A speech therapy treatment protocol for exercise induced laryngeal obstruction.

Background: Exercise induced laryngeal obstruction (EILO) is a common cause of exertional breathing problems in young individuals, relevant to 5%-7% of young people. It is caused by paradoxical inspiratory adduction of laryngeal structures and diagnosed by continuous visualization of the larynx during high intensity exercise. Empirical data suggest that EILO consists of different subtypes that require different therapeutic approaches. Currently applied treatment approaches do not rest on randomized controlled trials (RCTs), and thus evidence-based guidelines cannot be established. This protocol describes the speech therapy treatment approach which is applied to EILO patients in a large prospective RCT called HelpILO. Methods and analysis: Consenting patients consecutively diagnosed with EILO at Haukeland University Hospital are randomized into four treatment arms. Speech therapy is represented in two of these, provided in a structured manner based on general speech therapy principles and abdominal breathing, combined with experience obtained with these patients at our hospital during the last decades. The main outcome measure of HelpILO is changes of laryngoscopically visualized laryngeal adduction, rated at peak exercise before vs. after interventions, using a validated scoring system. Ethics and dissemination: Despite widespread use of speech therapy in patients with EILO, this approach is insufficiently tested in RCTs, and the study is therefore considered ethically appropriate. The study will provide knowledge listed as a priority in a recent statement issued by major respiratory and laryngological societies and requested by clinicians and researchers engaged in this area. The results will be presented at relevant conferences, patient fora, and media platforms, and published in relevant peer reviewed international journals.

Is it feasible to perform a continuous laryngoscopy exercise test with a cold air inhalate? A case report.

The continuous laryngoscopy exercise (CLE) test can be combined with breathing cold air. Combining the CLE test and cold air does not cause excessive discomfort. The indicated laryngeal responses with cold air support the need for more research. https://bit.ly/3AChJ7V.

Exercise-induced Laryngeal Obstruction: Protocol for a Randomized Controlled Treatment Trial.

BACKGROUND: Exercise-induced laryngeal obstruction (EILO) is a common cause of exertional breathing problems in young individuals, caused by paradoxical inspiratory adduction of laryngeal structures, and diagnosed by continuous visualization of the larynx during high-intensity exercise. Empirical data suggest that EILO consists of different subtypes, possibly requiring different therapeutic approaches. Currently applied treatments do not rest on randomized controlled trials, and international guidelines based on good evidence can therefore not be established. This study aims to provide evidence-based information on treatment schemes commonly applied in patients with EILO. METHODS AND ANALYSIS: Consenting patients consecutively diagnosed with EILO at Haukeland University Hospital will be randomized into four non-invasive treatment arms, based on promising reports from non-randomized studies: (A) standardized information and breathing advice only (IBA), (B) IBA plus inspiratory muscle training, (C) IBA plus speech therapy, and (D) IBA plus inspiratory muscle training and speech therapy. Differential effects in predefined EILO subtypes will be addressed. Patients failing the non-invasive approach and otherwise qualifying for surgical treatment by current department policy will be considered for randomization into (E) standard or (F) minimally invasive laser supraglottoplasty or (G) no surgery. Power calculations are based on the main outcomes, laryngeal adduction during peak exercise, rated by a validated scoring system before and after the interventions. ETHICS AND DISSEMINATION: The study will assess approaches to EILO treatments that despite widespread use, are insufficiently tested in structured, verifiable, randomized, controlled studies, and is therefore considered ethically sound. The study will provide knowledge listed as a priority in a recent statement issued by the European Respiratory Society, requested by clinicians and researchers engaged in this area, and relevant to 5-7% of young people. Dissemination will occur in peer-reviewed journals, at relevant media platforms and conferences, and by engaging with patient organizations and the healthcare bureaucracy.

Self-Reported Health in Adolescents With Exercise-Induced Laryngeal Obstruction; A Cross-Sectional Study.

