reducing rescue medication use in patients with asthma. [source] Sensitivity of electrophysiological tests for upper and lower motor neuron dysfunction in ALS: A six-month longitudinal studyMUSCLE AND NERVE, Issue 2 2010Mamede de Carvalho MD Abstract By following a group of amyotrophic lateral sclerosis (ALS) patients longitudinally using lower motor neuron (LMN) and upper motor neuron (UMN) markers of dysfunction it may be possible to better understand the functional relationships between these motor systems in this disease. We used neurophysiological techniques to follow UMN and LMN dysfunction in a group of 28 patients with ALS, in comparison with the ALS functional rating scale (ALS-FRS) score and the forced vital capacity (FVC). We used motor unit number estimation (MUNE), compound muscle action potential (CMAP) amplitude, and the Neurophysiological Index (NI) to quantify the LMN disorder, and transcranial motor stimulation to study cortical motor threshold, motor-evoked response amplitude, central motor conduction time, and cortical silent period (CSP). The patients were studied shortly after diagnosis and then 6 months later, using both abductor digiti minimi muscles (ADM); ADM strength was initially >MRC 3 (Medical Research Council, UK). The NI and MUNE changed more than any other variable. CSP increased by about 30%, a change more marked than the slight increase observed in the cortical motor threshold (9%). The normal increase of CSP after acute muscle fatigue was preserved during disease progression. The CSP increase correlated with the MUNE rate of decay but not to the NI reduction, perhaps because NI includes F-wave frequency in itscalculation. There was no definite correlation between UMN and LMNdysfunction or progression, but there was a link between CSP and LMN changes in ALS. The CSP may be a useful variable in following UMN dysfunction in clinical practice and in clinical trials. Muscle Nerve, 2010 [source] Disease progression in amyotrophic lateral sclerosis: Predictors of survivalMUSCLE AND NERVE, Issue 5 2002T. Magnus MD Abstract Predicting the rate of disease progression has become important as trials of new medical treatments for amyotrophic lateral sclerosis (ALS) are planned. Bulbar onset, early impairment of forced vital capacity, and older age have all been associated with shorter survival. We performed a retrospective study to compare survival factors with disease progression in a German ALS population. We analyzed disease progression in 155 patients at intervals of 4 months over a period of 3 years. To evaluate disease progression, the ALS functional rating scale (ALS-FRS), forced vital capacity (FVC%), and a Medical Research Council (MRC) compound score based on a nine-step modified MRC scale were used. We compared age (<55 years vs. ,55 years), different sites of disease onset (bulbar vs. limb), and gender to the rate of disease progression and performed survival analyses. No overall significant difference could be detected when analyzing these subgroups with regard to disease progression. By contrast, significantly longer survival was observed in the younger age group (56 months vs. 38 months, P < 0.0001) and in patients with limb-onset disease (51 months vs. 37 months, P = 0.0002). Using Cox analyses values we found that the declines of ALS-FRS, FVC%, and MRC compound score were predictive of survival (P < 0.0001, P = 0.002, and P = 0.003, respectively). Future studies are needed to clarify whether nonspecific factors including muscle atrophy, dysphagia, and coexisting diseases influence prediction of survival in ALS patients. A more precise set of predictors may help to better stratify patient subgroups for future treatment trials. © 2002 Wiley Periodicals, Inc. Muscle Nerve 25:000,000, 2002 [source] The effects of exposure to environmental tobacco smoke on pulmonary function in children undergoing anesthesia for minor surgeryPEDIATRIC ANESTHESIA, Issue 5 2006JAMES M. O'ROURKE FFARCSI Summary Background:, The objectives of this study were to assess whether children exposed to environmental tobacco smoke (ETS) present for surgery with poorer pulmonary function, and experience a more pronounced deterioration in pulmonary function following anesthesia and surgery, than non-ETS-exposed