{"title":"A comparison of peak cough flow and peak expiratory flow in children with neuromuscular disorders","authors":"","doi":"10.1016/j.prrv.2024.04.001","DOIUrl":"10.1016/j.prrv.2024.04.001","url":null,"abstract":"<div><p><span><span>Spirometry and peak cough flow testing (PCF) are commonly used in the respiratory assessment of children with a </span>neuromuscular disorder (NMD). Testing uses two different machines, increases laboratory time, costs and resource utilisation. No studies have assessed the correlation between peak expiratory flow (PEF) obtained from spirometry and PCF in children with NMD using one device. An audit of children with a NMD managed at the Children’s Hospital at Westmead in 2022–2024 aged < 20 years who performed spirometry and PCF testing on the same device (Vyaire Body Box</span><sup>TM</sup>, Ultrasonic flow meter-based, or Vyaire Pneumotachograph<sup>TM</sup>, Pneumotach flow meter-based; Germany) was conducted to assess the correlation between PCF and PEF. Fifty-one sets of testing were identified, and 40 subjects (9F) had reproducible testing and were included. Median (range) age was 14.95 (7.20–19.00) years. Median PEF (L/min) was 4.05 (1.22–10.26) and median PCF (L/min) was 4.29 (1.69–10.82). PEF and PCF had a strong Pearson’s correlation coefficient, (R = 0.97, p = 0.03). The coefficient of determination was 0.93. If laboratory resources permit, spirometry should be the test of choice for children with NMD. On average, spirometry required multiple practices to achieve reproducibility to meet ATS/ERS standards. PCF testing can be utilised for children where performing technically acceptable spirometry is not possible.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"51 ","pages":"Pages 26-31"},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140577331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"How to deliver effective paediatric simulation based education","authors":"","doi":"10.1016/j.prrv.2024.05.001","DOIUrl":"10.1016/j.prrv.2024.05.001","url":null,"abstract":"<div><p>Simulation based education (SBE) is an educational tool increasingly used in the approach to the initial and ongoing education of healthcare professionals. Like all education tools, SBE needs to be used appropriately to achieve the desired outcomes. Using Cognitive Load Theory (CLT) in the instructional design of simulations is essential to maximise participant learning by reducing extraneous load and optimising intrinsic load. Educators can modify task fidelity, task complexity and instructional support to optimise learning. Specific methodologies can be used in program design such as rapid cycle deliberate practice, round the table teaching, low dose high frequency and flipped classroom. Fidelity and authenticity are important factors to consider when choosing design elements to ensure learner engagement, but not to overwhelm cognitive load. An integral part of SBE is the feedback or debriefing component. Several evidence-based methodologies can be employed to facilitate post simulation learning, including Debriefing with Good Judgement and PEARLS. Educators also need to consider faculty education and development, such as the discovery, growth and maturity model.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"51 ","pages":"Pages 10-18"},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054224000459/pdfft?md5=383550ee0f562b49b2974a43484a2a11&pid=1-s2.0-S1526054224000459-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141042823","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"“Antibiotic hypersensitivity reactions in Cystic Fibrosis: A thorough inspection on a stumbling block in patient care”","authors":"","doi":"10.1016/j.prrv.2024.01.004","DOIUrl":"10.1016/j.prrv.2024.01.004","url":null,"abstract":"<div><p>One hurdle in the management of CF<span>, a disease characterized by progressive endobronchial infection, is the presence of hypersensitivity reactions to antimicrobials due to prolonged and repetitive treatment courses. The aim of this review is to compile existing data and provide insight to medical professionals on a long-debated topic for optimum patient care. Clinical studies were inducted from the last 15 years and filtered based on their relativity to drug hypersensitivity<span><span> reactions (DHRs), antibiotics and CF. After completing the selection process, 10 clinical studies were thoroughly examined. The most frequent antibiotic<span> group related to DHRs were beta-lactams. Frequency of the most common overall type of reaction (immediate or nonimmediate) differed among clinical studies. Although severe reactions seem rare comparatively, they do occur during and even after completion of treatment regimens. The prevalence of true drug allergies should be confirmed using a variety of tests available, however, should not be confused with overall DHR rates. </span></span>Genetic mutations, gender and lifetime antibiotic dose were not related with an increased risk for DHR development. On the contrary, the most important factor according to most studies was the cumulative antimicrobial dose in a given period of time, especially when delivered parenterally. DHRs are an indisputable problem in the management of CF patients. Understanding possible risk factors and increased awareness is vital in both hospital and outpatient settings as early detection can decrease the severity of the reactions.