Restrictive Lung Function Pattern Research Articles

Pulmonary function abnormalities in young adults with thalassemia major

Abstract Thalassemia seems to be the most common genetic disorder. The pathophysiologic mechanisms for lung impairment is not clear. Lung function tests in children showed small airway obstruction and restrictive lung changes. Forty-two patients, 22 females and 20 males, aged from 17 to 36 years old, with homozygous β -thalassemia were examined in a lung function laboratory. All patients received chelation therapy and none of them had history of respiratory disease. Lung function tests included spirometry, static lung volumes measured by a closed circuit helium rebreathing technique and diffusion capacity measured by the method of single breath diffusion of carbon monoxide. All patients, before these tests, had haematologic examinations (Hb, serum ferritin), electrocardiogram and echocardiogram. Only five out of 42 patients had completely normal pulmonary function. Four had only diffusion impairment, 18 restrictive and 15 combined (restrictive and obstructive) pattern of lung disease. Vital capacity (in 27 patients), total lung capacity (in 21 patients) and forced expiratory volume in the first second (in 30 patients) were abnormally reduced. The ratio of residual volume (RV) to total lung capacity (TLC) was increased in 19 patients. Patients with only diffusion impairment had statistically significant more increased serum ferritin value (4415 ng/ml) than other patterns of lung function. Patients with combined or restrictive pattern of lung function, had heart failure and osteoporosis more often than other patients. Heart failure and the aberrant growth process that limits the volume of peripheral airspace are perhaps the possible mechanisms for the restrictive lung disease, small airways obstruction and hyperinflation.

Pulmonary Functions in Congenital Scoliosis

Thirty-six patients with congenital scoliosis underwent full clinical and radiologic evaluation of their deformity and their full pulmonary functions. This study observed pulmonary functions in congenital scoliosis in detail, evaluated pulmonary functions in nonsurgically treated patients, and established whether, in surgically treated patients, there are any differences between those with multiple thoracic anomalies and those with lumbar or one of two thoracic anomalies. Eighty-six of our patients in both groups (surgically and nonsurgically treated) showed abnormal increases in residual volumes, indicating a restrictive pattern of lung function. The results of their pulmonary functions were analyzed using predicted values to eliminate age affect. Pulmonary functions were assessed using the Gould 5000IV Computerized Pulmonary Function System. Overall, mean total lung capacity was 89% of predicted value, and mean vital capacity and forced vital capacity were 74% of predicted value. The mean residual volume was significantly increased, being 154% of predicted value. Nonsurgically treated patients showed normal total lung capacity (mean 99.8% of predicted value); this mean value was 82% of predicted value in surgically treated patients. Vital capacity was found to be significantly reduced in surgically treated patients (68% of predicted value), especially in those patients who had multiple thoracic anomalies. We believe that children with congenital scoliosis due to multiple anomalies should be operated on at an early age before deformity is too severe.

Pulmonary function in systemic lupus erythematosus is related to distinct clinical, serologic, and nailfold capillary patterns

purpose: The purpose of this study was to investigate whether systemic lupus erythematosus (SLE) patients with interstitial lung disease represent a particular subset of patients characterized by the presence of clinical, serologic, and nailfold capillary patterns overlapping scleroderma. patients and methods: In 57 consecutive patients with SLE, a standardized detailed history was obtained and a physical examination performed, directed at signs and symptoms of connective tissue diseases, in particular scleroderma. Additionally, pulmonary function testing, chest radiography, radionuclide transit studies of the esophagus, nailfold capillary microscopy, and detailed serologic studies directed at the antigenic specificities of antinuclear antibodies were performed. Patients were divided into three groups based on the results of pulmonary function testing, i.e., normal lung function, restriction, or isolated impairment of diffusion. Clinical, serologic, and nailfold capillary microscopic findings were compared among these three groups. results: Twenty patients had normal lung function, 19 had restrictive lung function loss, and 9 had an isolated impairment of the diffusing capacity (T 1,CO). Patients with obstructive lung disease (n = 9) were excluded from analysis. Sclerodermatous changes of the hands were associated with a restrictive lung function pattern. Interstitial changes on chest radiograph were associated with isolated impairment of T 1,CO- Nailfold capillary abnormalities correlated with decreased T 1,CO and Dm, the component of T 1,CO representing the diffusing capacity of the alveolocapillary membrane. Antibodies to U1-RNA were associated with restrictive lung function and decreased T 1,CO. conclusion: We conclude that interstitial lung disease is present in a subset of SLE patients characterized by an increased prevalence of scleroderma traits and anti-(U1)RNA antibodies. Microvascular changes may contribute to the development of interstitial lung disease in SLE as well as in scleroderma.

Asbestos exposure, cigarette smoking, and airflow limitation in long-term Canadian chrysotile miners and millers.

