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Pulmonary function and resting breathing pattern in myotonic dystrophy.

In 17 patients with myotonic dystrophy, spirometric, flow-volume, and maximal mouth occlusion variables were obtained and compared with 8 normal subjects. Ventilatory CO2 response was measured by the estimation of the steady-state effect of a sufficiently large serial dead space. Variability of resting breathing pattern was expressed by the variation coefficients of respiratory cycle time and tidal volume. The group means of the total lung capacity (TLC), vital capacity (VC), forced expiratory volume in 1 sec (FEV)1 and forced inspiratory volume in 1 sec (FIV)1 showed a restrictive pattern. Only maximal static mouth pressure (Pi,max), measured at residual volume (RV) level, showed a significant positive correlation with both VC (p = 0.03) and FIV1 (p = 0.02), suggesting inspiratory muscle weakness as a determinant of the restriction. Although the differences were just not significant, both variation coefficients of the respiratory cycle time and tidal volume were larger in the group with a CO2 sensitivity below the lower limit of normal compared to those with a normal ventilatory response to CO2. In 3 patients, fluctuations in FRC were also present. We hypothesize that, in addition to the already documented FRC fluctuations by uncoordinated spontaneous intercostal muscle action, a defect of integration of afferent neural input and chemical drive in the medullary region may also be present in these patients.

Adult↗

Timing of recovery of lung function after severe hypoxemic respiratory failure in children.

OBJECTIVE: To describe the timing of recovery of lung function after severe acute hypoxemic respiratory failure (AHRF) in children. DESIGN: A serial observational follow-up study of clinical and lung function measurements up to 53 months after acute illness. SETTING: University pediatric intensive care unit in a national children's hospital. PATIENTS: Five critically ill children aged 5-14 years. INTERVENTIONS: None RESULTS: Clinical recovery: each patient required a 3-5 month convalescence before being able to attend full-time school because of lethargy and dyspnea. All patients developed wheeze 3-12 months after illness and four received long-term bronchodilator therapy. Lung function recovery: for both the forced vital capacity (FVC) and forced vital capacity in the first second (FEV1) four patients had abnormally low values, regaining only 60-70% of predicted values for their height and sex, and all of this improvement had occurred by 6-12 months after illness. Beyond this interval, patients remained on their same FVC and FEV1 centile. FEV1/FVC ratios were consistently within the normal range, indicating a predominantly restrictive defect. Changes in peak expiratory flow exhibited a time course of improvement similar to the other lung function tests. CONCLUSION: In children, pulmonary recovery after severe AHRF may occur for 6-12 months. A 1-year follow-up could offer a rational single point for assessment of outcome and long-term counselling of child and parents.

Adolescent↗

Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis.

Forty-nine patients with progressive systemic sclerosis who had undergone extensive studies including pulmonary artery catheterization as part of an ongoing prospective study of the natural course of progressive systemic sclerosis were evaluated. The overall prevalence of pulmonary arterial hypertension in this population of patients with progressive systemic sclerosis was 33 percent, and among 10 subjects with the CREST syndrome the prevalence of pulmonary hypertension was 50 percent. The relation between pulmonary arterial hypertension documented at catheterization and abnormal results of noninvasive studies suggesting pulmonary hypertension, including physical examination, chest x-ray, electrocardiography, echocardiography, single-breath diffusing capacity, and vital capacity, was studied. Diffusing capacity was significantly lower in those patients with definite pulmonary hypertension (mean pulmonary artery pressure of 22 mg Hg or more) compared with those with a normal mean pulmonary artery pressure, and a diffusing capacity below 43 percent of predicted showed the greatest sensitivity (67 percent) of any single diagnostic test in detecting definite pulmonary hypertension. Chest x-ray suggesting pulmonary hypertension was the least sensitive of the tests evaluated, but showed the greatest specificity (100 percent) in identifying patients with pulmonary hypertension. A classification matrix based on discriminant function analysis utilizing the combination of diffusing capacity below 43 percent of predicted and chest x-ray and electrocardiographic findings correctly identified 75 percent of patients with definite pulmonary hypertension and 97 percent of patients with a normal pulmonary artery pressure, but failed to identify correctly patients with mild pulmonary hypertension (mean pulmonary artery pressure of 20 mm Hg). These findings indicate that specific noninvasive studies are helpful in assessing the likelihood of normal or definitely elevated pulmonary artery pressures in patients with progressive systemic sclerosis, but patients with mild pulmonary hypertension are not likely to be identified by these noninvasive studies.

