Aspects Of Pulmonary Function Research Articles

Pulmonary Manifestations of GATA2 Deficiency

GATA2 deficiency is a genetic disorder of hematopoiesis, lymphatics, and immunity caused by autosomal dominant or sporadic mutations in GATA2. The disease has a broad phenotype encompassing immunodeficiency, myelodysplasia, leukemia, and vascular or lymphatic dysfunction as well as prominent pulmonary manifestations. What are the pulmonary manifestations of GATA2 deficiency? A retrospective review was conducted of clinical medical records, diagnostic imaging, pulmonary pathologic specimens, and tests of pulmonary function. Of 124 patients (95 probands and 29 ascertained), the lung was affected in 56%. In addition to chronic infections, pulmonary alveolar proteinosis (11 probands) and pulmonary arterial hypertension (nine probands) were present. Thoracic CT imaging found small nodules in 54%(54 probands and 12 relatives), reticular infiltrates in 40%(45 probands and four relatives), paraseptal emphysema in 25%(30 probands and one relative), ground-glass opacities in 35%(41 probands and two relatives), consolidation in 21%(23 probands and two relatives), and a typical crazy-paving pattern in 7%(eight probands and no relatives). Nontuberculous mycobacteria were the most frequent organisms associated with chronic infection. Allogeneic hematopoietic stem cell transplantation successfully reversed myelodysplasia and immune deficiency and also improved pulmonary hypertension and pulmonary alveolar proteinosis in most patients. GATA2 deficiency has prominent pulmonary manifestations. These clinical observations confirm the essential role of hematopoietic cells in many aspects of pulmonary function, including infections, alveolar proteinosis, and pulmonary hypertension, many of which precede the formal diagnosis, and many of which respond to stem cell transplantation.

Comparison of the effects of core stabilization and chest mobilization exercises on lung function and chest wall expansion in stroke patients.

[Purpose] The main purpose of this study was to compare the effects of core stabilization and chest mobilization exercises on pulmonary function and chest expansion in chronic stroke patients. [Subjects and Methods] Thirty stroke patients were randomly divided into two groups: a core stabilization exercise group (n=15) and a chest mobilization exercise group (n=15). Each exercise was performed 3 times per week for 30 minutes for 4 weeks, and pulmonary function and chest expansion when breathing were measured for both groups. [Results] There were significant increases in both forced vital capacity and forced expiratory volume in 1 second before and after intervention. Core stabilization exercise resulted in a significant increase in peak expiratory flow, and significant increases in upper and lower chest expansion were detected with chest mobilization exercise. However, no significant difference was revealed between the two groups. [Conclusion] This study suggested that both exercises were effective in some aspects of pulmonary function while core stabilization can help increase peak expiratory flow and chest mobilization can assist with chest expansion.

Year in review 2015: Interstitial lung disease, pulmonary vascular disease, pulmonary function, sleep and ventilation, cystic fibrosis and paediatric lung disease.

Keywords: interstitial lung disease; lung function; paediatric lung disease; pulmonary vascular disease; sleep

Career perspective: John B West.

I have been fortunate to work in two areas of extreme physiology and medicine: very high altitude and the microgravity of spaceflight. My introduction to high altitude medicine was as a member of Sir Edmund Hillary's Silver Hut Expedition in 1960–1961 when a small group of physiologists spent the winter and spring at an altitude of 5,800 m just south of Mt. Everest. The physiological objective was to obtain a better understanding of the acclimatization process of lowlanders during exposure to a very high altitude for several months. As far as we knew, no one had ever spent so long at such a high altitude before. The success of this expedition prompted me to organize the 1981 American Medical Research Expedition to Everest where the scientific objective was to determine the physiological changes that allow humans to survive in the extreme hypoxia of the highest point on earth. There is good evidence that this altitude is very near the limit of human tolerance to oxygen deprivation. Much novel information was obtained including an extraordinary degree of hyperventilation which reduced the alveolar partial pressure of carbon dioxide (Pco2) to about 8 mmHg (1.1 kPa) on the summit, and this in turn allowed the alveolar partial pressure of oxygen, PO2, to be maintained at a viable level of about 35 mmHg (4.7 kPa). The low Pco2 caused a severe degree of respiratory alkalosis with an arterial pH exceeding 7.7. These were the first physiological measurements to be made on the Everest summit, and essentially, none has been made since. The second extreme environment is microgravity. We carried out an extensive series of measurements on astronauts in the orbiting laboratory known as SpaceLab in the 1990s. Many aspects of pulmonary function are affected by gravity, so it was not surprising that many changes were found. However, overall gas exchange remained efficient. Some of the findings such as an anomalous behavior of inhaled helium and sulfur hexafluoride have still not been explained. Measurements made after astronauts were exposed to 6 months of microgravity in the International Space Station indicate that the function of the lung returns to its preexposure state within a few days.