</span></span></p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"51 ","pages":"Pages 56-65"},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139815878","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The impact of maternal asthma on the fetal lung: Outcomes, mechanisms and interventions","authors":"","doi":"10.1016/j.prrv.2023.12.004","DOIUrl":"10.1016/j.prrv.2023.12.004","url":null,"abstract":"<div><p>Maternal asthma affects up to 17% of pregnancies and is associated with adverse infant, childhood, and adult respiratory outcomes, including increased risks of neonatal respiratory distress syndrome, childhood wheeze and asthma. In addition to genetics, these poor outcomes are likely due to the mediating influence of maternal asthma on the <em>in-utero</em> environment, altering fetal lung and immune development and predisposing the offspring to later lung disease. Maternal asthma may impair glucocorticoid signalling in the fetus, a process critical for lung maturation, and increase fetal exposure to proinflammatory cytokines. Therefore, interventions to control maternal asthma, increase glucocorticoid signalling in the fetal lung, or Vitamin A, C, and D supplementation to improve alveologenesis and surfactant production may be beneficial for later lung function. This review highlights potential mechanisms underlying maternal asthma and offspring respiratory morbidities and describes how pregnancy interventions can promote optimal fetal lung development in babies of asthmatic mothers.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"51 ","pages":"Pages 38-45"},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054223000866/pdfft?md5=1add1cb172faa202e6b911055c66cf87&pid=1-s2.0-S1526054223000866-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139020502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Biochemical and genetic tools to predict the progression to Cystic Fibrosis in CRMS/CFSPID subjects: A systematic review","authors":"","doi":"10.1016/j.prrv.2024.01.001","DOIUrl":"10.1016/j.prrv.2024.01.001","url":null,"abstract":"<div><h3>Objectives</h3><p>Aim of this study was to identify risk factors for a progression to cystic fibrosis (CF) in individuals detected as CF Screening Positive, Inconclusive Diagnosis (CFSPID).</p></div><div><h3>Methods</h3><p>This is a systematic review through literature databases (2015–2023). Blood immunoreactive trypsinogen (b-IRT) values, <em>CFTR</em> genotype, sweat chloride (SC) values, isolation of <em>Pseudomonas aeruginosa</em> (Pa) from respiratory samples, Lung Clearance Index (LCI) values in CFSPIDs who converted to CF (CFSPID > CF) and age at CF transition were assessed.</p></div><div><h3>Results</h3><p>Percentage of CFSPID > CF varies from 5.3 % to 44 %. Presence of one CF-causing <em>CFTR</em> variant in trans with a variant with variable clinical consequences (VVCC), an initial SC ≥ 40 mmol/L, an increase of SC > 2.5 mmol/L/year and recurrent isolation of pseudomonas aeruginosa (Pa) from airway samples could allow identification of subjects at risk of progression to CF.</p></div><div><h3>Conclusions</h3><p>CFSPIDs with CF causing variant/VVCC genotype and first SC in the higher borderline range may require more frequent and prolonged clinical follow-up.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"51 ","pages":"Pages 46-55"},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054224000022/pdfft?md5=3444d1ed965aa205ceb61fe210d668de&pid=1-s2.0-S1526054224000022-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139498422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rebecca Dobra, Sarah Carroll, Jane C. Davies, Fiona Dowdall, Alistair Duff, Anna Elderton, Anna M. Georgiopoulos, Rachel Massey-Chase, Paul McNally, Michèle Puckey, Susan Madge
{"title":"Exploring the complexity of cystic fibrosis (CF) and psychosocial wellbeing in the 2020s: Current and future challenges","authors":"Rebecca Dobra, Sarah Carroll, Jane C. Davies, Fiona Dowdall, Alistair Duff, Anna Elderton, Anna M. Georgiopoulos, Rachel Massey-Chase, Paul McNally, Michèle Puckey, Susan Madge","doi":"10.1016/j.prrv.2024.08.001","DOIUrl":"https://doi.org/10.1016/j.prrv.2024.08.001","url":null,"abstract":"Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy .This decade has seen transformative change in management for many, but not all, people with CF. The most notable change comes from Cystic Fibrosis Transmembrane Receptor (CFTR) modulators, which bring significant benefits for people who are eligible for, and able to access, them . However alongside, or perhaps because of, this exciting progress, the past few years have also brought important novel challenges to the psychosocial wellbeing of people with CF.","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"62 1","pages":""},"PeriodicalIF":5.8,"publicationDate":"2024-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142224056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yoshua Selvadurai, Emily R Le Fevre, Jonathan Mervis, Dominic A Fitzgerald
