Distorted body image and anorexia complicating cystic fibrosis in an adolescent

AbstractA 15 year old girl with cystic fibrosis has been dieting and losing weight for 2 years. Despite being underweight she aims to lose a further 6 kg to become a “size zero”. Her falling weight has been associated with deteriorations in her general health and lung function, which is exacerbated by poor compliance. The situation has been complicated further by her becoming pregnant

Is hypnotherapy an acceptable treatment option for children with habit cough?

Habit cough is a chronic, persistent dry cough which occurs in children only when awake. It is considered functional (non-organic) and can have a significant impact on the quality of life of the child and their family. One possible treatment option for habit cough is hypnotherapy. At our centre we offered hypnotherapy sessions to patients diagnosed with habit cough, and conducted telephone interviews with patients' parents to determine the acceptability of this therapy. Nine patients' parents were interviewed, and despite being unsure of what to expect with hypnotherapy, all nine found it an acceptable treatment option. Parents reported that hypnotherapy appeared to result in cough reduction or cessation in 6 out of 9 cases

Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis

BACKGROUND: Cystic fibrosis is the most common life-limiting autosomal recessive genetic disorder in white populations. Distal intestinal obstruction syndrome (DIOS) is an important morbidity in cystic fibrosis. It is the result of the accumulation of viscid faecal material within the bowel which combines with thick, sticky mucus produced in the intestines of people with cystic fibrosis. The intestine may be completely blocked (complete DIOS) or only partially blocked (incomplete DIOS). Once a diagnosis of DIOS has been made, the goal of therapy is to relieve the acute complete or incomplete faecal obstruction and ultimately prevent the need for surgical intervention. OBJECTIVES: This review aimed to evaluate the effectiveness and safety of different treatment regimens for the treatment of DIOS (complete and incomplete) in children and adults with cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of search: 09 September 2021. We also searched online trial registries. Date of last search: 12 October 2021. SELECTION CRITERIA: Randomised controlled trials, quasi-randomised controlled trials (including cross-over trials (to be judged on an individual basis)) comparing the use of laxative agents or surgery for treating DIOS in children, young people and adults with cystic fibrosis to each other, placebo or no intervention. DATA COLLECTION AND ANALYSIS: Two authors independently screened papers, extracted trial details and assessed for risk of bias. The authors assessed the quality of evidence using GRADE. MAIN RESULTS: There was one trial with 20 participants (16 females) included in the review. The mean age of participants was 13.1 years. The trial was a double-blinded, randomised cross-over trial which had a duration of 12 months in total and compared high-dose and low-dose pancreatic enzyme therapy. As only the abstract of the trial was available, the overall risk of bias was judged to be unclear. The trial did not address either of our primary outcomes (time until resolution of DIOS and treatment failure rate), but reported episodes of acute DIOS, presence of abdominal mass and abdominal pain. There were no numerical data available for these outcomes, but the authors stated that there was no difference between treatment with high-dose or low-dose pancreatic enzymes. The overall certainty of the evidence was found to be very low. AUTHORS' CONCLUSIONS: There is a clear lack of evidence for the treatment of DIOS in people with cystic fibrosis. The included abstract did not address our primary outcome measures and did not provide numerical data for the two secondary outcomes it did address. Therefore, we cannot justify the use of high-dose pancreatic enzymes for treating DIOS, nor can we comment on the efficacy and safety of other laxative agents. From our findings, it is clear that more randomised controlled trials need to be conducted in this area

ALPINE2: Efficacy and safety of 14-day vs 28-day inhaled aztreonam for Pa eradication in children with cystic fibrosis

Cystic fibrosis; Paediatric; Pseudomonas aeruginosaFibrosi quística; Pediàtric; Pseudomonas aeruginosaFibrosis quística; Pediátrico; Pseudomonas aeruginosaBackground: Antibiotic eradication therapies recommended for newly isolated Pseudomonas aeruginosa (Pa) in people with cystic fibrosis (pwCF) can be burdensome. ALPINE2 compared the efficacy and safety of a shortened 14-day course of aztreonam for inhalation solution (AZLI) with 28-day AZLI in paediatric pwCF. Methods: ALPINE2 (a double-blind, phase 3b study) included children aged 3 months to <18 years with CF and new-onset Pa infection. Participants were randomized to receive 75 mg AZLI three times daily for either 28 or 14 days followed by 14 days' matched placebo. The primary endpoint was rate of primary Pa eradication (no Pa detected during the 4 weeks post AZLI treatment). Non-inferiority was achieved if the lower 95% CI bound of the treatment difference between the two arms was above -20%. Secondary endpoints included assessments of Pa recurrence during 108 weeks of follow-up after primary eradication. Safety endpoints included treatment-emergent adverse events (TEAEs). Results: In total, 149 participants were randomized (14-day AZLI, n = 74; 28-day AZLI, n = 75) and 142 (95.3%) completed treatment. Median age: 6.0 years (range: 0.3-17.0). Baseline characteristics were similar between treatment arms. Primary Pa eradication rates: 14-day AZLI, 55.9%; 28-day AZLI, 63.4%; treatment difference (CI), -8.0% (-24.6, 8.6%). Pa recurrence rates at follow-up end: 14-day AZLI, 54.1% (n = 20/37); 28-day AZLI, 41.9% (n = 18/43). TEAEs were similar between treatment arms. No new safety signals were observed. Conclusions: Non-inferiority of 14-day AZLI versus 28-day AZLI was not demonstrated. Both courses were well tolerated, further supporting AZLI short-term safety in paediatric and adolescent pwCF. Clinicaltrials: GOV: NCT03219164.This ALPINE2 study was sponsored by Gilead Sciences, Inc. Gilead Sciences, Inc. were responsible for study design, data collection and analysis, writing the study report and making the decision to submit the article for publication. Medical writing support for the preparation of this manuscript was provided by Elvi Nimali, MBiochem, of PharmaGenesis London, London, UK, and funded by Gilead Sciences, Inc