Adult↗

Changing relationships between stature and lung volumes during puberty.

In a follow-up study the relationship between growth in body dimensions and in lung volumes during the adolescent growth spurt was studied in 149 girls and 477 boys, aged 11.5-14.0 years at the start of the study. Total lung capacity (TLC), residual volume, vital capacity (VC) and series dead space were measured twice a year for 6 years. When analyzed cross-sectionally lung volumes can be described by a power function of stature, both for each set of half-yearly measurements and when all data are pooled. The powers were close to 3, increasing at each time of measurement in boys and decreasing in girls. These trends reflect that in each subject the growth spurt does not start at the same age, and that individual growth occurs non-linearly along a steeper line than found in the cross-section. Using simple allometric relationships between stature and lung volumes, volume predictions are too high in the youngest age group, and too low in the oldest adolescents. Including age in the regression equation removes this trend in the cross-sectional data, and for VC and TLC also in the longitudinal data.

Adolescent↗

Indomethacin and cromolyn sodium alter ozone-induced changes in lung function and plasma eicosanoid concentrations in guinea pigs.

Male Hartley guinea pigs were given either indomethacin (IN), cromolyn sodium (CS), or no drug (ND) and then exposed either to filtered air or to 1 ppm ozone (O3) for 1 hr. At 2 or 24 hr postexposure, ventilation, respiratory mechanics, lung volumes, carbon monoxide-diffusing capacity (DLCO), and alveolar volume (VA) were measured, and in separate groups of animals, plasma eicosanoids (EC) were measured. Both drugs blocked the increase in flow resistance noted at 2 hr after O3 and prevented O3-induced increases in the wet lung weight to body weight ratio seen at 2 and 24 hr in the ND group. In the ND animals O3 also decreased total lung capacity (TLC), vital capacity (VC), functional residual capacity (FRC), and residual volume (RV). IN as well as CS blocked reductions in FRC and RV at both 2 and 24 hr after O3. TLC was reduced by both drug treatments in air- and O3-exposed animals. CS treatment also decreased VC in all groups. IN blocked reductions in VA after O3 but did not prevent decreases in DLCO. CS blocked reductions in both VA and DLCO after O3, but the drug decreased DLCO in air-exposed animals. The prostaglandins PGF2 alpha and 6-keto PGF1 alpha were largely unaffected by O3 exposure or drug treatment. Prostaglandin E1 (PGE1) was not affected by O3, but both drugs significantly increased PGE1 in all exposure groups. Effects on plasma thromboxane B2 (TxB2) were variable although in most groups TxB2 was lower than in the O3-exposed ND groups. Although our findings suggest that both drugs block some effects of O3 exposure on the lungs and on plasma EC concentrations, the degree to which EC contribute to O3-induced pulmonary effects is not clearly apparent.

Animals↗

Enhanced pulmonary toxicity in smokers with germ-cell cancer treated with cis-platinum, vinblastine and bleomycin: a long-term follow-up.

The long-term effects of bleomycin on pulmonary function were studied. Thirty-four patients with germ-cell cancer were followed for an average of 64 months (range 43-98 months). All had obtained complete remission during treatment and none had relapsed at the follow-up examination. Pulmonary function was tested by measurements of total lung capacity (TLC), vital capacity (VC), forced expiratory volume in one second (FEV1) and single breath diffusion capacity for carbon monoxide (TLCO). TLC and VC were significantly reduced by the treatment (P less than 0.05), but normalized during the follow-up. TLCO was initially reduced to a predicted median of 83%. In the smokers the initial TLCO was at a predicted median of 79%, while in non-smokers a median of 88% was predicted. During the first 2 months of treatment, TLCO increased both in smokers and non-smokers to predicted medians of 90% and 91%. Subsequently, however, a significant decrease to 72% was noted in the smokers, while the non-smokers had only an insignificant decrease to 84%. The decrease in TLCO was irreversible but not progressive. We conclude that bleomycin treatment is associated with a long-term sustained reduction in TLCO. The changes were most pronounced in the smokers.

Adult↗

A comparison of the effects of paraquat and diquat on lung compliance, lung volumes and single breath diffusing capacity in the rat.

