Journal of paediatrics and child health最新文献

{"title":"Successful Desensitisation With Cystic Fibrosis Transmembrane Conductance Regulator Modulatory Drugs in Delayed Cutaneous Hypersensitivity Reactions.","authors":"Alp Kazancioglu, Umit Murat Sahiner, Meltem Akgul Erdal, Ebru Yalcin, Nagehan Emiralioglu, Deniz Dogru, Ugur Ozcelik, Ozge Soyer","doi":"10.1111/jpc.70175","DOIUrl":"10.1111/jpc.70175","url":null,"abstract":"<p><strong>Aim: </strong>Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies (MT) are recognised as novel and breakthrough treatments for people with cystic fibrosis (PwCF). Use of CFTR modulators has been shown to improve lung function and reduce pulmonary exacerbations in PwCF. However, in clinical studies, 4%-12% of PwCF developed a rash following initiation of MT. This case series aimed to improve the management of delayed hypersensitivity reactions (DHR) to MT in PwCF with the largest paediatric case series of desensitisation achieved to date and a literature review.</p><p><strong>Methods: </strong>We successfully administered the slow desensitisation protocol in five paediatric PwCF referred to our clinic who had to discontinue MT due to delayed non-severe skin reactions. The desensitisation protocol scheduled weekly dose escalations according to the pharmacokinetics of the drugs.</p><p><strong>Results: </strong>Four of the five patients underwent a combination of elexacaftor-tezacaftor-ivacaftor desensitisation, and one patient desensitised with ivacaftor alone. Desensitisation was achieved in all patients, but two patients required protocol modifications. Case 1 started with 0.25 tablets of elexacaftor-tezacaftor-ivacaftor based on the protocol, but the initial desensitisation dose had to be reduced to 0.125 tablets due to the development of a macular rash. In Case 5, a 0.125 tablet dose of ivacaftor was administered due to the severity of the index clinical presentation. In the other cases, the desensitisation was initiated with 0.25 tablets.</p><p><strong>Conclusions: </strong>Desensitisation is an effective treatment approach for non-severe DHR to MT. Our case series enhances the management of DHR to MT, which is essential for PwCF.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"On the Methodological and Justice Considerations in the Australasian Bronchiolitis Guidelines 2025","authors":"Soumyadeep Bhaumik,&nbsp;Habib Bhurawala","doi":"10.1111/jpc.70177","DOIUrl":"10.1111/jpc.70177","url":null,"abstract":"<p>Clinical variation in the management of bronchiolitis is a significant and well-documented feature of paediatric respiratory care worldwide [<span>1</span>]. The first attempt to promote evidence-based approaches for care of infants with bronchiolitis in Australia and Aotearoa New Zealand (AoNZ) was in the form of a landmark piece by Fitzgerald and Kilham [<span>2</span>]. It was expert-driven, reflecting contemporaneous norms. More than a decade later, in 2016, came the first guideline to harmonise treatment for bronchiolitis in emergency departments of Australia and AoNZ, under the auspices of the Paediatric Research in Emergency Departments International Collaborative (PREDICT). A multi-component targeted intervention [<span>3</span>] to improve guideline uptake in primary care found a 14.1% improvement in compliance with guideline recommendations. However, this also means significant variability in practice remains. The 2025 update of the Australasian Bronchiolitis Guidelines is timely and welcome, given its significantly expanded scope [<span>4</span>]. It covers 41 recommendations (11 new and 7 key updates) on 25 topics on management of bronchiolitis in patients presenting to hospital or hospitalised with bronchiolitis (including with SARS-CoV-2 coinfection) and for RSV prevention, thus enhancing its utility.</p><p>While reducing unwarranted variation in clinical practice is the central goal of any guideline, it is equally important to recognise that not all variation is inappropriate. Some degree of variation is necessary to ensure that care remains responsive to local needs and preferences. The <i>raison d'être</i> for a clinical practice guideline is not to eliminate all variation, but to distinguish between what variation is warranted (based on context, equity, and patient need) and what variation is not. Striking this balance within a clinical practice guideline is inherently challenging—medicine after all is both a science and an art. Methodologists seek to achieve this balance through a range of strategies: a structured, transparent process, rigorous evaluation of scientific evidence, and engagement of diverse interest-holders\u0000 ¹\u0000 . A meta-research study [<span>5</span>] found that half of the diagnostic guidelines for children in primary care failed to involve parent representatives, lacked adequate conflict of interest management, and real-world applicability.