首页 > 最新文献

Journal of paediatrics and child health最新文献

英文 中文
The Prevalence of Scabies, Skin Infection and Rheumatic Heart Disease in a Cross-Sectional Study of Tongan Primary School Children. 同安小学儿童疥疮、皮肤感染和风湿性心脏病流行的横断面调查
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-12-04 DOI: 10.1111/jpc.70248
Simon Thornley, Toakase Fakakovikaetau, Mele Tilema Cama, Mele 'Atuekaho Manu, Kalolaine Tahitua, Shunsuke Moritsuka, Richard J Johnson, Viliami Puloka, Losana Vuki, Joseph Takai, Jacqui Williamson, Saptorshi Gupta, Arthur J Morris, Gerhard Sundborn

Background: Scabies is a common health issue in the Pacific and may contribute to rheumatic heart disease (RHD). This study assessed the prevalence of scabies and other skin conditions among Tongan schoolchildren and examined the association between these conditions and RHD.

Methods: Children from four primary schools on Tongatapu were screened for scabies, other skin conditions and RHD. Health workers were trained to recognise scabies and related skin conditions accurately. All children also underwent echocardiographic screening to detect heart valve abnormalities consistent with RHD. Statistical analysis included prevalence ratio calculations, and Fisher's exact tests to evaluate associations between skin and heart findings.

Results: Four hundred children were screened (mean age: 8 years 6 months), with 18 (4.5%) having echocardiographic features of RHD. The prevalence of scabies was 29.8% (119/400), while the prevalence of impetigo was 20% and more severe bacterial skin infection was 15.5%. Children with both scabies and skin infection were 4.65 times more likely than children without any such lesions to have RHD (95% confidence interval: 1.60-13.6). Children with scabies, impetigo and bacterial infection were associated with progressively higher prevalence of RHD (p for trend = 0.008). The population attributable fraction for scabies and infection as a potential cause of RHD was 26.5%.

Conclusions: The results indicate a very high prevalence of scabies, bacterial skin infection, and RHD among Tongan school children. Enhanced treatment and control of scabies are likely to reduce the incidence of both bacterial infection and RHD. Mass drug administration is recommended.