Paraquat intoxication in its initial stage is characterized histologically in the lungs by atelectasis, hyaline membrane formation, alveolar edema and vascular hemorrhage often into the interstitium or air spaces. Information on the functional modification of paraquat-damaged lungs has been lacking. We evaluated lung volumes, single breath diffusing capacity of the lungs for carbon monoxide (DLCO) and static lung compliance (Cst(L)) in rats treated with paraquat or diquat. Measurements were made 12, 24, 48, and 72 h after treatment. Paraquat by intratracheal (i.t.) instillation 0.5 mg/kg or by intraperitoneal injection (i.p.) 27 mg/kg significantly decreased (P less than 0.01) the body weight, total lung capacity (TLC), functional residual capacity (FRC), vital capacity (VC), residual volume (RV), DLCO, apparent alveolar volume (VA) and Cst(L). At a lower dose level (13.5 mg/kg), the effects of paraquat peaked at about 24 h following treatment, causing a significantly decreased (P less than 0.01) VC and TLC. Diquat i.t. or i.p. had little effect on the lungs. However, diquat i.p. decreased body weight (P less than 0.01) and caused a slight increase (P less than 0.05) in VC. The data obtained are consistent with the known pathological changes seen in paraquat-damaged lungs in that, by both routes, paraquat caused severe lung damage associated with decreased elasticity of the lungs and thorax, destruction of gas exchanging alveolar surfaces, and edema. These changes were detected reliably by lung function measurements.

Animals↗

Long-term reduction of hyperinflation in stable COPD by non-invasive nocturnal home ventilation.

OBJECTIVE: The role of non-invasive positive pressure ventilation (NPPV) in stable COPD with chronic ventilatory failure remains controversial. The impact of long-term home nocturnal NPPV treatment on deflation has not yet been evaluated in detail. METHODS: Retrospective explorative study of 46 patients with stable COPD undergoing NPPV treatment. Effects of NPPV on body plethysmographic parameters, blood gas tensions and inspiratory muscle function after 6.2 (+/-1.7) and 12.7 (+/-2.1) months of treatment. Further, evaluation of 1-year survival, compliance and ventilation parameters. RESULTS: One-year survival was 89.1%. The effectiveness of ventilation was proven by a significant reduction in nocturnal and daytime PaCO2. We observed a decrease in the ratio of residual volume (RV) to total lung capacity (TLC) on the average of 5.2+/-9.8% (or 15.2+/-29.7% pred.; P<0.01) at six and 3.9+/-9.0% (or 12.9+/-18.6% pred.; P<0.001) at 12 months. As a consequence, we found significant improvements in inspiratory capacity (IC), vital capacity (VC) and forced expiratory volume in one second (FEV1). For patients with the most severe hyperinflation (RV/TLC>75%), we found a significant positive correlation between inspiratory positive airway pressure (IPAP) and reductions in PaCO2 (r=0.56; P<0.05) and RV/TLC (r=0.50; P<0.05). CONCLUSIONS: In severe hypercapnic stable COPD long-term nocturnal NPPV can reduce hyperinflation with sustained improved daytime blood gas parameters.

Aged↗

Late cardiopulmonary toxicity after treatment for Hodgkin's disease.

Cardiac and pulmonary functions were evaluated in 75 patients aged 50 years or under, treated for Hodgkin's disease by mantle radiotherapy at least 3 years earlier; all received the same mantle field radiotherapy: radiotherapy alone, MOPP chemotherapy plus radiotherapy, MOPP and ABVD chemotherapy plus radiotherapy. No patient had any symptom of heart disease. Only borderline abnormalities of ECG or echocardiogram were observed in 12 patients. One of them showed a moderate aortic stenosis which was known before the treatment; apical or septum hypokinesia were present in four patients and one patient had a slightly right ventricular dilatation. Twelve (16%) chest radiographs showed moderate or severe abnormalities, but there was no significant correlation between the results of pulmonary function tests and Xenon ventilation/perfusion scintigraphy, the clinical examination and the intensity of the radiological sequelae. Twenty-nine (64%) Xenon scintigraphies showed a reduction of lung perfusion in the irradiated areas without any symptom. The resting mean pulmonary function test was significantly lower for the patients than for the control group with regard to Total Capacity and Vital Capacity. The exercise tolerance, as indicated by analysis of blood gases, was below the one expected for only two patients who were dyspneic during the low level of exercise. We did not find any significant difference between the three treatment groups. We conclude that the treatment with mantle field under good technical conditions (high energy photons, moderate doses...) can result in minimal cardiopulmonary dysfunction.

Adolescent↗

Long-term follow-up study of pulmonary findings in patients with primary Sjögren's syndrome.