Background: Exercise-induced laryngeal obstruction (EILO) is common in young people with exertional breathing difficulties. Psychological characteristics have been proposed as underlying contributors; however, the evidence for this is limited. Objectives: Describe self-reported health, self-efficacy, and anxiety symptoms in adolescents with EILO, and address possible associations with EILO subtypes and severity. Methods: Cross-sectional study of 71/180 (39%) adolescents tested for EILO at Haukeland University Hospital during 2014-2016, age range 14-18 years. Validated questionnaires were used to assess general self-rated health, subjective health complaints (Health Behavior in School-aged Children-Symptom Check List; HBSC-SCL), general self-efficacy (GSE), and anxiety symptoms (SCARED). The outcomes were compared with normative data from comparable unselected populations. Results: The HBSC-SCL items for somatic complaints revealed weekly or more often occurrence of headache in 42%, abdominal pain in 30%, backache in 31%, and dizziness in 32%. For psychological complaints, corresponding figures were 26% for feeling low, 43% for irritability or bad mood, 33% for feeling nervous, and 38% for sleep problems. Mean (range) GSE score was 3.13 (2.2-4.0), and reports suggesting anxiety symptoms were rare. The outcomes were in line with normative data from comparable unselected populations. Self-rated health, and scores obtained for HBSC-SCL, GSE, and SCARED were similarly distributed across EILO subtypes and severity. Conclusion: Self-reported health, self-efficacy, and level of anxiety symptoms in adolescents with laryngoscopically confirmed EILO were similar to data obtained in comparable unselected populations, irrespective of EILO subtype and severity. The findings challenge the notion that pediatric EILO is causally related to psychological problems.

Left Vocal Cord Paralysis, Lung Function and Exercise Capacity in Young Adults Born Extremely Preterm With a History of Neonatal Patent Ductus Arteriosus Surgery-A National Cohort Study.

Background: Left vocal cord paralysis (LVCP) is a known complication of patent ductus arteriosus (PDA) surgery in extremely preterm (EP) born neonates; however, consequences of LVCP beyond the first year of life are insufficiently described. Both voice problems and breathing difficulties during physical activity could be expected with an impaired laryngeal inlet. More knowledge may improve the follow-up of EP-born subjects who underwent PDA surgery and prevent confusion between LVCP and other diagnoses. Objectives: Examine the prevalence of LVCP in a nationwide cohort of adults born EP with a history of PDA surgery, and compare symptoms, lung function, and exercise capacity between groups with and without LVCP, and vs. controls born EP and at term. Methods: Adults born EP (<28 weeks' gestation or birth weight <1,000 g) in Norway during 1999-2000 who underwent neonatal PDA surgery and controls born EP and at term were invited to complete questionnaires mapping voice-and respiratory symptoms, and to perform spirometry and maximal treadmill exercise testing. In the PDA-surgery group, exercise tests were performed with a laryngoscope positioned to evaluate laryngeal function. Results: Thirty out of 48 (63%) eligible PDA-surgery subjects were examined at mean (standard deviation) age 19.4 (0.8) years, sixteen (53%) had LVCP. LVCP was associated with self-reported voice symptoms and laryngeal obstruction during exercise, not with lung function or peak oxygen consumption (VO2peak). In the PDA-surgery group, forced expiratory volume in 1 second z-score (z-FEV1) was reduced compared to EP-born controls (n = 30) and term-born controls (n = 36); mean (95% confidence interval) z-FEV1 was -1.8 (-2.3, -1.2), -0.7 (-1.1, -0.3) and -0.3 (-0.5, -0.0), respectively. For VO2peak, corresponding figures were 37.5 (34.9, 40.2), 38.1 (35.1, 41.1), and 43.6 (41.0, 46.5) ml/kg/min, respectively. Conclusions: LVCP was common in EP-born young adults who had undergone neonatal PDA surgery. Within the PDA-surgery group, LVCP was associated with self-reported voice symptoms and laryngeal obstruction during exercise, however we did not find an association with lung function or exercise capacity. Overall, the PDA-surgery group had reduced lung function compared to EP-born and term-born controls, whereas exercise capacity was similarly reduced for both the PDA-surgery and EP-born control groups when compared to term-born controls.

