Pulmonary lymphangioleiomyomatosis in Korea.
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BACKGROUND: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease occurring in women of reproductive age and leading to progressive respiratory failure in spite of treatment. In Korea the first case was reported in 1984 and by 1997 a total of 23 cases had been reported. The clinical findings of these Korean cases are reviewed. METHODS: The details of 10 cases of LAM on file at Seoul National University Hospital were reviewed together with those of 13 cases previously reported from other Korean institutes. Two, including the only one to be reported in a man, were excluded after reviewing the clinical, radiological, and pathological findings, leaving a total of 21 cases in the present study. RESULTS: All 21 patients were women and in all cases the disease was proven pathologically. The mean (SD) age at onset of symptoms was 32 (8.6) years. The most common symptoms were dyspnoea and pneumothorax which were seen in 19 (90%) and 13 (76%) patients, respectively. Pulmonary function tests showed decreased transfer factor (TLCO) (100%) and airflow limitation (67%). All the cases had characteristic cysts on high resolution computed tomographic (HRCT) scanning. The overall severity score based on HRCT scans correlated with the percentage predicted TLCO/VA (p = 0.03) and FEV1/FVC (p = 0.02). The patients were all treated with medroxyprogesterone and/or tamoxifen. Follow up was possible in 10 cases. Two of these patients appeared to stabilise with no appreciable change clinically or in lung function on medroxyprogesterone and/or tamoxifen, but the remaining patients all deteriorated with two dying of respiratory insufficiency and one of infection following lung transplantation. CONCLUSIONS: As in other countries, in Korea LAM occurs exclusively in women and progresses despite hormonal treatment. (+info)
Pulmonary sarcoidosis: comparison of patients at a university and a municipal hospital.
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Charts and radiographs of sarcoidosis patients seen at a private university hospital and at a municipal hospital were reviewed to determine whether there was a difference in the severity of disease retrospectively. A standardized abstract form was used to identify and abstract information on new and continuing sarcoidosis patients seen at either Georgetown University Medical Center (GUMC) or District of Columbia General Hospital (DCGH) during a 2-year period. Because there were too few white sarcoidosis patients for comparison, analysis was done for African-American patients only. African-American patients at GUMC were slightly older, with a higher percentage of women. For GUMC patients, 76% had private insurance and 21% had public insurance, and for DCGH patients, one-half had public insurance and 29% had no insurance. Significantly fewer GUMC patients (7% versus 36%) reported moderate to severe dyspnea. Chest radiographs showed a larger percentage of patients with stage 1 disease at GUMC and more patients with stage 4 disease at DCGH. Spirometry showed more impairment of forced expired volume in one second (FEV1) in GUMC patients, but diffusing capacity of the lung for carbon monoxide (DLCO) values were significantly lower among DCGH patients. Less than 8% of GUMC patients showed disease progression compared with almost one-third of DCGH patients. These results demonstrate that substantially less severe pulmonary sarcoidosis was seen in African-American patients treated at a private, nonprofit university hospital compared with a municipal hospital. Factors that determine the use of municipal hospitals, such as limited financial access to care and sources of patients, may have played a major role in the differences seen. (+info)
Diesel exhaust exposure among adolescents in Harlem: a community-driven study.
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OBJECTIVES: This study sought individual-level data on diesel exhaust exposure and lung function among adolescents in Harlem as part of a community-driven research agenda. METHODS: High school students administered in-person surveys to seventh grade students to ascertain information on demographics, asthma history, and self-reported and maternal smoking. Urine samples were assayed for 1-hydroxypyrene (1-HP), a marker of diesel exhaust exposure, and cotinine, a marker of tobacco smoke exposure. Computer-assisted spirometry was used to measure lung function. RESULTS: Three quarters (76%) of the participating students had detectable levels of 1-HP. Three students (13%) had an FEF25-75 of less than or equal to 80% of their predicted measurements, and 4 students (17%) had results between 80% and 90% of the predicted value, all of which are suggestive of possible lung impairment. CONCLUSIONS: These data suggest that most adolescents in Harlem are exposed to detectable levels of diesel exhaust, a known exacerbator and possible cause of chronic lung disorders such as asthma. Community-driven research initiatives are important for empowering communities to make needed changes to improve their environments and health. (+info)
Pulmonary epithelial sodium-channel dysfunction and excess airway liquid in pseudohypoaldosteronism.
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BACKGROUND: Active sodium absorption is the dominant mechanism of ion transport in airway epithelium, but its role in pulmonary physiology and airway host defense is unknown. To address this question, we studied the function of airway epithelial cells and determined the frequency of pulmonary symptoms in patients with systemic pseudohypoaldosteronism, a salt-losing disorder caused by loss-of-function mutations in the genes for the epithelial sodium channel. METHODS: In nine patients 1.5 to 22 years of age who had systemic pseudohypoaldosteronism, we tested for mutations in the genes for the epithelial sodium channel, estimated the rate of sodium transport in the airway, determined the volume and ion composition of airway surface liquid, reviewed clinical features, collected laboratory data pertinent to pulmonary function, and, in three adults, measured mucociliary clearance. RESULTS: The patients with systemic pseudohypoaldosteronism had loss-of-function mutations in the genes for the epithelial sodium-channel subunits, no sodium absorption from airway surfaces, and a volume of airway surface liquid that was more than twice the normal value. The mean (+/-SE) mucociliary transport rate was higher in the 3 adult patients than in 12 normal subjects (2.0+/-0.7 vs. 0.5+/-0.3 percent per minute, P=0.009). Young patients (those five years of age or less) all had recurrent episodes of chest congestion, coughing, and wheezing, but no airway infections with Staphylococcus aureus or Pseudomonas aeruginosa. Older patients (those more than five years of age) had less frequent respiratory symptoms. CONCLUSIONS: Patients with systemic pseudohypoaldosteronism fail to absorb liquid from airway surfaces; the result is an increased volume of liquid in the airways. These results demonstrate that sodium transport has a role in regulating the volume of liquid on airway surfaces. (+info)
Monitoring respiratory function and sleep in the obese Vietnamese pot-bellied pig.
