Significant Airflow Research Articles

Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy

Lung function in children with neuroendocrine cell hyperplasia of infancy (NEHI) and correlations with future clinical outcomes are needed to guide clinical management. To compare results of infant pulmonary function tests (IPFTs) in children with NEHI to disease control (DC) subjects and to correlate NEHI IPFTs with future outcomes. We performed a retrospective, single center study of IPFT in subjects diagnosed by lung biopsy (NEHI) or clinically (NEHI syndrome) and in DC subjects evaluated for cancer or pre-hematopoietic stem cell transplantation (HSCT). Raised volume rapid thoracoabdominal compression (RVRTC) and plethysmography were performed on all infants and evaluated for quality. Standard spirometry measures, room air oxygen saturations (RA O2 sat), and weight percentiles were collected during follow up. Fifty-seven IPFTs were performed in 15 NEHI, 22 NEHI syndrome, and 20 DC subjects. RVRTC and FRC measurements were obtained in 85% or more of subjects in all groups. Significant airflow limitation (FEV0.5 P-value ≤ 0.01) and air trapping (FRC P-value ≤ 0.01) were seen in NEHI and NEHI syndrome subjects compared to DCs. No significant correlations were found between IPFT, oxygen use, RA O2 sat, and weight at the time of the IPFTs. Initial FEV0.5 and FRC z-scores correlated with RA O2 sat (r = 0.60 and -0.49) at short-term follow up (6-12 months). Most measurements of RVRTC correlated with FEV1 (n = 5) measured 4-5 years later (r > 0.50). IPFTs in NEHI subjects are feasible, demonstrate significant obstruction and air trapping, and correlate with future RA O2 sat and FEV1 . IPFTs may provide valuable clinical information when caring for NEHI patients. Pediatr Pulmonol. 2013; 48:1008-1015. © 2012 Wiley Periodicals, Inc.

Questionnaires and Pocket Spirometers Provide an Alternative Approach for COPD Screening in the General Population

In response to the Agency for Healthcare Research and Quality statement questioning the usefulness of “screening spirometry,” the National Heart, Lung, and Blood Institute and the COPD Foundation held a consensus conference in June 2008 to establish a procedure to detect cases of COPD in the general population. Conference participants developed a three-stage approach, using a brief questionnaire, peak flow measurement with a pocket spirometer, and diagnostic quality spirometry. The overall objective of this study was to examine the usefulness of a simple questionnaire and peak flow measurement in screening for COPD in a self-selected population. We hypothesized that this combination would efficiently screen for clinically relevant COPD. We queried individuals attending public events regarding the presence of wheeze and/or asthma, mucus production, dyspnea, exposure to irritants, and tobacco use. Peak expiratory flow (PEF) was then measured with a pocket spirometer. If PEF was < 70% predicted, spirometry was performed. In order to estimate the false-negative rate, a random sample of every 10th participant was also selected for spirometry. Between June 2008 and December 2009, 5,761 adults completed the risk assessment questionnaire. The mean age of the respondents was 54 years, 58% were women, and 88% were white. Of these, 5,638 participants completed pocket spirometry, and 315 (5.6%) had PEF < 70% predicted. Of 5,323 with normal PEF, 651 underwent spirometry. The performance of PEF was assessed via positive and negative predictive values relative to a diagnosis of clinically significant airflow obstruction, defined as FEV(1)/FEV(6) < the lower limit of normal and FEV(1) < 60% predicted. Of 4,238 subjects with at least two risk factors, 267 (6.3%) had PEF < 70%, compared with 48 of the 1,400 subjects (3.4%) with fewer than two risk factors (P < .001). Based on 729 participants with acceptable spirometry, 63.1% (113 of 179) of those with abnormal PEF tested positive for clinically significant airflow obstruction, compared with 5.5% (30 of 550) with normal PEF (P < .001). The estimated prevalence of significant COPD among the 5,638 screened was 8.7%, and sensitivity and specificity were 40.7% and 97.7%, respectively. A staged approach to COPD screening in adults is useful for detecting clinically significant airflow obstruction in our study population.