背景:疥疮是太平洋地区常见的健康问题,可能导致风湿性心脏病(RHD)。本研究评估了汤加学龄儿童中疥疮和其他皮肤病的患病率,并研究了这些疾病与RHD之间的关系。方法:对汤加塔普四所小学的儿童进行疥疮、其他皮肤疾病和RHD筛查。卫生工作者接受了准确识别疥疮和相关皮肤状况的培训。所有儿童还接受了超声心动图筛查,以检测与RHD相符的心脏瓣膜异常。统计分析包括患病率计算和Fisher的精确测试,以评估皮肤和心脏发现之间的联系。结果:筛选了400名儿童(平均年龄:8岁6个月),其中18名(4.5%)具有RHD的超声心动图特征。疥疮患病率为29.8%(119/400),脓疱疮患病率为20%,较严重的细菌性皮肤感染患病率为15.5%。同时患有疥疮和皮肤感染的儿童患RHD的可能性是没有任何此类病变儿童的4.65倍(95%置信区间:1.60-13.6)。患有疥疮、脓疱疮和细菌感染的儿童与RHD患病率逐渐升高相关(趋势p = 0.008)。疥疮和感染作为RHD潜在病因的人群归因比例为26.5%。结论:汤加学龄儿童疥疮、细菌性皮肤感染和RHD患病率很高。加强治疗和控制疥疮可能会减少细菌感染和RHD的发病率。建议大量给药。
{"title":"The Prevalence of Scabies, Skin Infection and Rheumatic Heart Disease in a Cross-Sectional Study of Tongan Primary School Children.","authors":"Simon Thornley, Toakase Fakakovikaetau, Mele Tilema Cama, Mele 'Atuekaho Manu, Kalolaine Tahitua, Shunsuke Moritsuka, Richard J Johnson, Viliami Puloka, Losana Vuki, Joseph Takai, Jacqui Williamson, Saptorshi Gupta, Arthur J Morris, Gerhard Sundborn","doi":"10.1111/jpc.70248","DOIUrl":"https://doi.org/10.1111/jpc.70248","url":null,"abstract":"<p><strong>Background: </strong>Scabies is a common health issue in the Pacific and may contribute to rheumatic heart disease (RHD). This study assessed the prevalence of scabies and other skin conditions among Tongan schoolchildren and examined the association between these conditions and RHD.</p><p><strong>Methods: </strong>Children from four primary schools on Tongatapu were screened for scabies, other skin conditions and RHD. Health workers were trained to recognise scabies and related skin conditions accurately. All children also underwent echocardiographic screening to detect heart valve abnormalities consistent with RHD. Statistical analysis included prevalence ratio calculations, and Fisher's exact tests to evaluate associations between skin and heart findings.</p><p><strong>Results: </strong>Four hundred children were screened (mean age: 8 years 6 months), with 18 (4.5%) having echocardiographic features of RHD. The prevalence of scabies was 29.8% (119/400), while the prevalence of impetigo was 20% and more severe bacterial skin infection was 15.5%. Children with both scabies and skin infection were 4.65 times more likely than children without any such lesions to have RHD (95% confidence interval: 1.60-13.6). Children with scabies, impetigo and bacterial infection were associated with progressively higher prevalence of RHD (p for trend = 0.008). The population attributable fraction for scabies and infection as a potential cause of RHD was 26.5%.</p><p><strong>Conclusions: </strong>The results indicate a very high prevalence of scabies, bacterial skin infection, and RHD among Tongan school children. Enhanced treatment and control of scabies are likely to reduce the incidence of both bacterial infection and RHD. Mass drug administration is recommended.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145668637","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}
引用次数: 0
Impact of Elexacaftor/Tezacaftor/Ivacaftor (ETI) Treatment on Clinical Outcomes in a Single Centre Cohort of Paediatric Patients With Cystic Fibrosis elexaftor /Tezacaftor/Ivacaftor (ETI)治疗对囊性纤维化患儿单中心队列临床结果的影响
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-12-04 DOI: 10.1111/jpc.70249
Chloe Crainie, K. Frayman, J. Harrison
<p>Cystic Fibrosis (CF) affects approximately 3800 Australians, including 42% aged under 18 years [<span>1</span>]. CF is associated with significant morbidity, with many people spending at least 60 min per day on treatment. Life expectancy has progressively improved due to the development of new therapies: median survival is now 60.6 years, increased from 47 years in 2008 [<span>1</span>]. Elexacaftor, Tezacaftor and Ivacaftor (ETI) (Trikafta), the first triple-therapy CF transmembrane conductance regulator modulator, became available in Australia on the Pharmaceutical Benefits Scheme in 2022. This study aimed to measure changes in the clinical status of adolescents with CF who have completed 12-months of treatment with ETI in a real-world setting.</p><p>Adolescents with CF aged ≥ 12 years managed at the Royal Children's Hospital (RCH), Melbourne who had completed 12 months of ETI treatment by August 2023 were included. Demographic data and clinical outcomes, including lung function, weight, nutritional status, hospitalisations and microbiology were extracted from the electronic medical record and the Australian Cystic Fibrosis Data Registry (ACFDR). ‘Best’ and ‘mean’ values in the 12-month period pre- and post-ETI initiation were compared. Data analysis was performed in PRISM GraphPad V10.0.3. Rate of change of forced expiratory volume in 1-s (FEV<sub>1</sub>) per annum was calculated using linear regression; for each individual, a regression line was fitted across all FEV<sub>1</sub> measurements within each 12-month period. Paired <i>t</i>-tests and Wilcoxon signed rank tests were used to compare normally and non-normally distributed data, respectively. The study was approved by RCH Human Research Ethics Committee (#101289).</p><p>Sixty-eight adolescents were included in this study (54% male, 51% F508del homozygous, mean age 15.29 ± 1.96 years) (an additional nine patients had commenced ETI prior to 12 years (i.e., via clinical trials), and four had completed < 12 months treatment at the time of analysis). Forty study participants (59%) had prior modulator exposure (Lumacaftor-Ivacaftor 55%; Tezacaftor-Ivacaftor 37.5%; Ivacaftor 7.5%).</p><p>ETI was associated with significant changes in several clinical outcomes (Table 1). Fewer lung function tests were performed following the introduction of ETI (median six and four per patient pre- and post-ETI respectively (<i>p</i> < 0.00001)). Most patients achieved an increase in FEV<sub>1</sub>%-predicted of 1%–10% (Figure 1a), and the rate of change in FEV<sub>1</sub> increased after ETI commencement (<i>p</i> = 0.06). Although there was no significant change in weight or BMI post-ETI initiation, four patients (5.9%) moved from an ‘optimal’ BMI category (50th–85th percentile) to a ‘high’ BMI category (> 85th percentile). There were significant reductions in hospitalisation frequency and duration post-ETI initiation. The total number of hospital admissions decreased from 84 to 34; all patient