Voice and Exercise Related Respiratory Symptoms in Extremely Preterm Born Children After Neonatal Patent Ductus Arteriosus.

Objective: To investigate voice characteristics and exercise related respiratory symptoms in extremely preterm born 11-year-old children, focusing particularly on associations with management of a patent ductus arteriosus (PDA). Study design: Prospective follow-up of all children born in Norway during 1999-2000 at gestational age <28 weeks or with birthweight <1,000 g. Neonatal data were obtained prospectively on custom-made registration forms completed by neonatologists. Voice characteristics and exercise related respiratory symptoms were obtained at 11 years by parental questionnaires. Result: Questionnaires were returned for 228/372 (61%) eligible children, of whom 137 had no history of PDA. PDA had been noted in 91 participants, of whom 36 had been treated conservatively, 21 with indomethacin, and 34 with surgery. Compared to the children treated with indomethacin or conservatively, the odds ratio (95% confidence interval) for the surgically treated children were 3.4 (1.3; 9.2) for having breathing problems during exercise, 16.9 (2.0; 143.0) for having a hoarse voice, 4.7 (1.3; 16.7) for a voice that breaks when shouting, 4.6 (1.1; 19.1) for a voice that disturbs singing, and 3.7 (1.1; 12.3) for problems shouting or speaking loudly. The significance of surgery per se was uncertain since the duration of mechanical ventilation was associated with the same outcomes. Conclusion: Extremely preterm born children with a neonatal history of PDA surgery had more problems with voice and breathing during exercise in mid-childhood than those whose PDA had been handled otherwise. The study underlines the causal heterogeneity of exercise related respiratory symptoms in preterm born children.

Laryngeal Responses to Mechanically Assisted Cough in Progressing Amyotrophic Lateral Sclerosis.

BACKGROUND: Respiratory complications represent the major cause of death in amyotrophic lateral sclerosis (ALS). Noninvasive respiratory support is the mainstay therapy, but treatment becomes challenging as the disease progresses, possibly due to a malfunctioning larynx, which is the entrance to the airways. We studied laryngeal response patterns to mechanically assisted cough (mechanical insufflation-exsufflation) as ALS progresses. METHODS: This prospective longitudinal study of 13 consecutively included subjects with ALS were followed up during 2011-2016 with repeated tests of lung function, neurological status, and laryngeal responses to mechanical insufflation-exsufflation using video-recorded flexible transnasal fiberoptic laryngoscopy. RESULTS: Follow-up time was median 17 (range 6-59) months. In total, 751 laryngoscopy recordings from 67 individual examinations (median 4 per subject, range 2-11 per subject) were analyzed. Adverse laryngeal events that developed with disease progression during insufflation included adduction of true vocal folds in 8 of 9 spinal-onset subjects and adduction of aryepiglottic folds in all subjects, initially at the highest positive pressure and prior to onset of other bulbar symptoms in spinal-onset subjects. As cough became less expulsive with disease progression, laryngeal adduction occurred at lower insufflation pressures. Retroflex movement of the epiglottis was observed in 7 of 13 subjects regardless of insufflation pressures and independent of bulbar involvements. Backward movement of the tongue base occurred regardless of insufflation pressures in all but 1 subject. During exsufflation, constriction of the hypopharynx was observed in all subjects regardless of the presence of bulbar symptoms, after the adverse events that occurred during insufflation. CONCLUSIONS: Applying high insufflation pressures during mechanically assisted cough in ALS can become counterproductive as the disease progresses as well as prior to the onset of bulbar symptoms. The application of positive inspiratory pressures should be tailored to the individual patient, and laryngoscopy during ongoing treatment appears to be a feasible tool.

Ventilatory efficiency and aerobic capacity in people with multiple sclerosis: A randomized study.