Review of exhaled nitric oxide in chronic obstructive pulmonary disease

The up-regulation of nitric oxide (NO) by inflammatory cytokines and mediators in central and peripheral airway sites can be easily monitored in exhaled air (FENO). It is now possible to estimate the predominant airway site of increased FENO i.e. large versus peripheral airway/alveoli, and its potential pathologic and physiologic role in obstructive lung disease. In asthma, six double-blind, randomized, controlled algorithm trials have reported only equivocal benefits of add-on measurements of FENO to usual clinical guideline management including spirometry. Significant design issues, as emphasized by Gibson, may exist. However, meta-analysis of these six studies (Petsky et al 2012 Thorax 67 199–208) concluded that routine serial measurements of FENO for clinical asthma management does not appear warranted. In COPD including chronic bronchitis and emphysema, despite significant expiratory airflow limitation, when clinically stable as well as during exacerbation, FENO, j′awNO and CANO may all be normal or increased. Furthermore, the role of add-on monitoring of exhaled NO to GOLD management guidelines is less clear because of the absence of conclusive doubleblind, randomized, control trial studies concerning potential clinical benefits in the management of COPD.

Maxillomandibular Expansion and Advancement for the Treatment of Sleep-Disordered Breathing in Children and Adults

Sleep-disordered breathing is a prevalent disorder characterized by repetitive episodes of upper-airway collapse and mechanical obstruction of airflow during sleep. An anatomically smaller pharyngeal airway, often the result of maxillomandibular hypoplasia, causes clinically significant airflow limitation and resultant nocturnal collapse. We discuss the therapeutic effects of rapid maxillary expansion, maxillomandibular expansion, mandibular (or maxillary) distraction osteogenesis, and maxillomandibular advancement for the treatment of sleep-disordered breathing in children and adults.

Noise reduction of a double-skin façade considering opening for natural ventilation

The growing interest in natural or hybrid ventilation systems brings a challenge for good integration of openings in building façades. With a noisy environment, there is an important limitation for the use of direct openings in common building envelopes. As a part of a research project dedicated to this problem, it is possible to evaluate the impact of several double-skin configurations, modifying openings, space between façades or the choice of construction assemblies. Experimental measurements made in laboratory conditions lead to the estimation of usual noise reduction and sound transmission class. Moreover, the airflow at constant differential pressure was assessed as functions of the aperture and compared to sound insulation. Analyzing those parameters together give useful information for the design of passive ventilation with a significant airflow when acoustical performance is an important issue.

Severe asthma in childhood

Children with severe asthma have a high degree of respiratory morbidity despite treatment with high doses of inhaled corticosteroids and are therefore very difficult to treat. This review will discuss phenotypic and pathogenic aspects of severe asthma in childhood, as well as remaining knowledge gaps. As a group, children with severe asthma have a number of distinct phenotypic features compared with children with mild-to-moderate asthma. Clinically, children with severe asthma are differentiated by greater allergic sensitization, increased exhaled nitric oxide, and significant airflow limitation and air trapping that worsens as a function of age. These findings are accompanied by structural airway changes and increased and dysregulated airway inflammation and oxidant stress which may explain the differential nature of corticosteroid responsiveness in this population. Because children with severe asthma themselves are a heterogeneous group, current efforts are focused on improved definition and sub-phenotyping of the disorder. Whereas the clinical relevance of phenotyping approaches in severe asthma is not yet clear, they may provide important insight into the mechanisms underlying the disorder. Improved classification of severe asthma through unified definitions, careful phenotypic analyses, and mechanism-focused endotyping approaches may ultimately advance knowledge and personalized treatment.

