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Background: Cystic fibrosis (CF) has been associated with impaired cardiovascular and endothelial function. CF transmembrane conductance regulator (CFTR) modulator therapy, most recently Elexacaftor/Tezacaftor/Ivacaftor (ETI), has led to improved CFTR function and life expectancy. However, the rising prevalence of obesity in adults is concerning. This study assessed the micro- and macrovascular endothelial function, cardiovascular disease (CVD) risk factors, and physical activity (PA) profiles in people with CF (pwCF) on ETI compared to healthy matched controls., Methods: In 15 pwCF and 15 age- and sex-matched controls, microvascular endothelial function (via transdermal delivery of insulin [INS] and acetylcholine [ACh] on the forearm), macrovascular endothelial function (via flow-mediated dilation [FMD] of the brachial artery), central haemodynamic parameters, including heart rate (HR), stroke volume index (SVi) and cardiac output index (Q̇I) (via thoracic impedance cardiography), body mass index (BMI), blood pressure (BP), and accelerometer-assessed PA were measured., Results: There were no differences in INS or FMD-mediated vasodilation between the groups (P > 0.05). However, a reduced vasodilatory response was evident in pwCF following ACh-mediated vasodilation (P = 0.01) and FMD normalised for shear rate (P = 0.03). No differences in resting HR, SVi, Q̇I, BP, BMI or PA were found (P > 0.05)., Conclusion: This study demonstrated reduced micro- and macrovascular function in pwCF. This dysfunction may have potential health implications, particularly regarding long-term cardiovascular risk and further longitudinal assessments are warranted., Competing Interests: Declaration of competing interests JL, GC and DSU have all served as principal investigators on Vertex-sponsored studies evaluating the use of ETI in patients with cystic fibrosis. GC has received speaker honoraria and research grant funding from Vertex Pharmaceuticals. DSU has received speaker honoraria from Vertex Pharmaceuticals., (Copyright © 2025 The Author(s). Published by Elsevier B.V. All rights reserved.)

