Paediatric Respiratory Reviews最新文献

{"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":"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":"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 &gt; CF) and age at CF transition were assessed.</p></div><div><h3>Results</h3><p>Percentage of CFSPID &gt; 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 &gt; 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}

{"title":"Exploring the complexity of cystic fibrosis (CF) and psychosocial wellbeing in the 2020s: Current and future challenges","authors":"Rebecca Dobra ,&nbsp;Sarah Carroll ,&nbsp;Jane C. Davies ,&nbsp;Fiona Dowdall ,&nbsp;Alistair Duff ,&nbsp;Anna Elderton ,&nbsp;Anna M. Georgiopoulos ,&nbsp;Rachel Massey-Chase ,&nbsp;Paul McNally ,&nbsp;Michèle Puckey ,&nbsp;Susan Madge","doi":"10.1016/j.prrv.2024.08.001","DOIUrl":"10.1016/j.prrv.2024.08.001","url":null,"abstract":"<div><div>Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy <span><span>[1]</span></span>, <span><span>[2]</span></span>, <span><span>[3]</span></span>, <span><span>[4]</span></span>, <span><span>[5]</span></span>, <span><span>[6]</span></span>, <span><span>[7]</span></span>, <span><span>[8]</span></span>, <span><span>[9]</span></span>.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 <span><span>[10]</span></span>. 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.</div><div>This article, written as a collaboration between CF psychologists, social workers, physicians and nurses aims to provide an accessible overview of the novel psychosocial challenges now faced by children, their families, and adults with CF, and to invite consideration of their changing psychosocial requirements to inform future holistic care. Themes include geopolitical stressors such as the pandemic and its wake, a growing divide between those able or unable to access CFTR modulators, potential rapid changes in life expectancy secondary to these drugs and the inevitable associated challenges this brings; evolving body image, mental health side effects of CFTR modulators, the challenges of adherence in apparently well children and young adults, as well as the diagnostic conundrum and associated anxiety of the cystic fibrosis screen positive inconclusive diagnosis (CFSPID) label. It also highlights some unmet research and service delivery needs in the area.</div></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"54 ","pages":"Pages 35-42"},"PeriodicalIF":4.7,"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}

{"title":"Paediatric melioidosis","authors":"Olivia Jarrett ,&nbsp;Soputhirith Seng ,&nbsp;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}

{"title":"Racial and skin color mediated disparities in pulse oximetry in infants and young children","authors":"Megha Sharma ,&nbsp;Andrew W Brown ,&nbsp;Nicholas M. Powell ,&nbsp;Narasimhan Rajaram ,&nbsp;Lauren Tong ,&nbsp;Peter M. Mourani ,&nbsp;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}