Interaction between Asthma and Lung Function Growth in Early Life

The causal direction between asthma and lung function deficit is unknown, but important for the focus of preventive measures and research into the origins of asthma. To analyze the interaction between lung function development and asthma from birth to 7 years of age. The Copenhagen Prospective Studies on Asthma in Childhood is a prospective clinical study of a birth cohort of 411 at-risk children. Spirometry was completed in 403 (98%) neonates and again by age 7 in 317 children (77%). Neonatal spirometry and bronchial responsiveness to methacholine was measured during sedation by forced flow-volume measurements. Asthma was diagnosed prospectively from daily diary cards and clinic visits every 6 months. Children with asthma by age 7 (14%) already had a significant airflow deficit as neonates (forced expiratory flow at 50% of vital capacity second in neonates reduced by 0.34 z score by 1 mo; P = 0.03). This deficit progressed significantly during early childhood (forced expiratory flow at 0.5 seconds in neonates at age 7 reduced by 0.82 z score by age 7; P < 0.0001), suggesting that approximately 40% of the airflow deficit associated with asthma is present at birth, whereas 60% develops with clinical disease. Environmental tobacco exposure, but not allergic sensitization, also hampered airflow growth. Bronchial responsiveness to methacholine in the neonates was associated with the development of asthma (P = 0.01). Children developing asthma by age 7 had a lung function deficit and increased bronchial responsiveness as neonates. This lung function deficit progressed to age 7. Therefore, research into the origins and prevention of asthma should consider early life before and after birth.

Acute, Inflammatory-based Airflow Limitation Does Not Affect Pulmonary Responses To Exercise In Asthmatic Subjects

PURPOSE: Exercise capacity is thought to be compromised in asthmatic subjects. This would especially be the case when exercise is preceded by significant airflow limitation in asthmatics who exhibit variable airway function on a day-to-day basis. The strong bronchodilatory effect of exercise, however, might minimize potential for pre-exercise airflow limitation to influence the responses to exercise. METHODS: Mild-to-moderate asthmatic subjects (n=9) completed exercise-to-exhaustion on a cycle ergometer (85% peak power) on three separate occasions: 1) following a eucapnic voluntary hyperpnea challenge (EVH), 2) a sham to the EVH (SHAM), and 3) a control trial of seated relaxation (CON). Pulmonary function was assessed at baseline and following each intervention. Metabolic rate, minute ventilation and its determinants, and spontaneous exercise flow-volume loops were analyzed at regular intervals during exercise. RESULTS: Subjects exhibited a 21% decrease in the forced expiratory volume 1.0 s (FEV1.0) following EVH (p<0.05 vs. CON and SHAM), and pre-exercise FEV1.0 was significantly lower during EVH compared to CON and SHAM (pre-exercise FEV1.0 = 3.8 ± 0.8, 3.8 ± 1.0, 3.2 ± 0.5 L for CON, SHAM, and EVH, respectively; p<0.05). During exercise, partial forced expiratory maneuvers demonstrated increases in expiratory flow rates compared to baseline during all trials, but the increased airflow was most marked during EVH. Exercise time-to-exhaustion was not different among the three trials (5.6 ± 1.6, 5.1 ± 1.5, 5.6 ± 2.4 minutes for CON, SHAM, and EVH). The ventilatory equivalent for metabolic carbon dioxide production (VE/VCO2), tidal volume to inspiratory capacity ratio (VT/IC), and exercise lung volumes (determined by IC maneuvers) were not different among the three trials. CONCLUSIONS: Acute, inflammatory-based airway dysfunction present immediately before exercise does not affect exercise ventilation or the airway mechanical responses to exercise in asthmatic subjects with mild-to-moderate disease. The powerful bronchodilatory response to exercise thus appears to override the airway dysfunction seen prior to exercise. Funding: Vermont Genetics Network, P20 RR16462 from the INBRE Program of the National Center for Research Resources, a component of the National Institutes of Health (NIH).