The ERS approach to e-cigarettes is entirely rational

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A review of prednisolone prescribing for children with acute asthma in the UK

Abstract: Introduction: Worldwide asthma guidelines recommend short courses of oral prednisolone in children with acute exacerbations generating high prescription numbers. There is a paucity of evidence to inform the optimal dose and course duration. This has led to a variation in the recommendations for prednisolone prescribing. Our objective was to assess prednisolone prescribing practise for children with acute asthma in a representative sample of UK prescribers. Methods: We developed an online questionnaire asking prescribers the prednisolone dosage, course duration and formulation used, whether they discussed oral prednisolone side effects with the family and at what child's age they changed from prescribing soluble to non-soluble formulations. This was sent to 1006 UK prescribers including Paediatric Respiratory Consultants, doctors in training, asthma nurses and General Practitioners. Results: 200 complete responses were received (response rate 20%). The majority of surveyed prescribers follow the British National Formulary for Children recommendations on dosage rather than those included in the British Thoracic Society and the Scottish Intercollegiate Guidelines Network. Despite this, we highlighted a 4-fold variation in prednisolone dosages for acute asthma. The majority of prescribers chose 3 days as the course duration. High use of soluble formulations was highlighted. Conclusions: There is wide variation in the dose of prednisolone prescribed for children with acute asthma in the UK. This reflects a relative lack of evidence that needs addressing

Tidal breathing parameters measured by structured light plethysmography in children aged 2-12 years recovering from acute asthma/wheeze compared with healthy children

© 2018 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.Peer reviewedPublisher PD

Exhaled breath hydrogen cyanide as a marker of early Pseudomonas aeruginosa infection in children with cystic fibrosis

Hydrogen cyanide is readily detected in the headspace above Pseudomonas aeruginosa cultures and in the breath of cystic fibrosis (CF) patients with chronic (P. aeruginosa) infection. We investigated if exhaled breath HCN is an early marker of P. aeruginosa infection. 233 children with CF who were free from P. aeruginosa infection were followed for 2 years. Their median (interquartile range) age was 8.0 (5.0–12.2) years. At each study visit, an exhaled breath sample was collected for hydrogen cyanide analysis. In total, 2055 breath samples were analysed. At the end of the study, the hydrogen cyanide concentrations were compared to the results of routine microbiology surveillance. P. aeruginosa was isolated from 71 children during the study with an incidence (95% CI) of 0.19 (0.15–0.23) cases per patient-year. Using a random-effects logistic model, the estimated odds ratio (95% CI) was 3.1 (2.6–3.6), which showed that for a 1- ppbv increase in exhaled breath hydrogen cyanide, we expected a 212% increase in the odds of P. aeruginosa infection. The sensitivity and specificity were estimated at 33% and 99%, respectively. Exhaled breath hydrogen cyanide is a specific biomarker of new P. aeruginosa infection in children with CF. Its low sensitivity means that at present, hydrogen cyanide cannot be used as a screening test for this infection

ALPINE2:Efficacy and safety of 14-day vs 28-day inhaled aztreonam for <i>Pa </i>eradication in children with cystic fibrosis

Background: Antibiotic eradication therapies recommended for newly isolated Pseudomonas aeruginosa (Pa) in people with cystic fibrosis (pwCF) can be burdensome. ALPINE2 compared the efficacy and safety of a shortened 14-day course of aztreonam for inhalation solution (AZLI) with 28-day AZLI in paediatric pwCF. Methods: ALPINE2 (a double-blind, phase 3b study) included children aged 3 months to &lt;18 years with CF and new-onset Pa infection. Participants were randomized to receive 75 mg AZLI three times daily for either 28 or 14 days followed by 14 days' matched placebo. The primary endpoint was rate of primary Pa eradication (no Pa detected during the 4 weeks post AZLI treatment). Non-inferiority was achieved if the lower 95% CI bound of the treatment difference between the two arms was above −20%. Secondary endpoints included assessments of Pa recurrence during 108 weeks of follow-up after primary eradication. Safety endpoints included treatment-emergent adverse events (TEAEs). Results: In total, 149 participants were randomized (14-day AZLI, n = 74; 28-day AZLI, n = 75) and 142 (95.3%) completed treatment. Median age: 6.0 years (range: 0.3–17.0). Baseline characteristics were similar between treatment arms. Primary Pa eradication rates: 14-day AZLI, 55.9%; 28-day AZLI, 63.4%; treatment difference (CI), −8.0% (−24.6, 8.6%). Pa recurrence rates at follow-up end: 14-day AZLI, 54.1% (n = 20/37); 28-day AZLI, 41.9% (n = 18/43). TEAEs were similar between treatment arms. No new safety signals were observed. Conclusions: Non-inferiority of 14-day AZLI versus 28-day AZLI was not demonstrated. Both courses were well tolerated, further supporting AZLI short-term safety in paediatric and adolescent pwCF. ClinicalTrials.gov:</p