Shared decision-making (SDM) is a collaborative approach to healthcare decision-making that involves patients and healthcare professionals working together to make decisions that are informed by the best available medical evidence, as well as the patient's values, preferences and goals. The importance of SDM and the intricate interplay among parents, children and young people (CYP), and healthcare professionals are increasingly acknowledged as the crucial aspects of delivering high-quality paediatric care. While there is a substantial evidence base for SDM improving knowledge and reducing decisional conflict, the evidence for long-term measures such as improved health outcomes is limited and mainly inconclusive. To support healthcare teams in implementing SDM, the authors offer a practical guide to enhance decision-making processes and empower CYP and their families., Competing Interests: Competing interests: All authors are part of the Ready Steady Go-TIER Collaborative. Information about the Collaborative can be found at www.readysteadygo.net., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Most UK hospitals discharge children after acute wheeze with advice to give regular salbutamol using a fixed dose weaning regime. We have introduced and evaluated the safety and efficacy of changing practice to using bronchodilators only as needed after 4 hourly assessments., Design: A multidisciplinary team of healthcare professionals worked with eight families of children who had needed hospital treatment with acute wheeze to develop guidance for the use of salbutamol on an as required basis after 4 hourly assessments. Data on salbutamol used with this approach were compared with a similar period in the previous year., Results: Data from 103 families showed a 73% reduction in salbutamol on day 1, 69% on day 2 and 50% on day 3 compared with what would have been used according to previous advice. Families found the advice easy to follow. There was a trend towards lower reattendance rates within 1 week compared with those recorded in the previous year. Those who had previously attended preferred this change in practice., Conclusions: These data suggest that with information to support the use of salbutamol on an as required basis after hospital attendance, children can be safely managed by their parents/guardians with much lower doses of salbutamol than those recommended in commonly used fixed dose weaning regimes., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Elexacaftor/Tezacaftor/Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator with the potential to improve exercise capacity. This case series of three adolescents with CF aimed to investigate whether 6 weeks treatment with Elexacaftor/Tezacaftor/Ivacaftor could improve exercise capacity in CFTR modulator naive adolescents with CF., Methods: Three adolescents (14.0 ± 1.4 years) with CF (FEV 1 % predicted: 62.5 ± 17.1; F508del/F508del genotype) completed an exhaustive maximal cardiopulmonary exercise test on a cycle ergometer to determine peak oxygen uptake ( V ̇ $\dot{{\rm{V}}}$ O 2peak ) and measure changes in gas exchange and ventilation during exercise at 6 weeks. We also analyzed wrist-worn device-based physical activity (PA) data in two of the three cases. Validated acceleration thresholds were used to quantify time spent in each PA intensity category., Results: Clinically meaningful improvements in V ̇ $\dot{{\rm{V}}}$ O 2peak were observed in all three cases (+17.6%, +52.4%, and +32.9%, respectively), with improvements greatest in those with more severe lung disease and lower fitness at baseline. Although lung function increased in all cases, inconsistent changes in markers of ventilatory and peripheral muscle efficiency likely suggest different mechanisms of improvement in this case group of adolescents with CF. Device-based analysis of PA was variable, with one case increasing and one case decreasing., Conclusion: In this case series, we have observed, for the first time, improvements in exercise capacity following 6 weeks of treatment with Elexacaftor/Tezacaftor/Ivacaftor. Improvements were greatest in the presence of more severe CF lung disease and lower aerobic fitness at baseline. The mechanism(s) responsible for these changes warrant further investigation in larger trials., (© 2022 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: A 25-hydroxyvitamin D (25OHD) may exert immunomodulatory effects on respiratory health, which may translate to improvements in exercise physiology. Thus, we aimed to investigate whether plasma 25OHD is associated with lung function and aerobic fitness in people with cystic fibrosis (pwCF)., Methods: A multicentre retrospective review of pwCF (> 9 years old) attending the Royal Hospital for Sick Children (Edinburgh) or Wessex CF-Unit (Southampton) was performed between July 2017 and October 2019. Demographic and clinical data were collected. Plasma 25OHD measured closest in time to clinical cardiopulmonary exercise testing and/or spirometry [forced expiratory volume (FEV 1 )% predicted] was recorded. Pancreatic insufficiency was diagnosed based on faecal elastase of < 100 µg g -1 . We performed multiple-regression analysis with aerobic fitness outcomes [peak oxygen uptake (VO 2 peak )] and FEV 1 % predicted as primary outcomes., Results: Ninety pwCF [mean ± SD age: 19.1 ± 8.6 years, 54 (60%) children, 48 (53%) males and 88 (98%) Caucasian] were included. 25OHD deficiency and insufficiency was 15 (17%) and 44 (49%), respectively. 25OHD deficiency and insufficiency was significantly associated with pancreatic insufficiency (χ 2  = 4.8, p = 0.02). Plasma 25OHD was not significantly associated with FEV 1 % predicted (r 2  = 0.06, p = 0.42, 95% CI = -0.09 to 0.19) or VO 2 peak (r 2  = 0.04, p = 0.07, 95% CI = -011 to 0.005) in all pwCF. However, 25OHD was significantly associated with both FEV 1 % (r 2  = 0.15, p = 0.02, 95% CI = 1.99-2.64) and VO 2 peak (r 2  = 0.13, p = 0.05, 95% CI = -0.26 to -0.005) in the paediatric cohort., Conclusions: We showed that 25OHD is associated with improved lung function and aerobic fitness in children and adolescents with CF. Mechanistic and high-quality prospective studies including both lung function and aerobic fitness as primary outcomes are now warranted., (© 2021 The British Dietetic Association Ltd.)

Competing Interests: Declaration of Competing Interest None of the authors have any conflicts of interest in relation to this submitted manuscript

Background: The development of cystic fibrosis (CF)-related diabetes (CFRD) in paediatric groups is associated with a reduced aerobic fitness. However, this has yet to be investigated in adults with more severe lung disease., Methods: Cardiopulmonary exercise and glycaemic control tests were retrospectively analysed in 46 adults with CF (age: 26.9 y [range: 16.3-66.5 y]; forced expiratory volume in 1s: 65.3% [range: 26.8-105.7%]; 26 males), diagnosed with CFRD (n = 19), impaired glucose tolerance (IGT; n = 8) or normal glucose tolerance (NGT; n = 19)., Results: Maximal oxygen uptake (V˙O 2max ) was reduced in adults with IGT and CFRD compared to their age- and gender-matched counterparts with NGT (p < 0.05); however, there was no difference when lung function was included as a covariate (all p > 0.05). V˙O 2max was greater in adults who experienced post-reactive hypoglycaemia vs. NGT without hypoglycaemia (p < 0.05). The frequency of ventilatory limitation (84%, 63% and 37%, respectively; p < 0.05) but not ventilation-perfusion mismatch (42%, 38% and 16%, respectively; p > 0.05), was greater with CFRD and IGT vs. NGT. There was also no difference in arterial oxygen saturation changes between groups (p > 0.05). Gender and body mass index were significant predictors of V˙O 2max (adjusted R 2  = 0.37, p < 0.01), but glycaemic control did not explain additional variance (p > 0.05)., Conclusions: Adults with CF-related dysglycaemia had a reduced V˙O 2max compared to age- and gender-matched counterparts, due to a greater degree of CF lung disease in these populations., Competing Interests: Declaration of Competing Interest None., (Crown Copyright © 2019. Published by Elsevier B.V. All rights reserved.)