Peripheral blood gene expression profiles in COPD subjects

To identify non-invasive gene expression markers for chronic obstructive pulmonary disease (COPD), we performed genome-wide expression profiling of peripheral blood samples from 12 subjects with significant airflow obstruction and an equal number of non-obstructed controls. RNA was isolated from Peripheral Blood Mononuclear Cells (PBMCs) and gene expression was assessed using Affymetrix U133 Plus 2.0 arrays.Tests for gene expression changes that discriminate between COPD cases (FEV1< 70% predicted, FEV1/FVC < 0.7) and controls (FEV1> 80% predicted, FEV1/FVC > 0.7) were performed using Significance Analysis of Microarrays (SAM) and Bayesian Analysis of Differential Gene Expression (BADGE). Using either test at high stringency (SAM median FDR = 0 or BADGE p < 0.01) we identified differential expression for 45 known genes. Correlation of gene expression with lung function measurements (FEV1 & FEV1/FVC), using both Pearson and Spearman correlation coefficients (p < 0.05), identified a set of 86 genes. A total of 16 markers showed evidence of significant correlation (p < 0.05) with quantitative traits and differential expression between cases and controls. We further compared our peripheral gene expression markers with those we previously identified from lung tissue of the same cohort. Two genes, RP9and NAPE-PLD, were identified as decreased in COPD cases compared to controls in both lung tissue and blood. These results contribute to our understanding of gene expression changes in the peripheral blood of patients with COPD and may provide insight into potential mechanisms involved in the disease.

Longitudinal measures of lung function in infants with bronchopulmonary dysplasia

We previously demonstrated that infants with a history of bronchopulmonary dysplasia (BPD) exhibit airflow obstruction and air trapping. The purpose of this study was to assess longitudinal changes in pulmonary function in infants with a history of BPD over the first 3 years of life, and the relationship to somatic growth. Spirometry was measured using the raised volume rapid thoracoabdominal compression technique, and lung volumes measured by plethysmography. Eighteen infants (mean gestational age ± SD 27.3 ± 2.2 weeks, birthweight 971 ± 259 g) underwent two lung function studies. Average age at first test was 58.8 weeks. Spirometry demonstrated significant reductions in forced expiratory volume in 0.5 sec (FEV(0.5), 76.0 ± 15.9% predicted, Z-score -2.13 ± 1.69), forced expiratory flow at 75% of expired forced vital capacity (FEF(75), 54.8 ± 31.1%, -3.58 ± 2.73), and FEF(25-75) (67.8 ± 33.3%, -1.79 ± 1.76). Group mean total lung capacity (TLC) was in the low normal range (82.9 ± 13.5% predicted) and residual volume (RV)/TLC was mildly elevated (122.4 ± 38.2% predicted). Repeat testing was performed an average of 32.7 weeks after initial testing. At re-evaluation, group mean lung volumes and flows tracked at or near their previous values; thus, in general, there was a lack of catch-up growth. However, compared to infants with below average or average somatic growth (as represented by g/day), infants with above average growth showed significantly greater improvements in percent predicted FVC, FEV(0.5), TLC, and RV/TLC (all P < 0.05, ANOVA). We conclude that longitudinal measures of pulmonary function in infants and young children with BPD demonstrate significant airflow obstruction and modest restriction, which tends to persist with time. On the other hand, infants with above average somatic growth showed greater lung growth than their peers. Additional studies examining the effects of various nutritional regimens on lung function are warranted.

Planar nearfield acoustical holography in moving fluid medium at subsonic and uniform velocity

Nearfield acoustical holography (NAH) data measured by using a microphone array attached to a high-speed aircraft or ground vehicle include significant airflow effects. For the purpose of processing the measured NAH data, an improved nearfield acoustical holography procedure is introduced that includes the effects of a fluid medium moving at a subsonic and uniform velocity. The convective wave equation along with the convective Euler's equation is used to develop the proposed NAH procedure. A mapping function between static and moving fluid medium cases is derived from the convective wave equation. Then, a conventional wave number filter designed for static fluid media is modified to be applicable to the moving fluid cases by applying the mapping function to the static wave number filter. In order to validate the proposed NAH procedure, a monopole simulation at the airflow speed of Mach=-0.6 is conducted. The reconstructed acoustic fields obtained by applying the proposed NAH procedure to the simulation data agree well with directly-calculated acoustic fields. Through an experiment with two loudspeakers performed in a wind tunnel operating at Mach=-0.12, it is shown that the proposed NAH procedure can be also used to reconstruct the sound fields radiated from the two loudspeakers.