{"title":"Cystic fibrosis liver disease in the new era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators","authors":"Jessica A. Eldredge ,&nbsp;Mark R. Oliver ,&nbsp;Chee Y. Ooi","doi":"10.1016/j.prrv.2023.12.005","DOIUrl":"10.1016/j.prrv.2023.12.005","url":null,"abstract":"<div><h3>Summary</h3><p>Cystic fibrosis liver disease (CFLD) is characterised by a wide heterogenity of manifestations and severity. It represents a major cause of morbidity in people with cystic fibrosis (PwCF), which will be of increasing relevance as survival increases in the new era of cystic fibrosis care. No medical therapy currently available has evidence to treat or prevent progression of liver disease. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators may be transformative on pulmonary, nutritional and quality of life, but direct effect on long term liver disease outcomes is not yet established. Drug-associated hepatic adverse effects may be common, and clinician familiarity with drug-monitoring recommendations is essential. Longitudinal studies are required to understand the effect of CFTR modulators on the incidence and natural history of CFLD, including with early treatment initiation, in established advanced liver disease, and post liver transplantation.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 54-61"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054223000878/pdfft?md5=a103ef97f990bc20ce0578c92a341733&pid=1-s2.0-S1526054223000878-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139071226","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":"Neurodevelopmental outcomes of extremely preterm infants with bronchopulmonary dysplasia (BPD) – A retrospective cohort study","authors":"Khoa L. Nguyen ,&nbsp;Dominic A. Fitzgerald ,&nbsp;Annabel Webb ,&nbsp;Barbara Bajuk ,&nbsp;Himanshu Popat","doi":"10.1016/j.prrv.2024.02.004","DOIUrl":"10.1016/j.prrv.2024.02.004","url":null,"abstract":"<div><h3>Objective</h3><p>To investigate the neurodevelopmental outcomes for preterm infants born &lt; 29 weeks gestation with/without bronchopulmonary dysplasia (BPD).</p></div><div><h3>Study design</h3><p>Preterm infants &lt; 29 weeks’ gestation born 2007–2018 in New South Wales and the Australian Capital Territory, Australia, were included. Infants who died &lt; 36 weeks’ postmenstrual age and those with major congenital anomalies were excluded. Subjects were assessed at 18–42 months corrected age using the Bayley Scales of Infant Development, 3rd edition.</p></div><div><h3>Results</h3><p>1436 infants without BPD (non-BPD) and 1189 infants with BPD were followed. The BPD group, 69 % infants were discharged without respiratory support (BPD1), 29 % on oxygen (BPD2) and 2 % on pressure support/tracheostomy (BPD3). Moderate neurodevelopmental impairment (NDI) was evident in 5.7 % of non-BPD infants, 11 % BPD1, 15 % BPD2, 15 % BPD3 infants. Severe NDI was seen in 1.7 % non-BPD infants, 3.4 % BPD1, 7.3 % BPD2, 35 % BPD3 infants. After adjusting for confounders, infants with BPD2 (OR 2.24, 99.9 % CI 1.25 to 5.77) or BPD3 (OR 5.99, 99.9 % CI 1.27 to 46.77) were more likely to have moderate-severe NDI compared to non-BPD infants.</p></div><div><h3>Conclusion</h3><p>The majority of infants with BPD were discharged home without respiratory support and had better neurocognitive outcomes in early childhood compared to those that required home-based oxygen or respiratory support.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 23-30"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140047067","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":"Learning from cystic fibrosis: How can we start to personalise treatment of Children’s Interstitial Lung Disease (chILD)?","authors":"Andrew Bush","doi":"10.1016/j.prrv.2023.11.001","DOIUrl":"10.1016/j.prrv.2023.11.001","url":null,"abstract":"<div><p>Cystic fibrosis (CF) is a monogenic disorder cause by mutations in the CF Transmembrane Regulator (<em>CFTR</em>) gene. The prognosis of cystic fibrosis has been transformed by the discovery of highly effective modulator therapies (HEMT). Treatment has changed from reactive therapy dealing with complications of the disease to pro-active correction of the underlying molecular functional abnormality. This has come about by discovering the detailed biology of the different CF molecular sub-endotypes; the development of biomarkers to assess response even in mild disease or young children; the performance of definitive large randomised controlled trials in patients with a common mutation and the development of <em>in vitro</em> testing systems to test efficacy in those patients with rare CFTR mutations. As a result, CF is now an umbrella term, rather than a specific diagnostic label; we have moved from clinical phenotypes to molecular subendotypes. Children’s Interstitial Lung Diseases (chILDs) comprise more than 200 entities, and are a diverse group of diseases, for an increasing number of which an underlying gene mutation has been discovered. Many of these entities are umbrella terms, such as pulmonary alveolar proteinosis or hypersensitivity pneumonitis, for each of which there are multiple and very different endotypes. Even those chILDs for which a specific gene mutation has been discovered comprise, as with CF, different molecular subendotypes likely mandating different therapies. For most chILDs, current treatment is non-specific (corticosteroids, azithromycin, hydroxychloroquine). The variability of the different entities means that there is little evidence for the efficacy of any treatment. This review considers how some of the lessons of the success story of CF are being applied to chILD, thus opening the opportunities for truly personalised medicine in these conditions. Advances in knowledge in the molecular biology of surfactant protein C and Adenosine triphosphate binding cassette subfamily A member 3 (ABCA3), and the possibilities of discovering novel therapies by <em>in vitro</em> studies will especially be highlighted.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 46-53"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054223000787/pdfft?md5=45bbcf973d6d171153110b14f0d05b42&pid=1-s2.0-S1526054223000787-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138299796","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}