Paediatric Respiratory Reviews最新文献

{"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}

{"title":"Expiratory airflow limitation in adults born extremely preterm: A systematic review and meta-analysis","authors":"Henriette Lahn-Johannessen Lillebøe ,&nbsp;Merete Salveson Engeset ,&nbsp;Hege H Clemm ,&nbsp;Thomas Halvorsen ,&nbsp;Ola Drange Røksund ,&nbsp;Thomas Potrebny ,&nbsp;Maria Vollsæter","doi":"10.1016/j.prrv.2024.02.002","DOIUrl":"10.1016/j.prrv.2024.02.002","url":null,"abstract":"<div><p>Extreme preterm (EP) birth, denoting delivery before the onset of the third trimester, interrupts intrauterine development and causes significant early-life pulmonary trauma, thereby posing a lifelong risk to respiratory health. We conducted a systematic review and <em>meta</em>-analysis to investigate adult lung function following EP birth (gestational age &lt;28 weeks); comparing forced expiratory volume in first second (FEV₁), forced vital capacity (FVC), and FEV₁/FVC to reference values. Subgroup differences were explored based on timing of birth relative to surfactant use (1991) and bronchopulmonary dysplasia (BPD) status. Systematic searches were performed in Medline, EMBASE, Web of Science and Cochrane Central. Quality assessments were carried out using a modified Newcastle-Ottawa Scale for cohort studies. Sixteen studies encompassing 1036 EP-born adults were included, with 14 studies (n = 787) reporting data as %predicted, and 11 (n = 879) as z-score (not mutually exclusive). Overall mean [95 % confidence interval (CI)] %FEV₁ was 85.30 (82.51; 88.09), %FVC was 94.33 (91.74; 96.91), and FEV₁/FVC was 79.54 (77.71 to 81.38), all three with high heterogeneity. Overall mean (95 %CI) zFEV₁ was −1.05 (-1.21; −0.90) and zFVC was.</p><p>−0.45 (-0.59; −0.31), both with moderate heterogeneity. Subgroup analyses revealed no difference in FEV₁ before versus after widespread use of surfactant, but more impairments after neonatal BPD. This <em>meta</em>-analysis revealed significant airflow limitation in EP-born adults, mostly explained by those with neonatal BPD. FEV₁ was more reduced than FVC, and FEV₁/FVC was at the lower limit of normal. Although at a group level, most adult EP-born individuals do not meet COPD criteria, these findings are concerning.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 2-22"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054224000198/pdfft?md5=1cbd340fc004995d5289d5a0dc978981&pid=1-s2.0-S1526054224000198-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139927099","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":"Characterising the lifelong consequences of bronchopulmonary dysplasia","authors":"Dominic A. Fitzgerald","doi":"10.1016/j.prrv.2024.03.001","DOIUrl":"10.1016/j.prrv.2024.03.001","url":null,"abstract":"","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Page 1"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140403352","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":"Adherence in paediatric respiratory medicine: A review of the literature","authors":"Ella A. Kotecha ,&nbsp;Dominic A. Fitzgerald ,&nbsp;Sailesh Kotecha","doi":"10.1016/j.prrv.2023.09.004","DOIUrl":"10.1016/j.prrv.2023.09.004","url":null,"abstract":"<div><p>Poor adherence is an important factor in unstable disease control and treatment failure. There are multiple ways to monitor a patient’s adherence, each with their own advantages and disadvantages. The reasons for poor adherence are multi-factorial, inter-related and often difficult to target for improvement. Although practitioners can implement different methods of adherence, the ultimate aim is to improve health outcomes for the individual and the health care system. Asthma is a common airway disease, particularly diagnosed in children, often treated with inhaled corticosteroids and long-acting bronchodilators. Due to the disease’s tendency for exacerbations and consequently, when severe will require unscheduled health care utilisation including hospital admissions, considerable research has been done into the effects of medication adherence on asthma control. This review discusses the difficulties in defining adherence, the reasons for and consequences of poor adherence, and the methods of recording and improving adherence in asthma patients, including an in-depth analysis of the uses of smart inhalers.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 41-45"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054223000660/pdfft?md5=633e8afb7aa539e37c80492304a0f737&pid=1-s2.0-S1526054223000660-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41208098","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":"Should an inhaled corticosteroid accompany each dose of fast-acting beta2-agonist for relief of asthma symptoms?","authors":"Leslie Hendeles ,&nbsp;Miles Weinberger","doi":"10.1016/j.prrv.2023.05.005","DOIUrl":"10.1016/j.prrv.2023.05.005","url":null,"abstract":"","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"50 ","pages":"Pages 38-40"},"PeriodicalIF":5.8,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140402126","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":"Positive end-expiratory pressure in chronic care of children with obstructive sleep apnoea","authors":"Brigitte Fauroux ,&nbsp;Meryl Vedrenne-Cloquet","doi":"10.1016/j.prrv.2023.01.001","DOIUrl":"10.1016/j.prrv.2023.01.001","url":null,"abstract":"<div><p>Positive end-expiratory pressure (PEEP) consists of the delivery of a constant positive pressure in the airways by means of a noninvasive interface aiming to maintain airway patency throughout the entire respiratory cycle. PEEP is increasingly used in the chronic care of children with anatomical or functional abnormalities of the upper airways to correct severe persistent obstructive sleep apnea despite optimal management which commonly includes adenotonsillectomy<span> in young children<span>. PEEP may be used at any age, due to improvements in equipment and interfaces. Criteria for CPAP/NIV initiation, optimal setting, follow-up and monitoring, as well as weaning criteria have been established by international experts, but validated criteria are lacking. As chronic PEEP is a highly specialised treatment<span>, patients should be managed by an expert pediatric multidisciplinary team.