Kronik Obstrüktif Akciğer Hastalığında (KOAH) Genetik Risk Faktörleri

COPD is a leading cause of morbidity and mortality among the adult population. The major risk factor of COPD is, undoubtedly, cigarette smoking. In a significant proportion of smokers clinically significant airflow obstruction develops. Exogenous factors outside the meeting, increasing susceptibility to disease and genetic factors in the development of the disease is thought to play an important role.Apart from the contribution by the well-recognized α-1 antitrypsin deficiency, the genetic basis of COPD is poorly understood. However, many genes are thought to be associated with COPD. The most important ones are α-1-antitrypsin (AAT), α-1-antichymotrypsin (AACT), matrix metalloproteinase (MMP), microsomal epoxide hydrolase (EPHX1), glutathione S-transferase (GST), tumor necrosis factor-α (TNF ) and transforming growth factor beta (TGFβ).

Theoretical study of the impact of tide-induced airflow on hydraulic head in air-confined coastal aquifers

Tide-induced hydraulic head fluctuation results in significant airflow above the water table if the aquifer is air-confined, for example, if the aquifer is capped by low permeability materials. Hypothetical studies on the interaction between tidal fluctuation and airflow in coastal aquifers are carried out using an air–water two-phase numerical model. The results show that both the amplitude of the hydraulic head fluctuation and the distance of influence of the tidal fluctuation increase due to the impact of airflow. For a fixed distance from the coastline, the amplitude of the hydraulic head fluctuation increases with depth, and the hydraulic head fluctuation at small depths lags that at great depths. Tide-induced airflow may be so strong that considerable errors in estimating hydraulic parameters could be generated when models are applied without considering airflow. In an air-confined aquifer, if airflow is neglected, the hydraulic diffusivity could be considerably overestimated. Citation Guo, H.-P. & Jiao, J. J. (2010) Theoretical study of the impact of tide-induced airflow on hydraulic head in air-confined coastal aquifers. Hydrol. Sci. J. 55(3), 435–445.

Planar nearfield acoustical holography in high‐speed subsonic flow.

The objective here is to develop a nearfield acoustical holography (NAH) theory that includes the effects of high‐speed, subsonic flow. Recently, the speed of a transportation means such as aircraft or vehicle has significantly increased, e.g., too close to the speed of sound. As a result, the NAH data measured with a microphone array fixed on an aircraft or vehicle entail significant airflow effects such as a Doppler shift in wave number domain. Here, a convective wave equation is used to define a mapping function. The mapping function is then used to derive a NAH theory that includes the Doppler shift effect by mapping the convectional NAH procedure in the wave number domain. A numerical simulation with a monopole is performed at the high‐speed airflow of Mach = 0.7. The reconstructed acoustic fields obtained by applying the proposed NAH procedure to the simulation data match well with the exact fields. Through an experiment with two loudspeakers performed in a wind tunnel at the speed of Mach = 0.1, it...

Bronchopulmonary dysplasia: Definitions and long-term respiratory outcome

Bronchopulmonary dysplasia (BPD) is a chronic respiratory disease that develops as a consequence of perinatal/neonatal lung injury, and it is one of the most important sequelae of premature birth. In this article we discuss recent changes in the definition of BPD, the main differences between the old and the new form and we summarize recent data on long-term respiratory outcome. The diagnosis of BPD is currently based on the need for supplemental oxygen for at least 28 days after birth, and its severity is graded according to the respiratory support required at 36 postmenstrual weeks. The “new BPD” is mainly a developmental disorder in which the immature lung fails to reach its full structural complexity. Longitudinal studies on children with BPD identified, at all ages, a greater need to use inhaled asthma medication and a significant airflow obstruction. Whether survivors of BPD and prematurity have a risk of developing a COPD-like phenotype with aging is a question that only lung function studies extended to middle-age and beyond will answer.