由于静息能量消耗减少、食欲增加和脂肪吸收不良减少等多种因素,ETI可导致BMI和体重增加。因此,ETI治疗可能导致肥胖率增加。在我们的研究中,超重和肥胖青少年比例的增加(总计19.2%)带来了新的临床挑战,包括修改饮食建议和监测肥胖的长期并发症。继续积极主动地接受饮食建议是至关重要的。可能样本量小,随访时间相对较短,纳入了相对健康的基线人群,影响了我们研究结果的幅度和统计意义,例如,FEV1的变化率。较大的IQR/SD可能反映了我们的队列中潜在的CFTR基因型/残留蛋白功能和基线疾病严重程度直接影响CFTR调节剂的反应,在现实世界的研究中,依从性、耐受性和潜在的剂量也会影响CFTR调节剂的反应[2,3,5]。但重要的是,我们的研究队列是澳大利亚CF青少年的代表,与2023年12-17岁CF患者的ACFDR数据相比,FEV1或体重没有显著差异。总的来说,我们证明了ETI对患有CF的澳大利亚青少年的显著影响,并预期对CF护理的前景产生重大影响。肺功能的改善,在前12个月,可能与护理负担的大幅减少和预期寿命的增加有关。护理建议需要更新,包括提高对肥胖风险的认识,关注长期健康,预期能活到成年后期。这项研究证明了澳大利亚儿童获得调节剂的广泛临床和医疗保健益处,为争取更快的资金批准和更公平地获得未来的新疗法提供了证据。几个关键的研究重点仍然是优化长期结果。需要进行纵向研究来评估ETI益处的持久性,以及在儿童早期开始时出现的不良影响。早期治疗是否能够预防或完全逆转结构性肺损伤,以及减少慢性阻塞性肺疾病相关糖尿病等长期并发症,这些问题仍然存在。未来的工作可能会探索治疗后儿童身体组成、脂肪代谢的变化,以及葡萄糖稳态改变的潜在风险——开发反映这些代谢变化的新的营养监测框架将是必不可少的。未来的研究应该旨在为个性化治疗和监测方法提供信息,确保ETI的早期益处转化为患者持续的健康收益。对于不符合条件或不能耐受ETI的患者,迫切需要开发新的治疗方法。作者声明无利益冲突。支持本研究结果的数据可根据通讯作者的合理要求提供。
{"title":"Impact of Elexacaftor/Tezacaftor/Ivacaftor (ETI) Treatment on Clinical Outcomes in a Single Centre Cohort of Paediatric Patients With Cystic Fibrosis","authors":"Chloe Crainie,&nbsp;K. Frayman,&nbsp;J. Harrison","doi":"10.1111/jpc.70249","DOIUrl":"10.1111/jpc.70249","url":null,"abstract":"&lt;p&gt;Cystic Fibrosis (CF) affects approximately 3800 Australians, including 42% aged under 18 years [&lt;span&gt;1&lt;/span&gt;]. CF is associated with significant morbidity, with many people spending at least 60 min per day on treatment. Life expectancy has progressively improved due to the development of new therapies: median survival is now 60.6 years, increased from 47 years in 2008 [&lt;span&gt;1&lt;/span&gt;]. Elexacaftor, Tezacaftor and Ivacaftor (ETI) (Trikafta), the first triple-therapy CF transmembrane conductance regulator modulator, became available in Australia on the Pharmaceutical Benefits Scheme in 2022. This study aimed to measure changes in the clinical status of adolescents with CF who have completed 12-months of treatment with ETI in a real-world setting.&lt;/p&gt;&lt;p&gt;Adolescents with CF aged ≥ 12 years managed at the Royal Children's Hospital (RCH), Melbourne who had completed 12 months of ETI treatment by August 2023 were included. Demographic data and clinical outcomes, including lung function, weight, nutritional status, hospitalisations and microbiology were extracted from the electronic medical record and the Australian Cystic Fibrosis Data Registry (ACFDR). ‘Best’ and ‘mean’ values in the 12-month period pre- and post-ETI initiation were compared. Data analysis was performed in PRISM GraphPad V10.0.3. Rate of change of forced expiratory volume in 1-s (FEV&lt;sub&gt;1&lt;/sub&gt;) per annum was calculated using linear regression; for each individual, a regression line was fitted across all FEV&lt;sub&gt;1&lt;/sub&gt; measurements within each 12-month period. Paired &lt;i&gt;t&lt;/i&gt;-tests and Wilcoxon signed rank tests were used to compare normally and non-normally distributed data, respectively. The study was approved by RCH Human Research Ethics Committee (#101289).&lt;/p&gt;&lt;p&gt;Sixty-eight adolescents were included in this study (54% male, 51% F508del homozygous, mean age 15.29 ± 1.96 years) (an additional nine patients had commenced ETI prior to 12 years (i.e., via clinical trials), and four had completed &lt; 12 months treatment at the time of analysis). Forty study participants (59%) had prior modulator exposure (Lumacaftor-Ivacaftor 55%; Tezacaftor-Ivacaftor 37.5%; Ivacaftor 7.5%).&lt;/p&gt;&lt;p&gt;ETI was associated with significant changes in several clinical outcomes (Table 1). Fewer lung function tests were performed following the introduction of ETI (median six and four per patient pre- and post-ETI respectively (&lt;i&gt;p&lt;/i&gt; &lt; 0.00001)). Most patients achieved an increase in FEV&lt;sub&gt;1&lt;/sub&gt;%-predicted of 1%–10% (Figure 1a), and the rate of change in FEV&lt;sub&gt;1&lt;/sub&gt; increased after ETI commencement (&lt;i&gt;p&lt;/i&gt; = 0.06). Although there was no significant change in weight or BMI post-ETI initiation, four patients (5.9%) moved from an ‘optimal’ BMI category (50th–85th percentile) to a ‘high’ BMI category (&gt; 85th percentile). There were significant reductions in hospitalisation frequency and duration post-ETI initiation. The total number of hospital admissions decreased from 84 to 34; all patient","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"151-154"},"PeriodicalIF":1.4,"publicationDate":"2025-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70249","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145668620","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}
引用次数: 0
A Post-Extubation Early Feeding Protocol (PEEF) in a Tertiary Paediatric Intensive Care Unit: A Quality Improvement Initiative. 第三儿科重症监护病房拔管后早期喂养方案(PEEF):质量改进倡议。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-12-02 DOI: 10.1111/jpc.70251
Sora Jung, Sainath Raman, Ian Hughes, Rahul Joshi