</span></span></span></p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"49 ","pages":"Pages 2-4"},"PeriodicalIF":5.8,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9179042","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":"Diagnostic challenges in CFTR-related metabolic syndrome: Where the guidelines fall short","authors":"Erin F. Kallam ,&nbsp;Ajay S. Kasi ,&nbsp;Eileen Barr ,&nbsp;Rachel W. Linnemann ,&nbsp;Lokesh Guglani","doi":"10.1016/j.prrv.2023.08.004","DOIUrl":"10.1016/j.prrv.2023.08.004","url":null,"abstract":"<div><p><span>Newborn screening<span> (NBS) for cystic fibrosis (CF) has enabled earlier diagnosis and has improved nutritional and growth-related outcomes in children with CF. For those with a positive NBS for CF that do not meet the diagnostic criteria for CF, the clinical entity called CFTR-Related </span></span>Metabolic Syndrome (CRMS) or CF Screen- Positive, Inconclusive Diagnosis (CFSPID) is used. Although most children with CRMS remain relatively asymptomatic, studies have shown that between 11% and 48% of these patients may eventually progress to a diagnosis of CF over time. Although the CF Foundation guidelines for CRMS management and European CF Society guidelines for CFSPID have some similarities, there are also some differences. Here, we review challenging case scenarios that highlight remaining gaps in CRMS guidelines, thus supporting the need to update and unify existing guidelines.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"49 ","pages":"Pages 28-33"},"PeriodicalIF":5.8,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10137990","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":"Positive end-expiratory pressure in the pediatric intensive care unit","authors":"Martin C.J. Kneyber","doi":"10.1016/j.prrv.2023.11.003","DOIUrl":"10.1016/j.prrv.2023.11.003","url":null,"abstract":"<div><p>Application of positive end-expiratory pressure (PEEP) targeted towards improving oxygenation is one of the components of the ventilatory management of pediatric acute respiratory distress syndrome (PARDS). Low end-expiratory airway pressures cause repetitive opening and closure of unstable alveoli, leading to surfactant dysfunction and parenchymal shear injury. Consequently, there is less lung volume available for tidal ventilation when there are atelectatic lung regions. This will increase lung strain in aerated lung areas to which the tidal volume is preferentially distributed. Pediatric critical care practitioners tend to use low levels of PEEP and inherently accept higher FiO₂, but these practices may negatively affect patient outcome. The Pediatric Acute Lung Injury Consensus Conference (PALICC) suggests that PEEP should be titrated to oxygenation/oxygen delivery, hemodynamics, and compliance measured under static conditions as compared to other clinical parameters or any of these parameters in isolation in patients with PARDS, while limiting plateau pressure and/or driving pressure limits.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"49 ","pages":"Pages 5-8"},"PeriodicalIF":5.8,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1526054223000805/pdfft?md5=8a8401dcb165410bb49f999923c16c82&pid=1-s2.0-S1526054223000805-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138461455","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":"Can postural changes in spirometry in children with Duchenne muscular dystrophy predict sleep hypoventilation?","authors":"C. Pandit ,&nbsp;B. Kennedy ,&nbsp;K. Waters ,&nbsp;H. Young ,&nbsp;K. Jones ,&nbsp;D.A. Fitzgerald","doi":"10.1016/j.prrv.2023.08.002","DOIUrl":"10.1016/j.prrv.2023.08.002","url":null,"abstract":"<div><h3>Aim</h3><p><span>To explore the relationship between postural changes in lung function and polysomnography (PSG) in children with </span>Duchenne muscular dystrophy (DMD).</p></div><div><h3>Methods</h3><p>In this prospective cross-sectional study, children with DMD performed spirometry in sitting and supine positions. A control group of age and gender matched healthy children also underwent postural lung function testing. PSG was performed within six months of spirometry.</p></div><div><h3>Results</h3><p>Seventeen children with DMD, aged 12.3 ± 3 years performed sitting spirometry. 14 (84%) performed acceptable spirometry in the supine position. Mean FEV₁<em>_sit</em><span> and FVC</span><em>_sit</em> were 77% (SD ± 22) and 74% (SD ± 20.4) respectively, with mean% ΔFVC₍<em>_sit–sup</em>₎ 9% (SD ± 11) (range 2% to 20%), and was significantly greater than healthy controls 4% (n = 30, SD ± 3, <em>P</em> &lt; 0.001). PSG data on the 14 DMD children with acceptable supine spirometry showed total AHI 6.9 ± 5.9/hour (0.3 to 29), obstructive AHI 5.2 ± 4.0/hour (0.2 to 10), and REM AHI 14.1 ± -5.3/hour (0.1 to 34.7). ΔFVC(sit–sup) had poor correlation with hypoventilation on polysomnography.</p></div><div><h3>Conclusion</h3><p>Children with DMD and mild restrictive lung disease showed greater postural changes in spirometry than healthy controls but lower supine spirometry was not predictive of sleep hypoventilation.</p></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"49 ","pages":"Pages 9-13"},"PeriodicalIF":5.8,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10579871","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}