</p><p>The 2025 Bronchiolitis guideline has made significant progress both on its scope and rigour, demonstrating the commitment to improving care within the PREDICT network [<span>4, 6</span>]. The guideline rates high on evidence evaluation, governance and transparency. The policy for determining, reporting, and managing conflicts of interest was robust and implemented well. The guideline has robust measures of collecting conflict of interests (over various time points), assessing for both presence and severity of conflicts, and inst","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 8","pages":"1166-1168"},"PeriodicalIF":1.4,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70177","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe Hemolytic Anaemia due to Pyruvate Kinase Deficiency in a Patient With Coexistent β-Thalassaemia Trait: A Case Report","authors":"Tugba Belgemen-Ozer","doi":"10.1111/jpc.70174","DOIUrl":"10.1111/jpc.70174","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 10","pages":"1681-1685"},"PeriodicalIF":1.4,"publicationDate":"2025-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144859324","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Newborn With Respiratory Worsening After Nasogastric Tube Insertion","authors":"Enrico Perre,&nbsp;Maria Sole Conte","doi":"10.1111/jpc.70173","DOIUrl":"10.1111/jpc.70173","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 8","pages":"1356-1357"},"PeriodicalIF":1.4,"publicationDate":"2025-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144835409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paediatric Eczema Hospital Admissions, and Topical Corticosteroid Dispensing Pre- and Post-Admission, in New Zealand From 2007 to 2019.","authors":"G Harvey, D J Purvis, J M D Thompson, L Haskell, H Kennedy, K Hoare, S R Dalziel","doi":"10.1111/jpc.70171","DOIUrl":"https://doi.org/10.1111/jpc.70171","url":null,"abstract":"<p><strong>Aim: </strong>To determine prevalence rates of paediatric hospital admissions for eczema/dermatitis in New Zealand (NZ), and assess demographics of these patients. Additionally, to evaluate dispensing of topical corticosteroids in the 12 months prior to and following hospital admission as a marker of healthcare access.</p><p><strong>Methods: </strong>National retrospective observational study of all children aged up to 14 years admitted to hospital in NZ for eczema/dermatitis from 1 January 2007 to 31 December 2019. Analysis of dispensing records of topical corticosteroids for all children aged up to 15 years from 1 January 2006 to 31 December 2020. Evaluation of topical corticosteroid dispensing during the 12 months pre- and post-hospital admission, plus comparison to those children dispensed topical corticosteroids, but not admitted to hospital.</p><p><strong>Results: </strong>The rate of paediatric hospital admission for eczema in NZ was 79.2/100 000 population/year. Hospital admission was significantly higher for Māori children (rate ratio [RR] 6.19, 95% confidence interval [CI] 5.60-6.86), Pacific children (RR 12.35, 95% CI 11.10-13.74), and those of low socioeconomic status (RR 3.89 95% CI 3.43-4.40). Almost one third of children aged < 1 year and just under one fifth of school-aged children were not dispensed topical corticosteroids prior to hospital admission. Most children (> 85%) were dispensed topical corticosteroids in the 12 months following admission.</p><p><strong>Conclusions: </strong>Inequity in hospital admissions for eczema/dermatitis exists for Māori and Pacific children, and those living in areas of greatest deprivation. This research provides unique data on healthcare access for children admitted to hospital for eczema, and highlights areas in which interventions could be targeted.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144835410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paediatric Gastrointestinal Procedural Patterns in New York City During the COVID-19 Pandemic","authors":"Caitlin Cain-Trivette,&nbsp;Natasha Dilwali,&nbsp;Will Simmons,&nbsp;Erika L. Abramson,&nbsp;Zachary Grinspan,&nbsp;Melissa Rose","doi":"10.1111/jpc.70166","DOIUrl":"10.1111/jpc.70166","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>We evaluated changes in paediatric endoscopy patterns in New York City (NYC) before, during, and after the COVID-19 pandemic to see the effects on paediatric procedures, medical utilisation, and medical trainees over 4 years.