Aim: Recommencing enteral feeding post-extubation in children is often delayed. This study evaluated a Post-Extubation Early Feeding (PEEF) protocol on early recommencement of enteral nutrition post-extubation in a tertiary paediatric intensive care unit (PICU).

Methods: The PEEF protocol involved direction to re-commence enteral nutrition at 50% of the pre-fasting rate within 1 h of extubation and upgrade to the pre-extubation feeding rate within 2 h. The PEEF protocol was a quality improvement project implemented at a tertiary PICU and was evaluated through a retrospective analysis. The study included children aged 0-18 years who were admitted to PICU, intubated and subsequently underwent planned extubation pre-PEEF (November 2019-October 2020) and post-PEEF (November 2021-October 2022).

Results: A total of 795 patients were included in the study, 372 pre-PEEF and 423 post-PEEF. Demographic variables and severity of illness variables were similar in the two groups. Post implementation of the PEEF protocol, the median (interquartile range) time to re-commence enteral nutrition after extubation was 3.2 h (1.9, 4.7) compared to pre-PEEF, 4.2 h (3.2, 5.5; p = 2.4 × 10-13). Post-PEEF the median time to reach the pre-fasting feeding rate was 5 h (2.6, 10.6), compared to 7.3 h (4.1, 13) pre-PEEF (p = 0.0005).

Conclusions: Implementation of the PEEF protocol reduced the time to resume enteral nutrition and the time to reach pre-extubation feeding rates although improvement was not consistent with the goals of PEEF. Continuing assessment and education are required to drive outcomes further towards target goals.