children. Methods:, Fifty-four children aged 5,15 years with a history of ETS exposure from one or both parents and 54 children with no such ETS history were included in the study. All participants were presenting for ambulatory surgery and were judged to conform to American Society of Anesthesiology class I or II. Spirometry was performed preoperatively, postoperatively in the recovery ward when the child met criteria for discharge (Aldrete score 8), and before discharge from the day ward. Results:, The ETS-exposed group had a significantly lower mean preoperative peak expiratory flow rate (PEFR) (9.5 points lower percent predicted, 95% confidence interval ,18.1 to ,1.0, P = 0.03). Although not statistically significant, they also had lower percent predicted baseline mean values of the other spirometric variables that were measured (forced expiratory volume in 1 s ,4.5%, P = 0.07; forced vital capacity ,4.1%, P = 0.10; forced expiratory flow between 25% and 75%,3.6%, P = 0.44). Pulmonary function tests (PFTs) performed in recovery were between 8% and 14% worse than preoperative values, but the results were similar in the two groups of children. PFTs performed before hospital discharge demonstrated an near-complete recovery to baseline values. Again the pattern was similar in exposed and nonexposed children. Conclusions:, Environmental tobacco smoke exposure is associated with lower preoperative PEFR values, but does not impact on recovery from anesthesia for healthy children undergoing ambulatory anesthesia. [source] Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?PEDIATRIC PULMONOLOGY, Issue 10 2010Jo-Anne Johnson MBChB Abstract It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS). We collected annual lung function data between the ages of 8,15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2,15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period. Thirty-eight MI and 76 non-MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non-MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non-MI groups apart from WtZ score in the non-MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non-significant trend for subjects presenting with MI to do better in all four parameters. We conclude that babies presenting with MI have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF. Pediatr Pulmonol. 2010; 45:951,958. © 2010 Wiley-Liss, Inc. [source] Growth of lung function in children with sickle cell anemiaPEDIATRIC PULMONOLOGY, Issue 11 2008Joshua J. Field MD Abstract Lung disease is a common cause of morbidity among children with sickle cell disease (SCD). Although cross-sectional studies of children with SCD describe abnormal pulmonary function, the pattern of lung function growth in these children compared to children in the general population is not known. To provide preliminary evidence that growth of lung function is attenuated in children with SCD, we conducted a retrospective cohort study of children with hemoglobin SS (HbSS) ages 6,19 years who received at least two spirometry assessments for clinical care. The growth of lung function in these cases was compared to age, gender, and race-specific children without SCD or respiratory complaints from the Harvard Six Cities Study (H6CS). Seventy-nine children with HbSS contributed 363 spirometry measurements (mean per child,=,4.6, median,=,4.0, range,=,2,17) and 255 controls contributed 1,543 spirometry measurements (mean per child,=,6.1, median,=,6.0, range,=,2,13). Longitudinal forced expiratory volume in 1 sec (FEV1) was lower for boys and girls with HbSS compared to children in the general population, P,=,0.031 and P,=,0.002, respectively. When compared to the H6CS cohort, girls with HbSS showed lower longitudinal forced vital capacity (FVC) (P,<,0.001) and FEV1/FVC (0.038); there was no difference in FVC or FEV1/FVC between boys in the HbSS and H6CS cohort. We conclude that growth of lung function is reduced in children with HbSS compared to children in the general population. Gender may influence the risk of developing abnormal lung function and airway obstruction in children with HbSS. Pediatr Pulmonol. 