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Clinical data on gastroenterology procedures performed on patients under 21 years old were collected from five NYC hospitals via the INSIGHT Clinical Research Network. We reviewed data from March to February for 4 years: 2019–2020 as baseline, 2020–2021 as the pandemic, and 2021–2022 and 2022–2023 as post-pandemic.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 40 218 paediatric GI procedures were performed on 18 362 patients. Yearly changes in procedures differed by subcategory, with a significant decrease in invasive procedures during the pandemic compared to the year prior: liver biopsies (43.3% decrease, 95% CI 26.4, 56.7), motility (26.0% decrease, 95% CI 2.89, 43.88), and suction rectal biopsies (48.6% decrease, 95% CI 5.7, 73.1). Urgent procedures such as endoscopic retrograde cholangiopancreatography (ERCP) and foreign body removal continued at a similar rate during the pandemic. In the 2021–2022 post-pandemic year, overall paediatric GI procedures significantly increased by 33.0% (95% CI 19.1, 48.6) compared to pre-pandemic rates in 2019, and remained significantly increased in 2022 by 41.8% (95% CI 27.2, 58.2) compared to 2019. This trend was true for both urgent and non-urgent procedures.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>During the COVID-19 pandemic, urgent procedures continued at similar rates. However, post-pandemic procedural volumes were higher than pre-pandemic numbers, suggesting the pandemic led to delays in paediatric endoscopic care.</p>\u0000 </section>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 10","pages":"1655-1661"},"PeriodicalIF":1.4,"publicationDate":"2025-08-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144816967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe Acute Hepatitis of Unknown Origin in Children: Exploring the Role of Adenovirus and Potential Cofactors","authors":"Jiten Kumar Sahoo,&nbsp;Ankit Agrawal","doi":"10.1111/jpc.70160","DOIUrl":"10.1111/jpc.70160","url":null,"abstract":"<div>\u0000 \u0000 <p>Adenoviruses are a known cause of self-limiting respiratory, ocular, and gastrointestinal infections in children. However, during the recent outbreak in 2021–2022, the identification of human adenoviruses (HAdV), particularly type F41, as a potential cause of severe acute hepatitis in immunocompetent children has sparked global debate. The unusual severity of liver injury and clustering of cases in immunocompetent children have prompted investigations into whether HAdV is truly hepatotropic or merely an incidental finding. Several hypotheses have been proposed, including adenovirus infection, prior severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2 ) infection or adeno-associated virus-2 (AAV2) with a helper virus (HAdV) co-infection. We aim to review the emerging literature on adenovirus and other cofactors as a potential cause of the recent outbreaks of severe acute hepatitis of unknown origin in children (AHUO).</p>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 10","pages":"1566-1572"},"PeriodicalIF":1.4,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144804276","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Factors for Severe Bronchiolitis in Australian and Aotearoa New Zealand Infants: A Systematic Review","authors":"Kate Loveys,&nbsp;Meredith L. Borland,&nbsp;Ed Oakley,&nbsp;Franz E. Babl,&nbsp;Elizabeth Cotterell,&nbsp;Libby Haskell,&nbsp;Sharon O’Brien,&nbsp;Catherine L. Wilson,&nbsp;Emma J. Tavender,&nbsp;Stuart R. Dalziel,&nbsp;the Paediatric Research in Emergency Departments International Collaborative (PREDICT) Network","doi":"10.1111/jpc.70165","DOIUrl":"10.1111/jpc.70165","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>Bronchiolitis is the leading cause of hospital admission in Australasian infants. Infants with risk factors for severe disease may have a greater likelihood of prolonged hospitalisation and intensive care admission. This study aimed to synthesise the literature on risk factors for severe bronchiolitis in Australasian infants.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Systematic review including observational studies of risk factors for severe bronchiolitis in Australasian infants (&lt; 12 months), published from 2000. Databases were searched (24 January 2024): MEDLINE, EMBASE, PubMed, Cochrane Library and CINAHL. Risk of bias (RoB) was assessed using the Newcastle–Ottawa Scale for cohort studies, and evidence quality was evaluated using GRADE. Results were narratively synthesised.