OBJECTIVES: To assess ventilatory efficiency and aerobic capacity in people with multiple sclerosis and whether treadmill walking or progressive strength training has an effect on these parameters in this population. METHODS: In all, 24 adults with multiple sclerosis with an Expanded Disability Status Scale score of ≤6 completed a cardiopulmonary exercise test before and after 8 weeks of exercise. They were randomized to treadmill walking of low-to-moderate intensity (50%-70% of peak heart rate) or progressive strength training (six repetitions × two at 80% of one repetition maximum). Both groups exercised for 30 min three times per week. Primary outcome measure was ventilatory efficiency measured as the minute ventilation/carbon dioxide production (VE/VCO2) ratio and oxygen uptake efficiency slope. Secondary outcome was aerobic capacity, measured as peak oxygen uptake (VO2peak). RESULTS: Despite low aerobic capacity, ventilatory efficiency was found to be within normal range. After 8 weeks of exercise, no significant between-group differences emerged in (1) VE/VCO2 ratio (26 ± 2.2 to 26 ± 2.0, 29 ± 2.0 to 28 ± 2.3, p = 0.66), (2) oxygen uptake efficiency slope (2697 ± 442 to 2701 ± 577, 2473 ± 800 to 2481 ± 896, p = 0.71), or (3) VO2peak in mL/kg/min (28 ± 4.4 to 30 ± 4.3, 29 ± 6.7 to 29 ± 6.4, p = 0.38) in treadmill walking and progressive strength training, respectively. There were no significant within-group differences either. No adverse events occurred during cardiopulmonary exercise test or exercise training. CONCLUSION: In people with mild-to-moderate multiple sclerosis, 8 weeks of treadmill walking of low-to-moderate intensity or progressive strength training did not have any effect on ventilatory efficiency or aerobic capacity. Although aerobic capacity was lower than reference values, ventilatory efficiency was not reduced.

Higher Drop in Speed during a Repeated Sprint Test in Soccer Players Reporting Former Hamstring Strain Injury.

Aim: Hamstring strain injury is common in soccer. The aim of this study was to evaluate the physical capacity of players who have and have not suffered from hamstring strain injury in a sample of semi-professional and professional Norwegian soccer players in order to evaluate characteristics and to identify possible indications of insufficient rehabilitation. Method: Seventy-five semi-professional and professional soccer players (19 ± 3 years) playing at the second and third level in the Norwegian league participated in the study. All players answered a questionnaire, including one question about hamstring strain injury (yes/no) during the previous 2 years. They also performed a 40 m maximal sprint test, a repeated sprint test (8 × 20 m), a countermovement jump, a maximal oxygen consumption (VO2max) test, strength tests and flexibility tests. Independent sample t-tests were used to evaluate differences in the physical capacity of the players who had suffered from hamstring strain injury and those who had not. Mixed between-within subject's analyses of variance was used to compare changes in speed during the repeated sprint test between groups. Results: Players who reported hamstring strain injury during the previous two years (16%) had a significantly higher drop in speed (0.07 vs. 0.02 s, p = 0.007) during the repeated sprint test, compared to players reporting no previous hamstring strain injury. In addition, there was a significant interaction (groups × time) (F = 3.22, p = 0.002), showing that speed in the two groups changed differently during the repeated sprint test. There were no significant differences in relations to age, weight, height, body fat, linear speed, countermovement jump height, leg strength, VO2max, or hamstring flexibility between the groups. Conclusion: Soccer players who reported hamstring strain injury during the previous 2 years showed significant higher drop in speed during the repeated sprint test compared to players with no hamstring strain injury. The maximal speed, leg strength, ability to produce maximal power, endurance capacity, and hamstring flexibility was similar for both groups. Thus, a repeated sprint test consisting of 8 × 20 m could be used as a field-based diagnostic tool to identify players in need of reconditioning programs to ensure complete post-injury rehabilitation.

Adolescents who were born extremely preterm demonstrate modest decreases in exercise capacity.