{"title":"Hereditary haemorrhagic telangiectasia: A primer for the paediatrician.","authors":"Yoshua Selvadurai, Emily R Le Fevre, Jonathan Mervis, Dominic A Fitzgerald","doi":"10.1016/j.prrv.2024.07.003","DOIUrl":"https://doi.org/10.1016/j.prrv.2024.07.003","url":null,"abstract":"<p><p>Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant condition characterised by small telangiectasias and larger multisystem arteriovenous malformations (AVMs). Common sites of AVMs include in the nose, lungs, brain and liver. These lesions are prone to rupture, leading to complications including recurrent epistaxis and significant haemorrhage. Pulmonary hypertension (PH) can also occur. This review presents an update on the genetics, clinical manifestations, management options, and screening recommendations for children with HHT.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2024-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142110526","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Climate change and children's respiratory health.","authors":"Camille Bignier, Lucile Havet, Margot Brisoux, Céline Omeiche, Swati Misra, Apolline Gonsard, David Drummond","doi":"10.1016/j.prrv.2024.07.002","DOIUrl":"https://doi.org/10.1016/j.prrv.2024.07.002","url":null,"abstract":"<p><p>Climate change has significant consequences for children's respiratory health. Rising temperatures and extreme weather events increase children's exposure to allergens, mould, and air pollutants. Children are particularly vulnerable to these airborne particles due to their higher ventilation per unit of body weight, more frequent mouth breathing, and outdoor activities. Children with asthma and cystic fibrosis are at particularly high risk, with increased risks of exacerbation, but the effects of climate change could also be observed in the general population, with a risk of impaired lung development and growth. Mitigation measures, including reducing greenhouse gas emissions by healthcare professionals and healthcare systems, and adaptation measures, such as limiting outdoor activities during pollution peaks, are essential to preserve children's respiratory health. The mobilisation of society as a whole, including paediatricians, is crucial to limit the impact of climate change on children's respiratory health.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.7,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141897945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Olivia Jarrett , Soputhirith Seng , Dominic A. Fitzgerald
{"title":"Paediatric melioidosis","authors":"Olivia Jarrett , Soputhirith Seng , Dominic A. Fitzgerald","doi":"10.1016/j.prrv.2023.11.002","DOIUrl":"10.1016/j.prrv.2023.11.002","url":null,"abstract":"<div><p>Melioidosis is a tropical infectious disease caused by the saprophytic gram-negative bacterium <span><em>Burkholderia pseudomallei</em></span><span>. Despite the infection being endemic in southeast Asia and northern Australia, the broad clinical presentations and diagnostic difficulties limit its early detection, particularly in children. Melioidosis more commonly affects the immunocompromised and adults. Melioidosis is increasingly being diagnosed around the world and whole-genome sequencing indicates that these cases are not linked with travel to endemic areas. Research has concentrated on the adult population with limited experience reported in the care of this uncommon, but potentially fatal condition in children presenting with bacteraemia and pneumonia.</span></p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 31-37"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138516921","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Megha Sharma , Andrew W Brown , Nicholas M. Powell , Narasimhan Rajaram , Lauren Tong , Peter M. Mourani , Mario Schootman
{"title":"Racial and skin color mediated disparities in pulse oximetry in infants and young children","authors":"Megha Sharma , Andrew W Brown , Nicholas M. Powell , Narasimhan Rajaram , Lauren Tong , Peter M. Mourani , Mario Schootman","doi":"10.1016/j.prrv.2023.12.006","DOIUrl":"10.1016/j.prrv.2023.12.006","url":null,"abstract":"<div><p><span><span><span>Race-based and skin pigmentation-related inaccuracies in pulse oximetry have recently been highlighted in several large electronic health record-based </span>retrospective cohort studies across diverse patient populations and healthcare settings. Overestimation of oxygen saturation by pulse oximeters, particularly in hypoxic states, is disparately higher in Black compared to other racial groups. Compared to adult literature, pediatric studies are relatively few and mostly reliant on birth certificates or maternal race-based classification of comparison groups. Neonates, infants, and </span>young children<span><span> are particularly susceptible to the adverse life-long consequences of hypoxia and </span>hyperoxia<span>. Successful neonatal resuscitation, precise monitoring of preterm and term neonates with predominantly lung pathology, screening for </span></span></span>congenital heart defects<span><span>, and critical decisions on home oxygen, ventilator support and medication therapies, are only a few examples of situations that are highly reliant on the accuracy of pulse oximetry. Undetected hypoxia, especially if systematically different in certain racial groups may delay appropriate therapies and may further perpetuate </span>health care disparities<span>. The role of biological factors that may differ between racial groups, particularly skin pigmentation that may contribute to biased pulse oximeter readings needs further evaluation. Developmental and maturational changes in skin physiology and pigmentation, and its interaction with the operating principles of pulse oximetry need further study. Importantly, clinicians should recognize the limitations of pulse oximetry and use additional objective measures of oxygenation (like co-oximetry measured arterial oxygen saturation) where hypoxia is a concern.</span></span></p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 62-72"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139376187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}