目的:儿童拔管后肠内喂养的重新开始经常延迟。本研究评估了拔管后早期喂养(PEEF)方案在三级儿科重症监护病房(PICU)拔管后早期重新开始肠内营养。方法:PEEF方案包括在拔管后1 h内以50%的禁食前速率重新开始肠内营养,并在2 h内升级到拔管前喂养速率。PEEF方案是在三级PICU实施的质量改进项目,并通过回顾性分析进行评估。该研究包括0-18岁的儿童,他们在peef前(2019年11月- 2020年10月)和peef后(2021年11月- 2022年10月)入住PICU,插管并随后计划拔管。结果:共有795例患者纳入研究,peef前372例,peef后423例。两组的人口学变量和疾病严重程度变量相似。实施PEEF方案后,拔管后重新开始肠内营养的中位数(四分位数范围)时间为3.2 h(1.9, 4.7),而PEEF前为4.2 h (3.2, 5.5; p = 2.4 × 10-13)。peef后达到禁食前摄食速率的中位时间为5 h(2.6, 10.6),而peef前为7.3 h (4.1, 13) (p = 0.0005)。结论:PEEF方案的实施减少了恢复肠内营养的时间和达到拔管前喂养率的时间,尽管这种改善与PEEF的目标不一致。需要继续进行评估和教育,以推动成果进一步实现具体目标。
{"title":"A Post-Extubation Early Feeding Protocol (PEEF) in a Tertiary Paediatric Intensive Care Unit: A Quality Improvement Initiative.","authors":"Sora Jung, Sainath Raman, Ian Hughes, Rahul Joshi","doi":"10.1111/jpc.70251","DOIUrl":"https://doi.org/10.1111/jpc.70251","url":null,"abstract":"<p><strong>Aim: </strong>Recommencing enteral feeding post-extubation in children is often delayed. This study evaluated a Post-Extubation Early Feeding (PEEF) protocol on early recommencement of enteral nutrition post-extubation in a tertiary paediatric intensive care unit (PICU).</p><p><strong>Methods: </strong>The PEEF protocol involved direction to re-commence enteral nutrition at 50% of the pre-fasting rate within 1 h of extubation and upgrade to the pre-extubation feeding rate within 2 h. The PEEF protocol was a quality improvement project implemented at a tertiary PICU and was evaluated through a retrospective analysis. The study included children aged 0-18 years who were admitted to PICU, intubated and subsequently underwent planned extubation pre-PEEF (November 2019-October 2020) and post-PEEF (November 2021-October 2022).</p><p><strong>Results: </strong>A total of 795 patients were included in the study, 372 pre-PEEF and 423 post-PEEF. Demographic variables and severity of illness variables were similar in the two groups. Post implementation of the PEEF protocol, the median (interquartile range) time to re-commence enteral nutrition after extubation was 3.2 h (1.9, 4.7) compared to pre-PEEF, 4.2 h (3.2, 5.5; p = 2.4 × 10<sup>-13</sup>). Post-PEEF the median time to reach the pre-fasting feeding rate was 5 h (2.6, 10.6), compared to 7.3 h (4.1, 13) pre-PEEF (p = 0.0005).</p><p><strong>Conclusions: </strong>Implementation of the PEEF protocol reduced the time to resume enteral nutrition and the time to reach pre-extubation feeding rates although improvement was not consistent with the goals of PEEF. Continuing assessment and education are required to drive outcomes further towards target goals.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145661080","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}
引用次数: 0
A Neonatal Case of Cyanide Toxicity Caused by Sodium Nitroprusside During Veno-Arterial Extracorporeal Membrane Oxygenation Management. 静脉-动脉体外膜氧合处理中硝普钠致新生儿氰化物中毒1例。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-12-02 DOI: 10.1111/jpc.70250
Tsubasa Nishinosono, Ken Yakame, Ayaka Sakai, Saori Aiga, Sanae Sato, Mitsunori Sato, Tatsuya Kawasaki
{"title":"A Neonatal Case of Cyanide Toxicity Caused by Sodium Nitroprusside During Veno-Arterial Extracorporeal Membrane Oxygenation Management.","authors":"Tsubasa Nishinosono, Ken Yakame, Ayaka Sakai, Saori Aiga, Sanae Sato, Mitsunori Sato, Tatsuya Kawasaki","doi":"10.1111/jpc.70250","DOIUrl":"https://doi.org/10.1111/jpc.70250","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145660916","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}
引用次数: 0
Trends in Community Antibiotic Dispensing for Children and Young People in Aotearoa New Zealand, 2010-2019: Implications for Antimicrobial Stewardship. 2010-2019年新西兰奥特罗阿儿童和青少年社区抗生素分配趋势:对抗菌药物管理的影响
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-11-29 DOI: 10.1111/jpc.70236
Anya Stephenson, Ibrahim S Al-Busaidi, Jonathan Williman, Kirsten Fanning, Phil Hider, Lesley Voss

Aim: To analyse trends in community systemic antibiotic dispensing to children and young people (0-14 years) in New Zealand between 2010 and 2019.