BACKGROUND: In a previous study pulmonary hyperinflation was observed frequently in patients with primary Sjogren's syndrome (pSS) and elevated serum beta-2 microglobulin (beta2m) concentrations were associated with hyperinflation. OBJECTIVE: To evaluate the significance of baseline serum beta2m concentration and to identify other possible risk factors for pulmonary involvement in long-term follow-up of patients with pSS. METHODS: Nineteen pSS patients whose pulmonary function tests (PFTs) had been previously studied were reexamined after a median follow-up of 10 years. Pulmonary symptoms were recorded, chest radiograph, and high-resolution computed tomography (HRCT) were evaluated and methacholine provocation and PFTs including flow-volume spirometry, body plethysmography, and diffusing capacity performed. RESULTS: Baseline serum beta2m concentrations correlated inversely with follow-up total lung capacity (TLC), vital capacity (VC), and diffusing capacity (DL), and positively with residual volume (RV), all expressed as percentage of predicted values. Diminished airways resistance (Raw) and, correspondingly, elevated specific conductance (SGaw) were frequent findings in pSS patients at follow-up, indirectly implying stiffness of the lungs and a restrictive decrease in lung volumes. Baseline serum protein concentration was higher and IgG concentration tended to be higher in pSS patients who at follow-up had elevated SGaw compared with others. Interstitial changes in HRCT were found more frequently in patients with elevated SGaw than in those without. CONCLUSION: Our results suggest that subtle restrictive changes in pulmonary function are more prone to develop in the long term in pSS patients with elevated serum beta2m concentration and other signs of immunological activity at baseline.

Follow-Up Studies↗

Pulmonary effects of ultrafine coal fly ash inhaled by guinea pigs.

Guinea pigs were exposed to ultrafine coal fly ash produced in a laboratory furnace. The average mass median aerodynamic diameter and the average mass concentration of Illinois no. 6 fly ash produced in all exposure conditions were 0.21 microns and 5.8 mg/m3, respectively. In guinea pigs exposed to Illinois no. 6 fly ash, total lung capacity (TLC), vital capacity (VC), and diffusing capacity for carbon monoxide (DLco) were significantly reduced below the control values immediately, 2 h, and 8 h postexposure. The diffusing capacity was still 10% below the control 96 h after exposure. The total sulfate in the Illinois no. 6 fly ash as determined using ion chromatography is 1105 +/- 120 micrograms/m3. Animals exposed to the Montana lignite fly ash at comparable concentration and particle size did not show alteration in diffusing capacity. The data suggest that part of the sulfate present in the fly ash of Illinois no. 6 could be in the form of sulfuric acid and is responsible for the adverse effects observed in the exposed animals. The sulfuric acid in the fly ash of Montana lignite is neutralized by its high alkali content and produces no change in lung functions.

Administration, Inhalation↗

Improvement in pulmonary function in patients having combined anterior and posterior spine fusion for myelomeningocele scoliosis.

Thirteen patients with myelomeningocele scoliosis whose surgery necessitated anterior exposure of the thoracic spine were tested for changes in pulmonary function following spinal stabilization. The majority of patients exhibited a T12 level paralysis and a mean scoliosis of 72 degrees. Average correction of scoliosis for the series was 63%, and two cases with one level pseudarthrosis had no loss of correction. Ten of 13 patients were tested for pulmonary function one month prior to surgery and an average of ten months postoperatively. Eight of the ten patients demonstrated increase in peak flow postoperatively. Five demonstrated increase in maximum voluntary ventilation, and six cases showed increased functional residual capacity. Forced vital capacity increased in two, decreased in five, and showed no change in three patients. Improvement in peak flow, maximum voluntary ventilation, and functional residual capacity was noted in all five cases with scoliotic deformity in excess of 60 degrees (mean 93 degrees). It is postulated that, following spinal stabilization, these patients are able to improve their thoracic mechanics, enhancing greater expiratory flow with greater endurance.

Adolescent↗

Scoliosis and pulmonary function.

The effect on pulmonary function of all three dimensions of the scoliotic deformity was analyzed with computer tomography in 33 patients with thoracic idiopathic scoliosis. Both static (total lung capacity [TLC], vital capacity [VC] and functional residual capacity [FRC] ) and dynamic volumes (forced expiratory volumes in one second [FEV-1]) were negatively correlated to the lateral deformity and, to a lesser degree, to rotation. Of the computer tomography variables reflecting kyphosis, the sagittal diameter was correlated positively to all lung volumes though negatively to the quotient FRC/TLC while the KLi was correlated positively to VC and FEV1. The conclusion is drawn that the Cobb angle gives the most accurate estimation of lung volume compromise and that operative treatment should aim of reducing the lateral dislocation of the vertebrae and at maintaining normal sagittal thoracic curve.