AIM: We aimed to investigate exercise capacity in adolescents who were born extremely preterm and to study changes through puberty and associations with neonatal data, exercise habits and lung function. METHODS: This Norwegian population-based controlled cohort study focused on all infants (n = 35) born at a gestational age of ≤ 28 weeks or with a birthweight of ≤ 1000 grams in 1991-1992, together with matched term-born controls. Participants underwent spirometry and a maximal cardiopulmonary treadmill exercise test at 10 and 18 years. RESULTS: At 18 years of age, mean (95% confidence interval) peak oxygen consumption (peak VO2 ) was 42.3 (39.2-45.4) vs 45.3 (41.3-49.3) mL/kg/min in the preterm- and term-born groups, while the completed treadmill distance was 915 (837-992) vs 1017 (912-1122) metres. Peak VO2 was unrelated to neonatal factors and current lung function. Changes between 10 and 18 were similar in the two groups, and positive associations between exercise habits and peak VO2 developed during the period. CONCLUSION: Exercise capacity was modestly reduced in adolescents born extremely preterm, but the values were considered normal in most participants. Changes during puberty were similar to those observed for term controls, and the findings suggest similar trainability.

Adult respiratory outcomes of extreme preterm birth. A regional cohort study.

RATIONALE: Lifetime respiratory function after extremely preterm birth (gestational age≤28 wk or birth weight≤1,000 g) is unknown. OBJECTIVES: To compare changes from 18-25 years of age in respiratory health, lung function, and airway responsiveness in young adults born extremely prematurely to that of term-born control subjects. METHODS: Comprehensive lung function investigations and interviews were conducted in a population-based sample of 25-year-old subjects born extremely prematurely in western Norway in 1982-1985, and in matched term-born control subjects. Comparison was made to similar data collected at 18 years of age. MEASUREMENTS AND MAIN RESULTS: At 25 years of age, 46/51 (90%) eligible subjects born extremely prematurely and 39/46 (85%) control subjects participated. z-Scores for FEV1, forced expiratory flow at 25-75% of vital capacity, and FEV1/FVC were significantly reduced in subjects born extremely prematurely by 1.02, 1.26, and 0.88, respectively, and airway resistance (kPa/L/s) was increased (0.23 versus 0.18). Residual volume to total lung capacity increased with severity of neonatal bronchopulmonary dysplasia. Responsiveness to methacholine (dose-response slope; 3.16 versus 0.85) and bronchial lability index (7.5 versus 4.8%) were increased in subjects born extremely prematurely. Lung function changes from 18 to 25 years and respiratory symptoms were similar in the prematurely born and term-born groups. CONCLUSIONS: Lung function in early adult life was in the normal range in the majority of subjects born extremely prematurely, but methacholine responsiveness was more pronounced than in term-born young adults, suggesting a need for ongoing pulmonary monitoring in this population.

Exercise capacity after extremely preterm birth. Development from adolescence to adulthood.

RATIONALE: Extremely preterm (EP) birth is associated with a series of adverse health outcomes, some of which may be alleviated by improved physical fitness. However, EP-born subjects are reportedly less physically active than term-born peers. Exercise capacity is poorly described in this group, and longitudinal data are needed. OBJECTIVES: To compare exercise capacity of adults born EP and at term, and to address developmental patterns from adolescence to adulthood. METHODS: An area-based cohort of adults, born in 1982-1985 at gestational age 28 weeks or earlier, or with birth weight of 1,000 g or less, originally examined at 18 years of age, were re-examined at 25 years of age together with individually matched term-born control subjects, using an identical maximal cardiopulmonary treadmill exercise test and validated questionnaires. MEASUREMENTS AND MAIN RESULTS: A total of 34 (76%) eligible preterm and 33 (85%) term control subjects successfully completed the exercise test at age 25 years. In the two groups, average (95% confidence interval) peak oxygen consumption was 40.7 (37.9-43.5) and 44.2 (41.0-47.4) ml ⋅ kg(-1)⋅min(-1), respectively, whereas the distance completed on the treadmill was 910 (827-993) m and 1,020 (927-1,113) m. Peak oxygen consumption was unrelated to neonatal factors and current FEV1, but was positively associated with leisure-time physical activity and negatively associated with age at examination. Values obtained at age 18 and 25 years were strongly correlated and within normal range at both examinations. CONCLUSIONS: Exercise capacity was modestly reduced in EP-born adults; however, values were within a normal range, positively associated with self-reported physical activity and unrelated to neonatal factors and current airway obstruction.