Introduction: Oxidative stress may play an important role in the pathophysiology of cystic fibrosis (CF). This review aimed to quantify CF-related redox imbalances., Methods: Systematic searches of the Medline, CINAHL, CENTRAL and PsycINFO databases were conducted. Mean content of blood biomarkers from people with clinically-stable CF and non-CF controls were used to calculate the standardized mean difference (SMD) and 95% confidence intervals (95% CI)., Results: Forty-nine studies were eligible for this review including a total of 1792 people with CF and 1675 controls. Meta-analysis revealed that protein carbonyls (SMD: 1.13, 95% CI: 0.48 to 1.77), total F 2 -isoprostane 8-iso-prostaglandin F 2α (SMD: 0.64, 95% CI: 0.23 to 1.05) and malondialdehyde (SMD: 1.34, 95% CI: 0.30 to 2.39) were significantly higher, and vitamins A (SMD: -0.66, 95% CI -1.14 to -0.17) and E (SMD: -0.74, 95% CI: -1.28 to -0.20), β-carotene (SMD: -1.80, 95% CI: -2.92 to -0.67), lutein (SMD: -1.52, 95% CI: -1.83 to -1.20) and albumin (SMD: -0.98, 95% CI: -1.68 to -0.27) were significantly lower in the plasma or serum of people with CF versus controls., Conclusions: This systematic review and meta-analysis found good evidence for reduced antioxidant capacity and elevated oxidative stress in people with clinically-stable CF., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Background/objectives: Pancreatic exocrine insufficiency (PEI) is commonly caused by chronic pancreatitis (CP) or cystic fibrosis (CF). There are no PEI-specific patient-reported assessments of symptoms and impacts. The PEI Questionnaire (PEI-Q) was developed through qualitative research with PEI patients and expert clinical input. This study evaluated the psychometric properties of the PEI-Q., Methods: 162 PEI patients (CF = 71 and CP = 91), 62 diarrhoea-specific irritable bowel syndrome (IBS-D) patients and 60 healthy controls completed the 26-item PEI-Q and the Gastrointestinal Quality of Life Index (GIQLI) at baseline. PEI patients completed the measures again two weeks later to assess the test-retest reliability of the PEI-Q. Analyses supported item reduction and scoring algorithm development, followed by psychometric evaluation., Results: Over 90% of PEI patients completed at least 23 of the 26 items at baseline. Item responses and clinical relevance supported retention of 18 items. Factor analysis supported a three-factor solution (abdominal symptoms, bowel movements, impacts) with adequate model fit. PEI-Q scores had good internal consistency (Cronbach's alpha: 0.77-0.82) and test-retest reliability (ICC: 0.73-0.87). Correlations between PEI-Q and GIQLI supported convergent validity. Known-groups and receiver operating characteristic analyses demonstrated that PEI-Q scores discriminated (p < 0.001) between differing PEI severities, and PEI patients and controls., Conclusions: The PEI-Q has good validity and reliability. Results indicate that the PEI-Q could be used to aid identification and diagnosis of PEI, assist in the management of patients already diagnosed with PEI, ensuring correct and optimum treatment as well as enhance patient-clinician communication., (Copyright © 2018 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