2008; 43:1061,1066. © 2008 Wiley-Liss, Inc. [source] Shrinking Lung Syndrome in a 14-Year-Old Boy with Systemic Lupus ErythematosusPEDIATRIC PULMONOLOGY, Issue 2 2006Polly J. Ferguson MD Abstract Pulmonary complications occur frequently in people with systemic lupus erythematosus. We report on an adolescent with an acute onset of dyspnea and pleuritic chest pain with severe restrictive lung physiology on pulmonary function testing (forced vital capacity, 20% of predicted) who had no evidence of parenchymal lung or pleural disease. He was found to have restricted diaphragmatic movement as assessed by fluoroscopy, without evidence of generalized respiratory muscle weakness. His clinical presentation and results of diagnostic tests were typical for shrinking lung syndrome. Given the rarity of shrinking lung syndrome in the pediatric age range, many clinicians are not aware of it as a clinical entity. Shrinking lung syndrome should be included in the differential diagnosis of dyspnea in both children and adults with systemic lupus erythematosus. Pediatr Pulmonol. © 2005 Wiley-Liss, Inc. [source] Longitudinal pulmonary status of cystic fibrosis children with meconium ileusPEDIATRIC PULMONOLOGY, Issue 4 2004Zhanhai Li PhD Abstract Although meconium ileus (MI) is the earliest manifestation of cystic fibrosis (CF), and is associated with poorer growth, the longitudinal pulmonary progression of CF children with MI is not clear. To test the hypothesis that MI is associated with worse pulmonary outcomes, we prospectively compared from diagnosis to 12 years of age 32 CF children with MI to 50 CF children without MI who were diagnosed during early infancy through neonatal screening. Pulmonary outcome measures included respiratory symptoms, respiratory infections, pathogens, antibiotic usage, hospitalizations, quantitative chest radiology, spirometry, and lung volume determinations. Obstructive lung disease was defined as percent predicted spirometry values below the lower limits of normal. Longitudinal analyses revealed no significant differences in cough, wheezing, respiratory infections, prevalence of and median times to acquisition of Pseudomonas aeruginosa or Staphylococcus aureus, antibiotic usage, and chest radiograph scores between the two groups. However, MI children showed significantly worse forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), forced expiratory flow between 25,75% of FVC (FEF25,75), % predicted FEV1, % predicted FEF25,75, and total lung capacity (TLC). These differences were particularly apparent beginning at age 8,10 years. MI children also had higher rates of and shorter median times to obstructive lung disease. Subgroup analyses showed MI children treated surgically and those treated medically had similar pulmonary outcomes. In conclusion, MI children have worse lung function and more obstructive lung disease than those without MI. Such abnormalities are accompanied by reduced lung volume. MI is a distinct CF phenotype with more severe pulmonary dysfunction. Pediatr Pulmonol. 2004; 38:277,284. © 2004 Wiley-Liss, Inc. [source] Diabetes mellitus and cystic fibrosis: Comparison of clinical parameters in patients treated with insulin versus oral glucose-lowering agentsPEDIATRIC PULMONOLOGY, Issue 5 2001Joseph Rosenecker MD Abstract The prevalence of cystic fibrosis-related diabetes melltitus (CFRD) is increasing as patients with cystic fibrosis (CF) live longer. Because patients with CFRD are insulin-deficient, the standard medical treatment is exogenous insulin. Sulfonylureas enhance insulin secretion by acting on a specific islet beta cell receptor. No data are available about the outcome of sulfonylurea treatment vs. insulin treatment. In this retrospective study, data from 45 patients with CFRD were analyzed regarding their clinical outcome as it related to the treatment protocol. The duration of DM treatment was 7.6,±,4.6 years in the insulin-treated group and 3.5,±,2.0 years in the sulfonylurea group (n.s.). The age of CFRD diagnosis was significantly earlier in patients treated with insulin (n,=,34) than in the patients treated with sulfonylurea (n,=,11) (16.4,±,3.6 vs. 24.2,±,4.8 years, P,<,0.001). No statistical differences were found between the two groups in the time of CF diagnosis, the most recent forced expired volume in 1 sec, forced vital capacity, Shwachman score, hemoglobin A1C levels, or weight for height index at the end of the study. Our data suggest that a subgroup of CFRD patients can be managed for a number of years with sulfonylurea, and that the clinical outcome was not different in this group compared with the insulin-treated patients. Pediatr Pulmonol. 