Audiovisual assessment of exercise-induced laryngeal obstruction: reliability and validity of observations.

Variable obstruction to airflow at the laryngeal level may cause respiratory distress during exercise. The Continuous Laryngoscopy Exercise (CLE)-test enables direct visualization of the larynx during ongoing exercise. The aims of this study were to establish a scoring system for laryngeal obstruction as visualized during the CLE-test as well as to assess reliability and validity of this scoring system. Continuous video recording of the larynx was performed in parallel with continuous video recording of the upper part of the body, and recording of breath sounds in 80 patients and 20 symptom-negative volunteers, running on a treadmill to respiratory maximal tolerable distress or exhaustion. Each participant scored the degree of symptoms during exercise. The scoring system contains four sub-scores, each graded from 0 to 3. Two independent laryngologists, blinded to clinical data, scored the video recordings of the larynx twice. The proportion of inter- and intra-observer agreement (equal scores) for each sub-score through these four sessions varied between 70 and 100% (weighted kappa values varied from 0.49 to 1.00 correspondingly). A positive correlation was found between CLE-test sum score and symptom score (rho = 0.75, P < 0.001). There was a significant difference in CLE-test sum score between patients (3.34 +/- 1.34) and volunteers (0.65 +/- 0.66) (P < 0.001). The single CLE-test sub-score that correlated most strongly with symptom score was glottic adduction at maximal effort (rho = 0.75, P < 0.001). The presented scoring system is reliable and valid, and we suggest that it can be used when laryngeal function during exercise is evaluated.

Surgical treatment of exercise-induced laryngeal dysfunction.

A method for combined ergo-spirometry and continuous laryngeal inspection during exercise, entitled continuous laryngoscopy exercise test (CLE-test) has been developed in order to study airway obstruction at the laryngeal level during exercise. The aim of the study was to apply the CLE-test on patients experiencing respiratory distress during exercise in order to reveal the usefulness of the CLE-test both as a diagnostic tool in the selection of patients for surgery and in evaluation of treatment effects postoperatively. Until now, 81 patients with a history of exercise-induced stridor have undergone the CLE-test. Ten of these patients were selected for surgical treatment based on the severity of symptoms and their motivation for treatment. All ten patients underwent endoscopic supraglottoplasty (ES), with laser incision in both aryepiglottic folds anterior to the cuneiform cartilages and removal of the mucosa around the top of the tubercles. Each patient was examined by the CLE-test before and 3 months after surgery. Eight patients felt subjectively that their breathing capacity during exercise was improved. When pre- and postoperative ergo-spirometry evaluations were compared, increased peak oxygen consumption was observed in four out of ten patients and better maximal minute ventilation in seven out of ten. Postoperative evaluation of the laryngeal images showed less prominent aryepiglottic folds. The typical adduction of the supraglottic structures concomitant with inspiratory stridor found preoperatively was not present in any of the patients during exercise postoperatively. The ES procedure is an efficient surgical treatment for exercise-induced laryngeal supraglottic obstruction and the CLE-test eases the selection of patients for surgery and facilitates the evaluation of treatment effects.

Continuous laryngoscopy exercise test: a method for visualizing laryngeal dysfunction during exercise.