Adolescent↗

Chip pile workers and mould exposure. A preliminary clinical and hygienic survey.

Chip pile workers are exposed to high concentrations of airborne mould spores. Few sporadic cases of allergic alveolitis have been reported in Finland. Eight non-smoking workers, six on chip piles and two on bark piles, were examined to study the immunological and clinical response to continuous mould spore exposure. The spore exposure ranged between 10(4) and 10(5) spores/m3. Three of the six chip pile workers had precipitating serum antibodies against Aspergillus fumigatus. The three antibody-positive workers had a tendency to decreased total pulmonary diffusion capacity and vital capacity. Significant respiratory symptoms were not noted. Long-term spore exposure may include an occupational health risk to chip pile workers.

Adult↗

Reduced lung function in leukaemia patients undergoing bone marrow transplantation.

20 patients with leukaemia in remission or early relapse received an allogeneic bone marrow graft. Patients were conditioned according to Seattle protocol with high dose cyclophosphamide and total body irradiation to a total dose of 8 Gray to the lungs delivered by a 6 MV linear accelerator at a dose rate of approximately 0.06 Gray/min. As prophylaxis against the graft versus host reaction, methotrexate and/or cyclosporin A were given. Lung function was studied prior to treatment and every 3 months thereafter. Before treatment a marked decrease was seen in the CO diffusion capacity when compared to the predicted value, while the flow volume relationships were within normal limits. The cause of this is unknown, but may be due to previous infections and the cytostatic treatment given. After bone marrow transplantation, a further irreversible decrease was seen in the CO diffusion capacity and vital capacity indicating an additive effect of the lung irradiation.

Adolescent↗

A hydrostatic weighing method using total lung capacity and a small tank.

The purpose of this study was to establish the validity and reliability of a hydrostatic weighing method using total lung capacity (measuring vital capacity with a respirometer at the time of weighing) the prone position, and a small oblong tank. The validity of the method was established by comparing the TLC prone (tank) method against three hydrostatic weighing methods administered in a pool. The three methods included residual volume seated, TLC seated and TLC prone. Eighty male and female subjects were underwater weighed using each of the four methods. Validity coefficients for per cent body fat between the TLC prone (tank) method and the RV seated (pool), TLC seated (pool) and TLC prone (pool) methods were .98, .99 and .99, respectively. A randomised complete block ANOVA found significant differences between the RV seated (pool) method and each of the three TLC methods with respect to both body density and per cent body fat. The differences were negligible with respect to HW error. Reliability of the TLC prone (tank) method was established by weighing twenty subjects three different times with ten-minute time intervals between testing. Multiple correlations yielded reliability coefficients for body density and per cent body fat values of .99 and .99, respectively. It was concluded that the TLC prone (tank) method is valid, reliable and a favourable method of hydrostatic weighing.

Adolescent↗

Pulmonary function in tropical eosinophilia before and after treatment with diethylcarbamazine.

Spirometric and lung volume measurements were carried out before and after treatment with diethylcarbamazine in 19 patients with tropical eosinophilia. The total lung capacity and vital capacity returned to or nearly to normal while the FEV1 and PEFR, though improved, tended to remain below normal, indicating some residual airways obstruction especially in patients whose treatment began more than one month from the onset of symptoms. The time taken for the pulmonary function to return to normal with treatment was found to be much longer than for the clinical and haematological response.

Airway Obstruction↗

Closing volume during normal pregnancy.

Serial lung function studies were performed in ten healthy, primiparous women aged 21--28. Measurements were made at two-monthly intervals during pregnancy and included functional residual capacity (FRC), total lung capacity (TLC), vital capacity (VC), specific conductance (SGaw) and closing volume (CV) on each occasion. Closing volume expressed as formular: (see text), showed a progressive rise during pregnancy in all subjects with a linear relationship to time (P less than 0.001, P greater than 0.01, respectively). No consistent changes in lung volume could be shown during pregnancy over the study period. It is suggested that the increase in closing volume during pregnancy might result in abnormalities of distribution of ventilation sufficient to explain the maternal blood gas disturbances of pregnancy.

Adult↗