2001; 32:351,355. © 2001 Wiley-Liss, Inc. [source] Body composition and respiratory function in healthy non-obese childrenPEDIATRICS INTERNATIONAL, Issue 5 2007FRANCISCO J. GONZALEZ-BARCALA Abstract Background: The purpose of the present paper was to evaluate the role that body composition plays in lung function, among healthy children and adolescents. Methods: Cross-sectional study was undertaken using sex- and age-stratified sampling among healthy children and adolescents aged 6,18 years. Spirometry was performed on every child who fulfilled inclusion criteria. Fat mass (FM) and fat-free mass (FFM) were calculated from triceps skinfold thickness and arm circumference. Multiple logistic regression was used to obtain adjusted prevalence odds ratios (OR) and 95% confidence intervals (95%CI) between low pulmonary function (PF) of schoolchildren and body composition expressed in FM and FFM. Calculations of OR imply that the outcome is dichotomous (low PF/normal PF), therefore those children who had parameters of pulmonary function below the 25th percentile were classed as cases and the rest of the subjects were considered as controls. Results: A total of 2408 children were included; 1270 (53%) of them were male and 1138 (47%), female. Among boys the increase of FFM was associated with an increase of forced expiratory volume in 1 s (FEV1). Among girls the increase of FFM was associated with an increase of FEV1, forced vital capacity, and peak expiratory flow. The increase in FM was associated with a decrease of spirometric parameters in both genders, with a stronger effect among boys. Conclusions: The present study confirms the negative effect of body fat on the PF of children and adolescents, even though obese subjects were excluded. The effect is different between boys and girls. [source] Validity of the EK scale: a functional assessment of non-ambulatory individuals with Duchenne muscular dystrophy or spinal muscular atrophyPHYSIOTHERAPY RESEARCH INTERNATIONAL, Issue 3 2001Birgit Steffensen Abstract Background and Purpose The EK scale comprises ten categories (EK 1,10), each contributing to an overall picture of function in the non-ambulatory stage of Duchenne muscular dystrophy (DMD). The purpose of the present study was to investigate content and construct validity of the EK scale as a tool to discriminate between levels of functional ability in individuals with DMD or spinal muscular atrophy (SMA) who were non-ambulatory. Method Data from a sample of 56 subjects with DMD and 38 with SMA, who were non-ambulatory, were obtained from four separate studies. The relationship of functional ability by use of the EK scale and (1) muscle strength, (2) contractures, (3) forced vital capacity and (4) years of wheelchair dependency were assessed. All items of the EK scale were used except the one representing severe hypoventilation. Results Regression analyses showed that the EK sum was the most significant explanatory variable (p<0.05) of all variables measured to explain muscle strength in both DMD and SMA subjects. The individual categories of EK (1,10) all contributed as significant explanatory variables (p<0.05) to the other variables measured. Conclusions The categories and items of the EK scale were relevant and valid as means of discriminating between levels of functional performance in the population studied which was evidence of content and construct validity. Copyright © 2001 Whurr Publishers Ltd. [source] Pulmonary Function After Pectoralis Major Myocutaneous Flap HarvestTHE LARYNGOSCOPE, Issue 3 2002FACS, Yoav P. Talmi MD Abstract Objective The pectoralis major myocutaneous flap is widely used in the reconstruction of surgical defects in the head and neck region. Pulmonary atelectasis has been reported in patients undergoing these procedures, and many of these patients are heavy smokers and drinkers and have associated cardiopulmonary disorders. Flap harvest and donor site closure may lead to impairment of pulmonary function before and after the use of pectoralis major