What Is the Role of PFTs in Monitoring Adverse Effects of Surgery, Drug Treatments, Radiation Therapy, and During Hospitalization?

Measurement of various aspects of pulmonary function is a relatively easy, noninvasive, and inexpensive way to gauge the status of the respiratory system. Interest in using these tests to determine risk from medical and surgical interventions stems from their presumed ability to be more sensitive than history or physical examination in detecting underlying lung disease. When coupled with the assumption that early detection of pulmonary abnormalities will lead to alterations in patient management, the ultimate goal of improved patient outcomes becomes very attractive. However, despite advances in technology, achievement of this goal has proved to be more challenging than expected in many areas. This paper attempts to review the literature addressing several of the more difficult of these areas. It is clear that more research, involving more rigorously designed studies, will be necessary, before definitive answers are available.

Silicose

This chapter presents a review of the national and international literature on the subject of silicosis. Epidemiological, pathological, diagnostic and pulmonary function aspects are discussed. The clinical classification and methods of treating silicosis are reviewed, as are other silicarelated diseases such as tuberculosis, lung cancer and scleroderma. Prevention and medical-legal aspects are also discussed.

<![CDATA[Pulmonary function aspects after myocardial revascularization related to preoperative risk]]>

<![CDATA[Pulmonary function aspects after myocardial revascularization related to preoperative risk]]>

Antenatal steroids, postnatal surfactant, and pulmonary function in premature rabbits.

Antenatal corticosteroids reduce the incidence of the respiratory distress syndrome and improve pulmonary mechanics at least in part by mechanisms other than surfactant stimulation. We measured several aspects of pulmonary function in rabbits to better understand the mechanisms involved. Seven does were given intramuscular betamethasone and six were given vehicle on days 25 and 26 of gestation. Delivery was on day 27 (term = 31). Half of the fetuses from each litter were given rabbit surfactant before the first breath. All fetuses were then ventilated at a consistent tidal volume for 1 h. Pulmonary function tests included static and dynamic compliance, expiratory time constant, stress relaxation, total lung resistance, and total lung conductance. Steroid or surfactant treatment increased dynamic compliance, and the effects of both together were greater than either alone. Static compliance was affected more by surfactant than steroids, whereas lung resistance and conductance were affected more by steroids. The differences in action of the two therapies help account for the increased dynamic compliance seen with combination therapy.

Detection of Circadian Variation in Pulmonary Function Using peak Expiratory Flow and Forced Expiratory Volume Measurements

IN the clinical environment, pulmonary function tests are used to monitor the progression of respiratory disorders and the patient's response to treatment. The t w o most commonly used by physiotherapists are those of forced expiratory volume (FEV, and peak expiratory flow rate (PEFR); both of these tests are considered indices of airway resistance. The purpose of this study was to determine whether FEV, and PEFR measurements could detect the presence of a circadian variation in airway resistance in healthy subjects. Circadian variation (variation occurring within a 24-hour period) is considered to be larger than the response of the airways to bronchodilator drugs. Hence an improvement in the results of these two tests may not necessarily reflect an improvement in a patient's condition. Measurements of FEV, and PEFR were recorded by a Vitalograph spirometer and function analyser, and a Wright peak f low meter. The validity and reliability of the equipment were confirmed prior to the main study. Measurements of FEV,, PEFR and oral temperature were recorded from eight healthy subjects at three-hourly intervals over a 24-hour period. This procedure was repeated on three days to determine the reliability of the data. Oral temperature was monitored as it is considered to be one of the main influencing factors on the 'body clock'. The activities and food/drink intake of the subjects was restricted and they were encouraged to maintain their normal sleep/wakefulness patterns. The data were analysed using cosinor analysis and t-tests. The former considers a mathematical model of the rhythm which is best approximated by a sine or cosine wave and tests the fit. The group of subjects demonstrated significant circadian rhythm (p<0.05) in oral temperature on all three days, while the measurements of PEFR and FEV, were non-significant (p<0.05) for each day. The circadian variation in oral temperature, PEFR and FEV, was significant (p<0.05) for the group of subjects on each of the three days. The analysis of the individual results revealed five significant rhythms (p<0.05) in FEV, and three in PEFA during the three days. These rhythms only occurred concomitantly on one day in one subject. This study indicates that airway resistance does vary significantly during the day. An interesting feature is the lack of concomitant rhythms between PEFR and FEV,. It suggests the t w o tests are not measuring the same aspects of pulmonary function. This has many clinical implications and requires further investigation.