The validity and safety of using supramaximal verification (S max ) to confirm a maximal effort during cardiopulmonary exercise testing (CPET) in people with cystic fibrosis (CF) and/or those with severe disease has been questioned. Therefore, this study aimed to investigate these concerns in children, adolescents, and adults with mild-to-severe CF lung disease. Retrospective analysis of 17 pediatric and 28 adult participants with CF [age range: 9.2-62.9 y; forced expiratory volume in 1 s: 66.7% (range: 29.9%-102.3%); 30 men] who completed a routine ramp-incremental cycling test to determine peak oxygen uptake (V̇o 2peak ) was studied. Maximal oxygen uptake (V̇o 2max ) was subsequently confirmed by S max at 110% of peak power output. All participants satisfied the criteria to verify a maximal effort during CPET. However, S max -V̇o 2peak exceeded ramp-V̇o 2peak in 3/14 (21.4%) of pediatric and 6/28 (21.4%) adult exercise tests. A valid measurement of V̇o 2max was attained in 85.7% of pediatric and 96.4% of adult exercise tests, as S max -V̇o 2peak did not exceed ramp-V̇o 2peak by >9%. Adults ( n = 9) experienced a ≥5% reduction in arterial O 2 saturation during CPET, 4 during both the ramp and S max , 3 during only the ramp, and 2 during only S max . S max did not significantly worsen perceived breathing effort, chest tightness, throat narrowing, or exertion compared with ramp-incremental testing. Given the clinical importance of aerobic fitness in people with CF, incorporating S max is recommended to provide a safe and valid measure of V̇o 2max in children, adolescents, and adults who span the spectrum of CF disease severity. NEW & NOTEWORTHY Incorporating supramaximal verification into cardiopulmonary exercise testing protocols did not increase the frequency of adverse events or perceived discomfort versus a single-phase incremental exercise test in people with mild-to-severe cystic fibrosis. Furthermore, a valid measure of maximal oxygen uptake (V̇o 2max ) was obtained from 85.7% of pediatric and 96.4% of adult exercise tests, whereas peak oxygen uptake underestimated aerobic fitness in comparison with V̇o 2max in 21.4% of cases (by up to 24.4%).

Background: Pancreatic exocrine insufficiency (PEI) affects patients with chronic pancreatitis (CP) and cystic fibrosis (CF) who produce insufficient digestive pancreatic enzymes. Common symptoms include steatorrhoea, diarrhea, and abdominal pain., Objective: The objective of the study was to develop and test the content validity of a patient-reported outcome (PRO) instrument assessing PEI symptoms and their impact on health-related quality of life., Methods: Instrument development was supported by a literature review, expert physician interviews (n = 10: Germany 4, UK 3, France 3), and exploratory, qualitative, concept-elicitation interviews with patients with CF and CP with PEI (n = 61: UK 29, Germany 18, France 14) and expert physicians (n = 10). Cognitive debriefing of the draft instrument was then performed with patients with PEI (n = 37: UK 24, Germany 8, France 5), and feasibility was assessed with physicians (n = 3). For all interviews, verbatim transcripts were qualitatively analysed using thematic analysis methods and Atlas.ti computerized qualitative software. All themes were data driven rather than a priori., Results: Patient interviews elicited symptoms and impacts not reported in the literature. Six symptom concepts emerged: pain, bloating, bowel symptoms, nausea/vomiting, eating problems, and tiredness/fatigue. Six impact domains were also identified. A 45-item instrument was developed in English, French, and German for testing in cognitive debriefing patient interviews. Following cognitive debriefing, 18 items were deleted., Conclusion: Rigorous qualitative patient research and expert clinical input supported development of a PEI-specific PRO with the potential to aid management and monitoring of unmet needs among patients with PEI. The next step is to perform psychometric evaluation of the resulting instrument.

Despite aggressive antibiotic therapy, bronchopulmonary colonization by Pseudomonas aeruginosa causes persistent morbidity and mortality in cystic fibrosis (CF). Chronic P. aeruginosa infection in the CF lung is associated with structured, antibiotic-tolerant bacterial aggregates known as biofilms. We have demonstrated the effects of non-bactericidal, low-dose nitric oxide (NO), a signaling molecule that induces biofilm dispersal, as a novel adjunctive therapy for P. aeruginosa biofilm infection in CF in an ex vivo model and a proof-of-concept double-blind clinical trial. Submicromolar NO concentrations alone caused disruption of biofilms within ex vivo CF sputum and a statistically significant decrease in ex vivo biofilm tolerance to tobramycin and tobramycin combined with ceftazidime. In the 12-patient randomized clinical trial, 10 ppm NO inhalation caused significant reduction in P. aeruginosa biofilm aggregates compared with placebo across 7 days of treatment. Our results suggest a benefit of using low-dose NO as adjunctive therapy to enhance the efficacy of antibiotics used to treat acute P. aeruginosa exacerbations in CF. Strategies to induce the disruption of biofilms have the potential to overcome biofilm-associated antibiotic tolerance in CF and other biofilm-related diseases., (Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.)