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Ten out of 26 467 articles were included (<i>N</i> = 895 276; 12 cohorts, prospective = 5, retrospective = 7). Studies were mostly rated low RoB. There was evidence for the following risk factors: younger chronological age, prematurity, plural birth, comorbidity (chronic lung disease, congenital heart disease, chronic neurological disease, any genetic disorder, any comorbidity), Indigenous ethnicity, economic disadvantage, tobacco smoke exposure and timing of illness onset at presentation. Most risk factors had moderate-quality evidence (range high to very low). Evidence was lacking for the following risk factors present in international literature: breastfeeding exposure and faltering growth. The following risk factors have not been reported in Australasian infants: trisomy-21, congenital diaphragmatic hernia and environmental pollutants.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Risk factors for severe bronchiolitis in Australasian infants are largely consistent with the international literature, although evidence is lacking for some. Knowledge of these risk factors is highly relevant to those assessing infants with bronchiolitis, and in guiding targeted delivery of respiratory syncytial virus immunisation and other preventative programmes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Trial Registration</h3>\u0000 \u0000 <p>PROSPERO (CRD42023463917)</p>\u0000 </section>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 10","pages":"1549-1565"},"PeriodicalIF":1.4,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70165","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144799435","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Practical Guide to Genetic Eye Conditions for Paediatricians","authors":"Richard Lin,&nbsp;Alan Ma,&nbsp;Benjamin M. Nash,&nbsp;Zachary McPherson,&nbsp;John R. Grigg,&nbsp;Robyn V. Jamieson","doi":"10.1111/jpc.70168","DOIUrl":"10.1111/jpc.70168","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Inherited eye disorders, though individually rare, are a collectively common cause of paediatric vision impairment. Many occur as part of a syndrome, in association with congenital anomalies and/or growth/developmental disorders. Paediatricians are well placed to recognise ocular disorders and syndromic associations, and help facilitate appropriate investigations and referrals, including genetic testing. Timely recognition of these conditions may allow patients to capitalise on the recent advances in ocular genetic therapy and clinical trials which are progressing for both non-syndromic and syndromic ocular conditions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>This review provides a practical guide for paediatricians on recognising genetic eye conditions in children, initiating appropriate investigations, and referring for genetic testing.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>An overview of the most common Mendelian paediatric eye conditions and their syndromic associations is provided, encompassing disorders which affect the anterior and/or posterior segments. A suggested framework including a flowchart for recognising potentially inherited ocular conditions and recognising syndromic diagnoses is included. Finally, a discussion regarding the utility of a genetic diagnosis, including information about inheritance, genetic counselling, and current gene therapy and clinical trials is provided.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Genetic eye conditions are an important cause of ocular morbidity in children. These conditions may be isolated to the eye or have multisystem syndromic associations. Additionally, in an era where genetic testing is increasingly being mainstreamed and given the availability of gene therapy, it is relevant for paediatricians to be familiar with genetic eye conditions. This review provides a practical approach for paediatricians to help navigate these conditions.</p>\u0000 </section>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 10","pages":"1538-1548"},"PeriodicalIF":1.4,"publicationDate":"2025-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70168","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789407","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erythematous Papules on the Pulps of the Fingers","authors":"André Aparício Martins,&nbsp;Inês Coutinho,&nbsp;José Carlos Cardoso","doi":"10.1111/jpc.70167","DOIUrl":"10.1111/jpc.70167","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 8","pages":"1354-1355"},"PeriodicalIF":1.4,"publicationDate":"2025-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}