The Asthmatic Child's Participation in Sports and Physical Education

Asthma is a chronic pulmonary disorder, frequently allergic in nature, and characterized by paroxysms of dyspnea, wheezing, tightness in the chest, and bronchospasm. Asthmatic attacks may be minor and short in duration with little discomfort, or very severe and of long duration, producing the characteristic picture of intractability. During symptomatic periods, it is usually possible to demonstrate change in certain aspects of pulmonary function, notably expiratory flow rate and forced expiratory volume. With mild symptoms or between the episodes of severe asthma, the individual may be at little or no disadvantage in most activities. Continuous exercise for five to eight minutes in cold air often causes dyspnea, wheezing, and bronchospasm (exercise-induced asthma) in an asymptomatic individual who may or may not have a history of having had asthma or hay fever. When symptoms of pulmonary distress become severe or prolonged, they may lead to interruption of the child's daily routine, including school attendance. Occasionally, such children may become home or hospital bound for periods of time. Between the two extremes of no symptoms and severe asthma, there is a spectrum of respiratory or pulmonary disability—the nature and severity of which require that each child receive individual consideration and evaluation in the matter of his daily activity. Control of asthma in children has significantly improved during the past decade. However, asthma may contribute to inefficiency in schoolwork because of associated chronic fatigue, irritability, decreased attention span, and emotional factors. Physical activities are useful to asthmatic children. The majority of asthmatic children can participate in physical activities at school and in sports with minimal difficulty, provided the asthma is under satisfactory control.

Hydrochlorothiazide-Induced Pulmonary Edema

Immediately following the initial ingestion of hydrochlorothiazide, acute pulmonary edema developed in a patient who previously used bendroflumethiazide. Unlike previously published reports, we studied the patient from immunologic and pulmonary function aspects. Using standard techniques, no significant immunological mechanisms could be demonstrated. The pulmonary function testing disclosed a widened alveolararterial oxygen gradient and mildly decreased diffusing capacity that cleared on serial testing. We believe that this demonstrates a rare idiosyncratic reaction that apparently is seen specifically with hydrochlorothiazide and not with other thiazide medications. (<i>Arch Intern Med</i>139:817-819, 1979)

Hydrochlorothiazide-induced pulmonary edema. Report of a case and review of the literature

Immediately following the initial ingestion of hydrochlorothiazide, acute pulmonary edema developed in a patient who previously used bendroflumethiazide. Unlike previously published reports, we studied the patient from immunologic and pulmonary function aspects. Using standard techniques, no significant immunological mechanisms could be demonstrated. The pulmonary function testing disclosed a widened alveolar-arterial oxygen gradient and mildly decreased diffusing capacity that cleared on serial testing. We believe that this demonstrates a rare idiosyncratic reaction that apparently is seen specifically with hydrochlorothiazide and not with other thiazide medications.

Changes of pulmonary function following transfusion of stored blood.

Ventilatory volumes, blood gases and other aspects of pulmonary function were measured before and after intra‐operative transfusion in 16 patients who had undergone operation under general anesthesia. One group consisted of eight patients transfused with 1275 ml of stored blood passed through a standard nylon mesh filter (SF group) and the other consisted of eight patients transfused with stored blood passed through a dacron wool filter (DWF group).Since respiration of the patients was depressed slightly due to preanesthetic sedation, lowered Ve, Vt, and elevated Paco2, respiratory dead space ratio Vd/Vt were observed either in the SF or DWF group before anesthesia and transfusion. Even after the recovery of patients from anesthesia Vd/Vt remained high and Feco2 decreased in the SF group. In contrast, Vd/Vt decreased almost to normal and Feco2 remained normal in the DWF group. Fraction of physiological shunt Qs/Qt tended to decrease after anesthesia and transfusion either in the SF or DWF group. The ventilation‐perfusion ratio increased relatively more markedly and pHa elevated definitely in the SF group after transfusion.The above data indicate that microembolism occurs after transfusion of stored blood, even in an amount of 1,000 ml, with a standard nylon mesh filter and concealed pulmonary dysfunction is observable. Therefore, a fine filter, such as a dacron wool filter or a polyester mesh filter, is recommended to avoid the pulmonary microembolism and the consequent respiratory distress following the transfusion of stored blood.