Pseudomonas aeruginosa is a remarkably versatile environmental bacterium with an extraordinary capacity to infect the cystic fibrosis (CF) lung. Infection with P. aeruginosa occurs early, and although eradication can be achieved following early detection, chronic infection occurs in over 60% of adults with CF. Chronic infection is associated with accelerated disease progression and increased mortality. Extensive research has revealed complex mechanisms by which P. aeruginosa adapts to and persists within the CF airway. Yet knowledge gaps remain, and prevention and treatment strategies are limited by the lack of sensitive detection methods and by a narrow armoury of antibiotics. Further developments in this field are urgently needed in order to improve morbidity and mortality in people with CF. Here, we summarize current knowledge of pathophysiological mechanisms underlying P. aeruginosa infection in CF. Established treatments are discussed, and an overview is offered of novel detection methods and therapeutic strategies in development.

Introduction: Inhaled corticosteroid therapy (ICS) for asthma is currently modified according to symptoms and lung function. Fractional exhaled nitric oxide (FENO) has been demonstrated to be a non-invasive marker of eosinophilic inflammation. Studies of FENO-driven asthma management show variable success. Objectives: This study aimed to evaluate whether monitoring FENO can improve outpatient management of children with moderate to severe asthma using a pragmatic design., Methods: Children aged 6–17 years with moderate to severe asthma were recruited. Their asthma was stabilised before randomisation to FENO-driven therapy or to a standard management group where therapy was driven by conventional markers of asthma control. ICS or long-acting bronchodilator therapies were altered according to FENO levels in combination with reported symptoms in the FENO group. Participants were assessed 2 monthly for 12 months. ICS dose and exacerbation frequency change were compared between groups in an intention to treat analysis., Results: Ninety children were randomised. No difference was found between the two groups in either change in corticosteroid dose or exacerbation frequency. Results were similar in a planned secondary analysis of atopic asthmatics., Conclusion: FENO-guided ICS titration does not appear to reduce corticosteroid usage or exacerbation frequency in paediatric outpatients with moderate to severe asthma. This may reflect limitations in FENO-driven management algorithms, as there are now concerns that FENO levels relate to atopy as much as they relate to asthma control., (© 2012 Blackwell Publishing Ltd.)

Persistent wheezing, poorly responsive to bronchodilator therapy, raises concerns about the progression of cystic fibrosis-related lung disease. We describe 3 infants with such symptoms who were observed to have primary tracheomalacia. The diagnoses were made using flexible bronchoscopy during spontaneous respiration. Early recognition of this etiology can limit unnecessary investigation and the overuse of empirical treatments such as oral and inhaled corticosteroids.

Intestinal obstruction and dysmotility occur throughout life in cystic fibrosis but rarely present as an acute obstruction beyond the neonatal period. We describe the previously unreported occurrence of acute obstruction of the sigmoid colon as a presenting feature of cystic fibrosis (CF) in a 6-month infant.

Objective: Newborn screening for cystic fibrosis remains controversial because improved pulmonary function has not been established. Studies to date have not accounted for differences in treatments delivered to clinically diagnosed children and newborn-screened controls. Here, we compare outcomes and treatment of patients clinically diagnosed within the newborn-screening reporting window (early-clinically diagnosed), those presenting after this period (late-clinically diagnosed), and patients diagnosed by newborn screening., Patients and Methods: In a cross-sectional analysis of cohorts retrospectively ascertained, patients who were homozygous deltaF508 with cystic fibrosis, attending specialist cystic fibrosis centers, and 1 to 10 years of age between 2000 and 2002 were identified from the United Kingdom Cystic Fibrosis Database and stratified into newborn-screened, early-clinically diagnosed, or late-clinically diagnosed cohorts. Two analyses were performed: (1) after restricting to the most recent year of data collection, early-clinically diagnosed and late-clinically diagnosed cohorts were matched to newborn-screened patients by patient age and year of data collection (133 patients per cohort were identified); and (2) for all years of data collection, annual sets of data for early-clinically diagnosed and late-clinically diagnosed patients were matched to newborn-screened patients by patient age and year of data collection (291 data sets per cohort were identified). Median height and weight z scores, proportion of patients with height and weight <10th percentile, prevalence of chronic Pseudomonas aeruginosa infection, Shwachman-Kulczyki morbidity scores, percent predicted forced expiratory volume in 1 second, and numbers of long-term therapies were compared., Results: In both analyses, newborn screening was associated with higher height z score, higher Shwachman-Kulczyki score, lower likelihood of height <10th percentile, and fewer long-term therapies compared with late-clinically diagnosed patients. No other differences were found., Conclusions: Newborn screening was associated with improved growth, reduced morbidity, and reduced therapy, yet generated equivalent pulmonary outcome compared with late clinical diagnosis, suggesting that newborn screening may slow cystic fibrosis lung disease progression.