OBJECTIVES/HYPOTHESIS: To assess the diagnostic feasibility and patient acceptance of a new developed diagnostic test for exercise induced upper airway flow limitation. STUDY DESIGN: Clinical case control study including evaluation of contemporary ergo-spirometry and laryngoscopy continuously performed during exercise. METHODS: Twelve nonsymptomatic controls and four young females with documented dyspnea and noisy breathing during exercise were studied. All subjects exercised to exhaustion on a treadmill while attached to a fully equipped ergo-spirometry unit and a fiberoptic laryngoscope linked to a video camera and a sound recorder. RESULTS: The test situation was well tolerated. Two control subjects had a minor inspiratory synchronous medial motion of the aryepiglottic folds without limitation of laryngeal airflow. In the four symptomatic subjects, exercise induced inspiratory synchronous medial motion of the dorsal part of the aryepiglottic folds as well as vocal cord adduction and inspiratory stridor was demonstrated. CONCLUSION: The continuous laryngoscopy exercise test was easy to perform, well tolerated, and can be implemented in future diagnostic work-up programs of laryngeal dysfunction.

Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis.

BACKGROUND: Bactericidal-permeability-increasing protein (BPI) is a potent anti-microbial protein produced by neutrophil granulocytes. Anti-neutrophil cytoplasmatic antibodies (ANCA) directed against BPI have been detected in up to 91% in patients with cystic fibrosis (CF). We aimed to evaluate the prevalence of BPI-ANCA in our CF patients and to determine whether presence of BPI-ANCA is correlated with organ damage. METHODS: Twenty-four patients performed respiratory function testing and pulmonary high-resolution computed tomography (HRCT). HRCT was scored by using a modified Bhalla method. Serum samples were analysed by direct binding enzyme-linked immunosorbent assay for BPI-ANCA. RESULTS: The prevalence of anti-BPI-IgG was 71% and anti-BPI-IgA 33%. Twenty-nine percent of our patients were positive for both BPI-ANCA isotypes. Mean HRCT score was 8.0 ranging from 0 to 22, bronchiectasis presented the most common finding (79%). There was a significant correlation between BPI-ANCA and both HRCT score and FEV(1) (p < 0.01). High levels of BPI-ANCA were correlated to chronic Pseudomonas aeruginosa lung infection (p < 0.01). CONCLUSIONS: BPI-ANCA was common in our study group. Highly significant correlations between BPI-ANCA and parameters to evaluate lung disease in CF may be a consequence of the inflammation process, or it may indicate a pathogenic role of BPI-ANCA levels in the development of lung disease. More research is needed and the clinical significance of our findings needs further evaluation.

Magnetic resonance imaging of patients with increased blood pressure and altered blood pressure response to exercise after coarctation repair.

OBJECTIVE: Patients successfully operated for coarctation of the aorta are frequently subjected to altered blood pressure (BP) at rest and BP response during exercise. The relationship between these variables and blood flow, peak velocity, restenosis and other morphological features of the thoracic aorta as revealed by magnetic resonance imaging (MRI) was evaluated. DESIGN: Fifty-one patients subjected to coarctectomy of the aorta were examined by MRI. In addition, a control group of 23 healthy volunteers was evaluated. Morphology of the aorta was demonstrated with both ECG-triggered SE imaging and gadolinium-enhanced MR aortography. Flow-weighted MRI was applied for quantitative flow and velocity measurements. RESULTS: Structural alteration of the aorta was more commonly seen in those patients having increased BP at rest or altered BP response during exercise than those with a normal BP profile. The luminal diameter of the narrowest site of the aorta was decreased in all patient groups. Accordingly, the peak velocity at the corresponding site was significantly (p < 0.01) increased. However, blood flow was significantly (p < 0.01) decreased among those patients with normal BP profile compared with the other patient groups as well as the controls. CONCLUSION: Other structural changes than restenosis may contribute as well to the altered BP profile of patients subjected to coarctectomy. Reduced blood flow appears to correlate with normal BP profile, whereas the peak velocity measurements that are obtained by MRI are not able to differentiate between the patient groups. The comprehensive and reliable data obtained by non-invasive techniques, i.e. MRI and Doppler, may replace catheterization when deciding the need for intervention.