Treatment of asthmatic bronchoconstriction by percutaneous low voltage vagal nerve stimulation: case report.

Background: A 34 year old Hispanic male patient presented himself in the emergency room with recurring severe asthma that was unresponsive to b-adrenergic and steroid therapy. Spirometry demonstrated significant airflow obstruction. Objective: We sought to test the ability of low voltage vagus nerve stimulation to induce bronchodilation and improve airflow.Method: A percutaneous electrode was inserted into the patient’s neck and positioned to lie in the vicinity of the carotid sheath. Following placement, stimulation was administered and the signal amplitude slowly increased until muscle twitching or discomfort was reported by the patient then reduced to a comfortable level. Results: Within minutes of stimulation, the patient reported reduced dyspnea that was confirmed by spirometry fifteen minutes later (FEV1 increased from 2.70 L to 3.18 L). The FEV1 remained elevated during the 180min treatment (3.29 ± 0.04 L) and at 30min post stimulation (3.36 L). The FEV1 then decreased to 2.84 L 60min after stimulation ended. Conclusion: This finding indicates that low voltage vagus nerve stimulation could be a useful critical care treatment to alleviate smooth muscle bronchoconstriction and offer a new therapeutic approach to the treatment of acute asthma.

Summary of Suggestions From the Task Force for Mass Critical Care Summit, January 26–27, 2007

2008;133;1S-7S Chest Geiling and Lewis Rubinson Asha Devereaux, Michael D. Christian, Jeffrey R. Dichter, James A. * 27, 2007 − January 26 Force for Mass Critical Care Summit, Summary of Suggestions From the Task http://chestjournal.chestpubs.org/content/133/5_suppl/1S.full.html services can be found online on the World Wide Web at: The online version of this article, along with updated information and ISSN:0012-3692 ) http://chestjournal.chestpubs.org/site/misc/reprints.xhtml ( written permission of the copyright holder. this article or PDF may be reproduced or distributed without the prior Dundee Road, Northbrook, IL 60062. All rights reserved. No part of Copyright2008by the American College of Chest Physicians, 3300 Physicians. It has been published monthly since 1935. is the official journal of the American College of Chest Chest

Electrical percutaneous tibial stimulation modulates within-a-breath respiratory drive in man

A sharp intake of breath followed by a strong vocalisation is widely observed in response to acute pain although its function and mechanism is poorly understood. This study investigated the effect of percutaneous (overlying the tibial bone) electrical stimulation delivered early (20-30% of inspiratory time) during inspiration (INSP) or expiration (EXP) (20-30% of expiratory time) at sensory intensities at (100%), above (125%) and below (50% and 75%) the pre-determined pain threshold (PT), upon within-a-breath respiratory parameters (via pneumotachography). All INSP stimulation intensities provoked significant inspiratory time shortening thereby elevating mean inspiratory flow. Tidal volume, but not peak flow was increased in response to 100% PT and 125% PT stimulation (vs. PRE). Shortening and increased tidal volume combined to evoke significant mean inspiratory airflow increments. In contrast, EXP stimulation failed to evoke any effect. Thus, our study provides evidence of a within-a-breath inspiratory-specific, augmentory response to noxious stimulation.