myocutaneous (PMC) in surgical reconstruction in patients with cancer of the head and neck. Methods Patients undergoing extirpation of head and neck tumors with PMC reconstruction were prospectively evaluated. Patient age, smoking history (pack-years), anesthesia duration, percentage predicted pre- and postoperative FEV1, percentage-predicted pre- and postoperative FVC (forced vital capacity), and preoperative SaO2 (oxygen saturation) were evaluated. Preoperative FEV1/FVC ratio was calculated. Chest x-rays were reviewed. Results Only 11 patients, 5 of whom smoked, could be evaluated postoperatively. Preoperative FEV1/FVC was more than 70 and FEV1 more than 75% predicted in all patients. A decrease in FVC was observed in 7 of the 11 patients, which ranged between 2% and 27% without any clinically obvious respiratory manifestations. A baseline SaO2 of more than 96% was noted in all patients. Four of 9 postoperative chest x-rays demonstrated atelectasis. Conclusions PMC harvest and donor site closure may lead to the recorded decrease in FVC measurements. These changes did not manifest clinically. Nevertheless, alternative methods of surgical defect closure should be considered in patients with severe preexisting pulmonary disorders. [source] Cardiac and respiratory failure in limb-girdle muscular dystrophy 2IANNALS OF NEUROLOGY, Issue 5 2004Maja Poppe MD Mutations in the gene encoding fukutin-related protein cause limb-girdle muscular dystrophy 2I. In this multicenter retrospective analysis of 38 patients, 55.3% had cardiac abnormalities, of which 24% had developed cardiac failure. Heterozygotes for the common C826A mutation developed cardiac involvement earlier than homozygotes. All patients initially improved while receiving standard therapy. Independent of cardiac status, forced vital capacity was below 75% in 44.4% of the patients. There was no absolute correlation between skeletal muscle weakness and cardiomyopathy or respiratory insufficiency. These complications are a primary part of this specific type of limb-girdle muscular dystrophy, with important implications for management. Ann Neurol 2004;56:738,741 [source] Elevated levels of leukotriene B4 and leukotriene E4 in bronchoalveolar lavage fluid from patients with scleroderma lung diseaseARTHRITIS & RHEUMATISM, Issue 6 2003Otylia Kowal-Bielecka Objective The leukotrienes are a family of arachidonic acid,derived lipid mediators with proinflammatory and profibrotic properties. The aim of this study was to analyze the role of leukotriene B4 (LTB4) and LTE4 in the pathogenesis of scleroderma lung disease (SLD). Methods Nineteen systemic sclerosis (SSc) patients with SLD, 11 SSc patients without SLD, and 10 healthy controls were studied. Bronchoalveolar lavage (BAL) fluid was obtained during routine bronchoscopy of the right middle lobe in all study subjects. Levels of LTB4 and LTE4 were measured using enzyme immunoassay kits. Results Levels of LTB4 and LTE4 were significantly higher in SSc patients with SLD (251 ± 170 pg/ml and 479 ± 301 pg/ml, respectively), than those in patients without SLD (114 ± 86 and 159 ± 149 pg/ml) and those in normal controls (86 ± 49 and 110 ± 67 pg/ml). In the total group of patients with SSc, levels of both leukotrienes correlated positively with the total number of cells in the BAL fluid and correlated negatively with the forced vital capacity. After intravenous pulse therapy with cyclophosphamide in 6 patients, there was a significant reduction in the concentration of LTB4 (from 380 ± 196 pg/ml to 155 ± 123 pg/ml) but no significant difference in the levels of LTE4 (from 697 ± 325 pg/ml to 418 ± 140 pg/ml). Conclusion Our findings show that LTB4 and LTE4 levels are elevated in SSc patients with SLD and correlate with parameters of inflammation in the lungs. These results indicate that leukotrienes may contribute to the pathogenesis of SLD and may represent a new therapeutic target. [source] Plethysmography and impulse oscillometry assessment of tiotropium and ipratropium bromide; a randomized, double-blind, placebo-controlled, cross-over study in healthy subjectsBRITISH JOURNAL OF CLINICAL PHARMACOLOGY, Issue 4 2006D. Singh Aims Spirometry, plethysmography and impulse oscillometry (IOS) measure different aspects of lung function. These