The effect of long-term oral administration of a bronchodilator, a sustained-release aminophylline preparation, on pulmonary function (author's transl)

Various aspects of pulmonary function were measured in eight patients with chronic bronchial obstructive disease, after five-month oral administration of a sustained-release aminophylline preparation (BY 108: Euphyllin retard). In addition to a sustained bronchodilator effect there was an improvement in static pulmonary volumes and a marked increase in forced expiratory volumes. The reduction in residual volume and of midcapacity is felt to be of particular importance in the prevention of pulmonary emphysema.

The Asthmatic Child and His Participation in Sports and Physical Education

Bronchial asthma is a chronic pulmonary disorder, frequently allergic in nature, and characterized by paroxysms of dyspnea, wheezing, tightness in the chest, and bronchospasm. Asthmatic attacks may be minor and short in duration with little discomfort, or they may be very severe and of long duration, producing the characteristic picture of intractability. During symptomatic periods, it is usually possible to demonstrate changes in certain aspects of pulmonary function. With mild symptoms or between the episodes of severe asthma, the individual may be at little or no disadvantage in any or all activities. However, when the symptoms of pulmonary distress become severe or prolonged, this may lead to interruption of the child's daily routine, including school attendance. Occasionally, such children may become home or hospital bound for long periods of time. Between the two extremes of no symptoms and severe asthma, there is a spectrum of respiratory or pulmonary disability—the nature and severity of which requires that each child receive individual consideration and evaluation in the matter of his daily activity. The outlooks for the control of asthma in children has been improving during the past several decades. However, with the increase in population, there is an increasing number of children who require medical management for this disorder. It is a leading medical cause for school absenteeism and probably contributes to inefficient school work because of chronic fatigue, irritability, decreased attention span, and secondary emotional disorders. There is general agreement among physicians that most children with bronchial asthma should attend regular school since, when under proper control and with no residual pulmonary defect the child needs no special facilities.

Some aspects of pulmonary function after rapid saline infusion in healthy subjects.

1. Rapid intravenous (i.v.) infusions of saline were administered to five healthy male volunteers. Measurements were made of static and dynamic lung volumes, ‘closing volume’ and pulmonary compliance before and after infusion; all measurements were made in the seated upright position. 2. Following a 1 litre infusion small decreases occurred in static and dynamic lung volumes in all studies and were associated in each case with an increase in ‘closing volume’. ‘Closing volume’ returned to normal within 1 h of the infusion. 3. After 2 litre infusions more marked decreases in all lung volumes occurred and were associated with variable changes in ‘closing volume’. The pattern of change varied between individual subjects and this is thought to reflect differences in localization within the lungs of the effects of the saline load in different subjects. 4. After 2 litre infusions static compliance was decreased in all subjects during the first 10–15 min. Changes in dynamic compliance showed marked individual variation.

THE ASTHMATIC CHILD AND HIS PARTICIPATION IN SPORTS AND PHYSICAL EDUCATION

Bronchial Asthma is a chronic pulmonary disorder, frequently allergic in nature, and characterized by paroxysms of dyspnea, wheezing, tightness in the chest, and bronchospasm. Asthmatic attacks may be minor and short in duration with little discomfort, or they may be very severe and of long duration, producing the characteristic picture of intractability. During symptomatic periods, it is usually possible to demonstrate changes in certain aspects of pulmonary function. With mild symptoms or between the episodes of severe asthma, the individual may be at little or no disadvantage in any or all activities. However, when the symptoms of pulmonary distress become severe or prolonged, this may lead to interruption of the child's daily routine, including school attendance. Occasionally, such children may become home or hospital bound for long periods of time. Between the two extremes of no symptoms and severe asthma, there is a spectrum of respiratory or pulmonary disability—the nature and severity of which requires that each child receive individual consideration and evaluation in the matter of his daily activity. The outlook for the control of asthma in children has been improving during the past several decades. However, with the increase in population, there is an increasing number of children who require medical management for this disorder. It is a leading medical cause for school absenteeism and probably contributes to inefficient school work because of chronic fatigue, irritability, decreased attention span, and secondary emotional disorders. There is general agreement among physicians that most children with bronchial asthma should attend regular school since, when under proper control and with no residual pulmonary defect, the child needs no special facilities.