To investigate further the relationships of asbestos exposure, cigarette smoking, and airflow limitation, we have obtained detailed pulmonary function tests (PFT) in 331 long-term Canadian chrysotile workers, 34 of whom were lifetime nonsmokers. Three disease categories were defined on the bases of standard diagnostic criteria, gallium-67 lung uptake, and the lung pressure-volume curve. Category A was composed of workers without changes suggestive of alveolitis or asbestosis. There were eight nonsmokers (ns), among whom we found a statistically significant 30% reduction in airflow conductance (Gus) at low lung volume, which is consistent with the concept of an asbestos airway lesion. The 85 smokers (sm) of category A had reduction of Gus at both high and low lung volumes. Category B was composed of workers without asbestosis but with evidence of asbestos alveolitis. In the six ns, Gus was significantly reduced to 50% normal at low lung volume. The 59 sm had reduction of Gus at both high and low lung volumes but less severely than sm in category A. Category C was composed of workers with asbestosis. The 20 ns had restrictive pattern of lung function, and Gus was decreased to 39% normal at 50% TLC. The 153 sm in C had airflow reduction comparable to sm in B. We concluded that asbestos exposure, which leads to asbestos airway disease, asbestos peribronchiolar alveolitis, and asbestosis, causes airflow limitation at low lung volume but does not reduce the expiratory flow rates on the flow-volume curve in lifetime nonsmokers. In the smoking asbestos workers with alveolitis or asbestosis, the major component of airflow limitation is a smoking effect. In these smoking workers, rigidity of the lung lessens airflow obstruction associated with smoking at the expense of increased work of breathing.

Airway function in lifetime-nonsmoking older asbestos workers

Previous studies of lung function in asbestos workers have documented airflow limitation in many of the workers, but the specific influence of asbestos exposure could not be clearly differentiated from the effects of the cigarette smoking habit. In this study, airway function was evaluated in lifetime-nonsmoking, long-term workers of the mines and mills of Québec. The 17 asbestos workers in this study had worked for an average of 28 years in the mines and mills of the local asbestos industry and did not have any other respiratory industrial dust exposure. They did not have a history of previous pulmonary disease and did not meet the usual diagnostic criteria for chronic bronchitis, emphysema, or asthma. Seven of the workers met the diagnostic criteria for asbestosis and 10 workers did not. The latter group of workers did not differ from a matched control group except in terms of a higher isoflow volume (p < 0.05). The workers with asbestosis, however, had a restrictive pattern of lung function, increased isoflow volume, and Increased upstream resistance at low lung volumes (p < 0.01). Lung biopsy in three of the patients with the disease demonstrated peribronchiolar alveolitls and fibrosis with obliteration and narrowing of the small airways. These data on lifetime-nonsmoking, long-term asbestos workers provide further evidence of small airway obstruction associated with asbestos exposure and independent of the smoking habit. This airflow limitation was observed predominantly in workers with a restrictive pattern of lung function associated with peribronchiolar alveolltis. The lifetime-nonsmoking asbestos workers without restrictive patterns of lung function had minimal dysfunction of the peripheral airways.

Restrictive pattern of lung function following Psittacosis treated with corticosteroids

Psittacosis is a well recognized cause of pneumonia, although in most cases infection remains mild or subclinical. Some patients are more severely ill with involvement of the respiratory, cardiovascular and central nervous systems. The chest radiograph often shows areas of consolidation greater than the physical signs would suggest. Recovery may be prolonged. A case of severe pneumonia due to psittacosis is reported in which a severe restrictive pattern of lung function complicated recovery. This has been described following mycoplasma pneumonia with evolution into diffuse interstitial fibrosis (Kaufman et al. 1980) but not as far as we can discover following psittacosis. Treatment with corticosteroids was associated with complete resolution of the lung function and chest radiograph abnormalities after nine months.

Restrictive pattern of lung function following psittacosis treated with corticosteroids

Psittacosis is a well recognized cause of pneumonia, although in most cases infection remains mild or subclinical. Some patients are more severely ill with involvement of the respiratory, cardiovascular and central nervous systems. The chest radiograph often shows areas of consolidation greater than the physical signs would suggest. Recovery may be prolonged. A case of severe pneumonia due to psittacosis is reported in which a severe restrictive pattern of lung function complicated recovery. This has been described following mycoplasma pneumonia with evolution into diffuse interstitial fibrosis (Kaufman et al. 1980) but not as far as we can discover following psittacosis. Treatment with corticosteroids was associated with complete resolution of the lung function and chest radiograph abnormalities after nine months.

Royal Naval dockyards asbestosis research project: nine-year follow-up study of men exposed to asbestos in Devonport Dockyard.

Progression of asbestos-related disease was assessed in a group of 253 dockyard workers examined in 1966 and 1975. Despite the almost complete protection from exposure to asbestos since 1966, radiographic parenchymal abnormalities increased and occurred more frequently during the next 9 years in those men who had been more heavily exposed to asbestos. Lung function values were lower in those most heavily exposed and were declining at a faster rate than in those with less dust exposure. The most sensitive lung function index was the transfer factor.Those men with persistent crackles in 1966 exhibited a restrictive pattern of lung function, whereas an obstructive pattern was seen in men with wheezes in 1966. The men with irregular small opacities of category 1/1 or more or with diffuse pleural change in 1966 and who survived to 1975 had worse lung functions than any other groups.Progression of disease was greater for smokers than non-smokers, with those who gave up smoking between 1966 and 1975 suffering the greatest changes. This latter group showed most increase in small opacities and included almost all new cases of diffuse pleural changes. They also showed the greatest declines in forced expiratory volume and forced vital capacity.