methods have not been compared for their ability to assess long- and short-acting anticholinergic agents. We therefore performed a double-blind, placebo-controlled, four-way cross-over study in 30 healthy subjects. Methods Single doses of tiotropium bromide (Tio) 54 and 18 mcg, ipratropium bromide (IB) 40 mcg and placebo were administered. Specific conductance (sGaw), total lung capacity (TLC), inspiratory capacity (IC) and residual volume (RV) were measured using plethysmography, while IOS measured resistance (R5,25) and reactance (RF and X5). Pulmonary function was measured for 26 h post dose. Results Tio caused significant improvements in sGaw, forced expiratory voume in 1 s (FEV1), maximum mid-expiratory flow (MMEF) and R5,R25 at time points up to 26 h, with no clear differences between doses. IB improved the same parameters, but only up to 8 h. The weighted mean change (0,24 h) caused by Tio 54 mcg compared with placebo for FEV1 was 240 ml (95% confidence interval 180, 300), while for sGaw the ratio of geometric means (Tio compared with placebo) was 1.35 (1.28, 1.41). Neither drug caused consistent statistically significant changes in RF, forced vital capacity, TLC or IC over 26 h. RV was significantly improved from 8 to 24 h by Tio 54 mcg only. Conclusions In addition to spirometry, IOS resistance measurements and sGaw can distinguish between the effects of long- and shortacting anticholinergic effects in healthy subjects. [source] Rituximab for severe myasthenia gravis , experience from five patientsACTA NEUROLOGICA SCANDINAVICA, Issue 4 2010C. Lindberg Lindberg C, Bokarewa M. Rituximab for severe myasthenia gravis , experience from five patients. Acta Neurol Scand: 2010: 122: 225,228. © 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard. Background,,, Rituximab (RTX), a monoclonal antibody directed against CD20+ B cells, is used in the treatment of several autoimmune disorders including severe generalized myasthenia gravis (MG). Aims of the study,,, To describe the experience with RTX in five MG patients treated at our Neuromuscular Centre. Methods,,, Effect of RTX treatment was monitored by quantitative MG score (QMG score), forced vital capacity (FVC) and records of clinical parameters. Three patients had thymoma. Duration of MG prior to the first course of RTX was 3, 7, 26, 26 and 38 years. Results,,, We found favourable response to RTX treatment in all five patients. QMG score was markedly lower after RTX and in the three patients with respiratory muscle affection the FVC was increased. A good relief of bulbar, respiratory or extremity MG weakness was thus also found in the three patients who had long-standing severe MG. Repeated RTX treatment was needed in four patients. Conclusions,,, We conclude that RTX is effective in recent onset MG as well as in long-standing cases. As thymoma is prevalent in patients with severe MG, further studies are needed to evaluate the risk of thymoma recurrence following RTX treatment. [source] Airway limitation and exercise intolerance in well-regulated myasthenia gravis patientsACTA NEUROLOGICA SCANDINAVICA, Issue 2010A. Elsais Elsais A, Johansen B, Kerty E. Airway limitation and exercise intolerance in well-regulated myasthenia gravis patients. Acta Neurol Scand: 2010: 122 (Suppl. 190): 12,17. © 2010 John Wiley & Sons A/S. Objectives,,, Myasthenia gravis (MG) is an autoimmune disease of neuromuscular synapses, characterized by muscular weakness and reduced endurance. Remission can be obtained in many patients. However, some of these patients complain of fatigue. The aim of this study was to assess exercise capacity and lung function in well-regulated MG patients. Patients and methods,,, Ten otherwise healthy MG patients and 10 matched controls underwent dynamic spirometry, and a ramped symptom-limited bicycle exercise test. Spirometric variables included forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and maximum voluntary ventilation (MVV). Exercise variables included maximal oxygen uptake (VO2 max), anaerobic threshold (VO2 AT) maximum work load (W), maximum ventilation (VE max), and limiting symptom. Results,,, Myasthenia gravis patients had significantly lower FEV1/FVC ratio than controls. This was more marked in patients on acetylcholine esterase