Clinical value of radioactive lung scanning.

Radioisotope scanning with I131 labelled mac-roaggregated human serum albumin was performed in a series of 177 patients. Scan indications were suspected pulmonary embolism, suspected or verified pulmonary tumors and miscellaneous conditions. Scanning was negative in only one of 38 patients with demonstrated pulmonary embolism. The scan data in combination with clinical picture and radiological findings greatly improved both recognition and localization of lung emboli, thus providing a basis for early and correct treatment. In the group of malignant tumors 48 out of 50 patients had positive findings on scanning. Little information was gained in the group of small and peripheral lung tumors, whether malignant or benign. Lung scanning seemed to be of doubtful value in the early diagnosis of pulmonary tumors. The examination was useful in tumor patients as a safe and simple method in the preoperative evaluation of some aspects of pulmonary function.

MIST THERAPY

AN ARTICLE on mist tent therapy in this issue opens with the remarkable comment that no objective evidence of the efficacy of mist in patients with cystic fibrosis has been published. The authors conducted a clinical study which led to the conclusion that under the conditions of their study mist was effective as judged by improvement in some aspects of pulmonary function. It may be even more remarkable to note the absence of objective evidence of the role of mist in the wide variety of circumstances in which it is commonly used on most pediatric services. A review of some physical principles involved in water exchange, of observations on the deposition of aerosolized particles in the lung, and of the uses and hazards of mist therapy seems pertinent.

Radiological diagnosis of nonuniform pulmonary ventilation.

Laboratory methods of determining many aspects of pulmonary function have become remarkably accurate and sophisticated in the past ten years. Application of these new tests, however, has been limited by the fact that many of them require expensive apparatus not readily available, skilled technicians, and considerable time and effort. In a previous paper (1) we described the qualitative information about disordered ventilatory capacity which one might obtain by appropriate roentgen methods which are easily available. In the present paper the author proposes to discuss another aspect of disturbed pulmonary function in which ventilation is uneven in relation to capillary blood flow and to show the radiologic evidence which suggests that this disturbance is present. The efficient performance of gas exchange (O2 and CO2), which is the primary function of the lungs, requires a nice matching between the amount of air circulating through alveoli and the quantity of blood passing along the alveolar walls. In the normal lung, the air-blood volume relationships are constant for any particular lung area although these constants vary somewhat depending on location within the chest (2). One might consider, however, the normal average situation to be one in which alveolar ventilation equaled 2 liters∕min.∕lung, and capillary blood flow 2.5 liters∕min.∕lung, resulting in a ratio of 0.8 (Fig. 1). When injury or disease affects a localized area of lung, either ventilation or blood flow may be impaired selectively (Figs. 2 and 3). This may be accompanied by a compensatory decrease in the reciprocal function, which, however, seldom produces a new balance with a normal ratio of 0.8. Thus, even though total lung volumes may be adequate, mismatching of ventilation and blood flow results in the hypoxemia that accompanies pulmonary insufficiency and failure in many different clinical conditions (3). Many years ago, Golden (4) showed that the pattern of the vascular markings in the lungs, as seen by fluoroscopy and films, reflected accurately the symmetrical changes which occur in the tracheobronchial tree during respiration in the normal person. Since the blood vessels parallel the branching of the airways, irregularity and asymmetry of the vascular shadows in any area of the lung mean that the distribution of air in that region is uneven. Therefore, close inspection of roentgenograms of the chest made in various projections may lead to a diagnosis of nonuniform ventilation, localized or generalized (Figs. 4 and 5). This evaluation of qualitative pulmonary dysfunction can be quite accurate when compared with laboratory tests of lung function (Fig. 6). Discussion Nonuniform pulmonary ventilation may lead to alveolar hypoventilation and result in chronic hypoxemia.