Always ask about hoarseness and quality of voice in a history of any child presenting with cough or asthma-like symptoms. Children presenting with what appears to be an acute onset of hoarseness, without any physical signs of airways obstruction, should be reviewed after two weeks. If there is chronic hoarseness, referral to an ENT specialist should be considered with a view to laryngoscopy. If the child develops clinical signs of acute airway obstruction such as stridor or respiratory distress, prompt paediatric review is indicated. When referring, it is important to emphasise whether or not there is chronic hoarseness in order to differentiate the diagnosis from croup. Juvenile Laryngeal Papillomatosis may present with cough, pneumonia, dysphagia, or stridor, as well as hoarseness. These patients are often misdiagnosed as having asthma or allergies.

A 16-year-old male with cystic fibrosis (CF) was admitted to hospital with a severe infective exacerbation. Despite standard management, including conventionally selected intravenous antibiotics for Pseudomonas aeruginosa, chest physiotherapy, and institution of noninvasive ventilation (NIV) for progressive hypercapneic respiratory failure, he continued to deteriorate. Direct sputum sensitivity testing (DSST) revealed a novel combination of antibiotics that resulted in a rapid and remarkable clinical improvement. DSST is a form of "whole" sputum sensitivity testing that provides information on antibiotic synergy, and may more accurately reflect in vivo antibiotic sensitivity patterns in cystic fibrosis., (Copyright 2003 Wiley-Liss, Inc.)

A sister (aged 6 years) and brother (aged 8 years) presented four months apart with severe molluscum contagiosum. Both children demonstrated clinical and laboratory evidence of combined immunodeficiency. The extent of skin involvement by molluscum contagiosum precluded conventional treatment as well as intralesional interferon alpha (IFN alpha). Both subjects responded well to subcutaneous IFN alpha.

Background: A small proportion of cystic fibrosis patients develop severe localised bronchiectasis. When this persists despite maximal medical therapy it presents a difficult management problem. Lung transplantation cannot be justified. We report encouraging results in six patients with severe localised bronchiectasis and cystic fibrosis who underwent pulmonary resection., Methods: Each child had severe localised bronchiectasis despite maximal medical therapy. Intensive preoperative toilet was instituted and pulmonary resection undertaken when lung function was optimal., Results: There was a marked improvement in symptoms in every case. No significant long-standing morbidity was associated with the resection. There was no significant decrease in pulmonary function following resection., Conclusion: Pulmonary resection should be considered in the management of severe localised bronchiectasis unresponsive to maximal medical therapy in cystic fibrosis patients.

Pulse oximetry is increasingly used to assess hypoxaemia in respiratory illnesses. Six children presenting with acute asthma and prolonged falls in oxygen saturation values after treatment with salbutamol are described who were subsequently shown to have pneumonic consolidation on chest radiography.

Thirty two preschool children were entered into a double blind, placebo controlled study using intermittent budesonide to treat viral induced wheeze. Active treatment was either 800 micrograms twice a day via a spacer or 1600 micrograms twice a day via a spacer and facemask in those children too young to use a mouthpiece. Treatment was started at the onset of an upper respiratory tract infection and continued for seven days or until symptoms had resolved for 24 hours. Each child remained in the study until they had completed using one pair of budesonide and placebo inhalers in random order without the need for additional oral prednisolone. Twenty five children completed 28 treatment pairs. All 25 families were asked to express a preference after completing their first treatment pair: 12 preferred budesonide and six preferred placebo; seven had no preference. Symptom scores were compared in 17 treatment pairs that were completed without the need for oral prednisolone. Mean day and night time wheeze in the first week after infection were significantly lower in those receiving budesonide. Intermittent inhalation of budesonide can modify the severity of wheezing in preschool children developing asthma after viral respiratory infections but improvements were modest with the doses used in this study.