inhibitors. On the contrary, patients not using acetylcholine esterase inhibitors had a significantly lower exercise endurance time. Conclusion,,, Well-regulated MG patients, especially those using pyridostigmine, tend to have an airway obstruction. The modest airway limitation might be a contributing factor to their fatigue. Patients who are not using acetylcholinesterase inhibitor seem to have diminished exercise endurance in spite of their clinically complete remission. [source] Predictors of operative death after oesophagectomy for carcinomaBRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 8 2005H. Abunasra Background: Oesophagectomy for carcinoma provides a chance of cure but carries significant risk. This study defined risk factors for death after oesophageal resection for malignant disease. Methods: Between 1990 and 2003, 773 oesophagectomies for oesophageal cancer were performed. Continuous variables were categorized into quartiles for analysis. Predictors of operative mortality were identified by univariate and multiple logistic regression analysis. Results: The operative mortality rate was 4·8 per cent (37 of 773). In univariate analysis, advanced age, reduced forced expiratory volume in 1 s (FEV1), reduced forced vital capacity, presence of diabetes and tumour located in the upper third of the oesophagus were associated with a higher mortality rate. Multivariate analysis identified age (highest relative to lowest quartile, odds ratio (OR) 4·87 (95 per cent confidence interval (c.i.) 1·35 to 17·55); P = 0·009), tumour position (upper third relative to other locations, OR 4·23 (95 per cent c.i. 1·06 to 16·86); P = 0·041) and FEV1 (lowest relative to highest quartile, OR 4·72 (95 per cent c.i. 1·01 to 21·99); P = 0·018) as independent predictors of death. Conclusion: Advanced age, impaired preoperative respiratory function and a tumour high in the oesophagus are associated with a significantly increased risk of death after oesophagectomy for carcinoma. Copyright © 2005 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd. [source] IMPROVING THE CADMIUM-INDUCED CENTRIACINAR EMPHYSEMA MODEL IN RATS BY CONCOMITANT ANTI-OXIDANT TREATMENTCLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY, Issue 11 2008S Heili Frades SUMMARY 1The aim of the present study was to perform an evolutionary analysis of the morphometrical, biochemical and functional parameters of centriacinar emphysema induced by cadmium chloride (CdCl2) in rats and to determine the effects of concomitant N -acetylcysteine (NAC) administration. 2Male Wistar rats were instilled orotracheally with either CdCl2 (n = 24) or saline (n = 24). One group of rats, consisting of both CdCl2 - and saline-treated rats, was fed a normal diet (n = 24), whereas the other group received NAC (n = 24). 3Changes in inspiratory capacity (IC), lung compliance (CL), expiratory flow at 75% (F75), forced vital capacity (FVC) and hydroxyproline content were assessed 2, 8, 21 and 45 days after instillation. Polymorphonuclear cells were evaluated 2 and 8 days after instillation and the mean linear intercept (Lm) was determined at 21 and 45 days. 4Over time, CdCl2 instillation causes several changes that are bound up with centriacinar emphysema. The concomitant administration of NAC to CdCl2 -treated rats partially reversed Lm at 21 days compared with CdCl2 alone (115 ± 2 vs 127 ± 2, respectively; P < 0.05). However, 45 days after instillation, NAC improved lung function in CdCl2 -treated rats compared with that in the saline-treated control group (IC 14.64 vs 15.25, respectively (P = 0.054); FVC 16.94 vs 16.28, respectively (P = 0.052), F75 31.41 vs 32.48, respectively (P = 0.062)). In addition, 45 days after instillation, NAC reduced lung collagen content in both the saline-treated control (100 vs 81% alone and in the presence of NAC, respectively) and CdCL2-treated groups (213 vs 161% alone and in the presence of NAC, respectively). In addition, although the results were not significant, NAC tended to reduce Lm and enhance CL in NAC + CdCl2 -treated rats. 5In conclusion, NAC partially improved emphysematous changes and reduced collagen deposition, which diminished the CdCl2 -induced fibrotic component of centriacinar emphysema. [source] | |||||||||||||||||||||||||