Peri‐operative considerations for Anderson‐Fabry disease

A patient with Anderson-Fabry disease presented for non-heart beating renal transplantation. A literature search revealed only one published case report on the anaesthetic implications [1]. Anderson-Fabry disease is a rare, panethnic, X-linked lysosomal storage disorder. It is the second commonest of the lysosomal storage disorders, following Gauchers, with an estimated incidence of between 1 in 117 000 in Australia [2] to 1 in 470 000 in the Netherlands [3]. The underlying defect is an inborn deficiency of α-galactosidase A. This leads to an accumulation of glycosphingolipids within the plasma and lysosomes of endothelial and smooth muscle cells. In particular, globotriasylceramide accumulates in the kidneys, myocardium, pancreas and lungs. The clinical features are numerous, but of particular clinical importance are impaired renal and respiratory function (which is significantly exacerbated in smokers), peripheral nervous system disease and cerebrovascular disease. Global concentric ventricular hypertrophy, valvular abnormalities and cardiac conduction disturbance are common in affected males, especially after the age of 30 years. Our patient had an estimated GFR < 15 ml.min−1. He had angiokeratomas, acroparaesthesia, ophthalmic signs and had a history of previous stroke. Pre-operative evaluation also revealed moderate concentric left ventricular hypertrophy. General anaesthesia was induced with fentanyl and propofol, and tracheal intubation facilitated with atracurium. Anaesthesia was maintained with sevoflurane in air and oxygen. Approximately 10 min after induction he developed bronchospasm. His peak airway pressures were elevated, tidal volumes reduced and on auscultation there was a widespread expiratory wheeze; there were no significant changes in his heart rate or blood pressure and oxygen saturations were maintained above 95%. Fibreoptic bronchoscopy revealed frothy sputum and oedematous airway mucosa. These respiratory signs settled spontaneously over the next 15–20 min without any specific intervention and the rest of the intra-operative course was uneventful. A blood sample taken 1 h later was sent for mast cell tryptase, which was normal. The bronchospasm was initially attributed to atracurium, but a further literature search revealed that many patients with Anderson-Fabry disease demonstrate significant airflow obstruction and have intrinsic airway disease. Disease of the airways appears to be significantly worse in smokers and older males [4]. This may account for the transient bronchospam or at least have contributed to it. Postoperatively, there were no thrombotic or respiratory complications, but graft function was delayed for 2 days and he was temporarily dialysed. He is now home and recovering well after a successful transplant. In summary, the pre-operative assessment of Anderson-Fabry disease should concentrate on end-organ damage to the heart, brain, lungs and kidneys. The older the patient, the more likely it is that they will have a significant degree of organ impairment that will require consideration before major surgery. Investigations should include urinalysis, 12-lead ECG, echocardiography, spirometry and a comprehensive renal assessment. Respiratory function should be carefully assessed in those who continue to smoke and pre-operative treatment with hydrocortisone should perhaps be considered. The need for bronchodilators must be anticipated and avoiding drugs and clinical interventions that are commonly associated with histamine release appears sensible. In elective cases we also advocate having a low threshold for noninvasive cardiac stress tests in those > 30 years of age and relevant symptoms.

The safety and tolerability of adenosine in patients with obstructive airways disease

Controversy exists regarding the safety of intravenous dipyridamole in patients with severe chronic obstructive pulmonary disease (COPD), and it is contraindicated in patients with asthma. There is currently little published literature on the safety of adenosine in patients with airways disease, despite potential advantages over dipyridamole with respect to side effects. We studied 46 consecutive patients with a history of COPD or asthma undergoing adenosine stress myocardial perfusion scintigraphy. Spirometry with measurement of forced expiratory volume in 1 s (FEV 1), forced vital capacity, peak expiratory flow rate and a repeat FEV 1 postinhaled bronchodilator in all those with a history of asthma was performed prior to receiving intravenous adenosine 140 mg/kg/min for 4 min. The cohort exhibited significant airflow limitation on spirometry (see Table 1), however the majority of patients (24/46) did not experience any dyspnoea or chest pain during adenosine infusion. Fourteen patients experienced chest discomfort during adenosine, and 9 complained of dyspnoea. No patient required aminophylline or resuscitative measures. In our cohort of patients with a history of COPD, asthma or both who demonstrated impaired lung function on spirometry, adenosine was safe and well tolerated.