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Development of drug treatments for obstructive sleep-disordered breathing has been impeded by the lack of animal models. The obese pig may be a suitable animal model, as it has been reported to experience sleep-disordered breathing resembling human obstructive sleep apnea. The purpose of this paper is to describe in detail techniques for chronic instrumentation of the obese Vietnamese pot-bellied pig and to study respiratory function during sleep. Under general anesthesia, four obese pigs were instrumented for long-term recording of intrapleural and tracheal pressures, genioglossal EMG, and bioelectric signals related to sleep. A custom-fitted face mask was used to record respiratory variables including airflow, snoring, and expired CO(2). Most chronic instrumentation provided robust signals for up to 6 wk after installation. All pigs displayed sleep-disordered breathing characterized by increased resistance to airflow, snoring, inspiratory flow limitation, and possible sleep disruption. Apneas and hypopneas were not a feature of breathing during sleep in these animals. Nonetheless, this animal preparation may be useful for exploring possible drug treatments for obstructive sleep-disordered breathing. (+info)
In normal subjects bracing impairs the function of the inspiratory muscles.
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Normal subjects can increase their capacity to sustain hyperpnoea by bracing their arms on fixed objects, a procedure which is also known to reduce dyspnoea in patients with chronic obstructive pulmonary disease (COPD). In the present study, it was tested whether bracing per se could improve the function of the diaphragm. The effect of bracing on diaphragm function was studied in six normal subjects by recording changes in oesophageal (delta Poes) and transdiaphragmatic (delta Pdi) pressure during inspiratory capacity (IC) manoeuvres in the seated and upright postures, and in the seated posture, also during bilateral phrenic nerve stimulation (BPNS) at functional residual capacity (FRC). The pattern of ribcage motion and deformation associated with bracing and with diaphragm contraction was also evaluated using inductance plethysmography and magnetometers. Bracing increased FRC by >300 mL and reduced IC by approximately 200 mL, in both postures. Delta Pdi during BPNS decreased on average by 15% indicating an impaired diaphragmatic function. The ribcage was deformed with bracing and was more distortable during BPNS. In conclusion, in normal subjects, bracing impairs the function of the inspiratory muscles and reduces ribcage stability. These negative effects cannot explain the improved capacity to sustain hyperpnoea when the arms are braced. (+info)
Prognostic value of lung function and pulmonary haemodynamics in OSA patients treated with CPAP.
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The aim of the present study was to determine survival rates of obstructive sleep apnoea patients treated with continuous positive airway pressure (CPAP) and to investigate the prognostic value of pretreatment lung function and pulmonary haemodynamics. Two hundred and ninety-six patients, exhibiting > or = 20 apnoeas plus hypopnoeas per hour of sleep, were included. Patients were treated with nasal CPAP and regularly followed up. The cumulative survival rates were 0.96 (95% confidence interval (CI): 0.94-0.99) at 3 yrs and 0.93 (95% CI: 0.91-0.97) at 5 yrs. Most patients died from cardiovascular disease. Apart from age, covariates associated with a lower survival were the presence of a heavy smoking history, a low vital capacity, a low forced expiratory volume in one second (FEV1) and a high mean pulmonary artery pressure. Only three covariates were included by forward stepwise selection in the multivariate analysis, smoking habit (>30 pack-yrs), age and FEV1. The observed survival rates of the group as a whole were similar to those of the general population matched in terms of age, sex and smoking habit, except for patients between 50 and 60 yrs old who had reduced survival. This difference disappeared when patients of the present study with an associated chronic obstructive pulmonary disease were excluded from the comparison. In conclusion, survival of obstructive sleep apnoea patients treated with nasal continuous positive airway pressure is near to that of the general population. The prognosis is worse in subgroups of patients with a history of heavy smoking and with an associated chronic obstructive pulmonary disease. (+info)
Socioeconomic status, lung function and admission to hospital for COPD: results from the Copenhagen City Heart Study.
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This study analysed the effect of education and income on development of chronic obstructive pulmonary disease (COPD) assessing lung function and hospital admission. The study population consisted of 14,223 subjects, aged 20-90 yrs, randomly sampled from the population of Copenhagen in 1976. Association between socioeconomic factors and forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) at study entry was analysed by linear regression. The relation between socioeconomic factors and risk of admission to hospital for COPD from study entry until 1993 was assessed by register linkage. Education and income were independently associated with FEV1 and FVC. The age- and height-adjusted difference in FEV1 (mean+/-SEM) between the highest and lowest level of education and income was 259+/-31 mL in females and 400+/-39 mL in males. After additional adjustment for quantity and duration of smoking and inhalation, the difference was 220+/-31 mL and 363+/-39 mL in females and males, respectively. Results for FVC were of the same magnitude. Using a socioeconomic index which combined information on education and household income the association with lung function did not differ by age. A total of 219 females and 265 males were admitted to hospital for COPD during follow-up. Education and income were significantly associated with admission to hospital. After detailed adjustment for smoking the relative risks (95% confidence intervals) for medium and high versus low socioeconomic index in females were 0.74 (0.55-1.02) and 0.27 (0.10-0.73), respectively. Corresponding relative risks in males were 0.47 (0.36-0.63) and 0.35 (0.17-0.70). The results indicate that socioeconomic factors operating from early in life affect the adult risk of developing chronic obstructive pulmonary disease independently of smoking in both females and males. (+info)