Methods: Dispensing data were retrieved from the National Pharmaceuticals Collection and population data from the Te Whatu Ora Populations Web Tool. Dispensing rates were described using the number of defined daily doses/1000 inhabitants per day (DID) and courses dispensed/1000 inhabitants/year.

Results: Total antibiotic dispensing declined over the study period, both in terms of courses dispensed (annual percentage change [APC] -3.4% per year) and DIDs (APC -0.67% per year). Amoxicillin/clavulanic acid showed the greatest decline in dispensing (APC -9.5% per year), while cefalexin had the most significant increase (APC +53.7% per year). The highest dispensing rates were observed among children aged 0-4 years, Pacific patients and those in the lowest socioeconomic quintile. Counties Manukau had the highest regional dispensing rates. Antibiotic stewardship, as per the WHO AWaRe guidelines, improved: Access antibiotics increased from 83% to 91%, while Watch antibiotics decreased from 17% to 9% of total dispensing.

Conclusions: These findings suggest an overall improvement in prescribing practices, likely due to the adoption of antimicrobial stewardship programmes. Further study is under way to understand the reasons for and any risks associated with, high community rates of cefalexin dispensing.

目的:分析2010年至2019年新西兰儿童和青少年(0-14岁)社区系统抗生素分配的趋势。方法:调剂数据来自国家药品数据库,人口数据来自the Whatu Ora人口网络工具。分配率用每日确定剂量/1000居民/天(DID)和分配疗程/1000居民/年来描述。结果:在研究期间,抗生素总配药量下降,无论是配药疗程(年百分比变化[APC] -3.4% /年)还是DIDs (APC -0.67% /年)。阿莫西林/克拉维酸的分配下降幅度最大(APC -9.5% /年),而头孢氨苄的增加幅度最大(APC +53.7% /年)。在0-4岁儿童、太平洋患者和社会经济最低五分位数的儿童中,分配率最高。马努考县的区域配药率最高。根据世卫组织AWaRe指南,抗生素管理得到改善:可获得抗生素从83%增加到91%,而观察抗生素从总分配的17%下降到9%。结论:这些发现表明处方实践的全面改进,可能是由于采用了抗微生物药物管理规划。目前正在进行进一步的研究,以了解社区高头孢氨苄配药率的原因和相关风险。
{"title":"Trends in Community Antibiotic Dispensing for Children and Young People in Aotearoa New Zealand, 2010-2019: Implications for Antimicrobial Stewardship.","authors":"Anya Stephenson, Ibrahim S Al-Busaidi, Jonathan Williman, Kirsten Fanning, Phil Hider, Lesley Voss","doi":"10.1111/jpc.70236","DOIUrl":"https://doi.org/10.1111/jpc.70236","url":null,"abstract":"<p><strong>Aim: </strong>To analyse trends in community systemic antibiotic dispensing to children and young people (0-14 years) in New Zealand between 2010 and 2019.</p><p><strong>Methods: </strong>Dispensing data were retrieved from the National Pharmaceuticals Collection and population data from the Te Whatu Ora Populations Web Tool. Dispensing rates were described using the number of defined daily doses/1000 inhabitants per day (DID) and courses dispensed/1000 inhabitants/year.</p><p><strong>Results: </strong>Total antibiotic dispensing declined over the study period, both in terms of courses dispensed (annual percentage change [APC] -3.4% per year) and DIDs (APC -0.67% per year). Amoxicillin/clavulanic acid showed the greatest decline in dispensing (APC -9.5% per year), while cefalexin had the most significant increase (APC +53.7% per year). The highest dispensing rates were observed among children aged 0-4 years, Pacific patients and those in the lowest socioeconomic quintile. Counties Manukau had the highest regional dispensing rates. Antibiotic stewardship, as per the WHO AWaRe guidelines, improved: Access antibiotics increased from 83% to 91%, while Watch antibiotics decreased from 17% to 9% of total dispensing.</p><p><strong>Conclusions: </strong>These findings suggest an overall improvement in prescribing practices, likely due to the adoption of antimicrobial stewardship programmes. Further study is under way to understand the reasons for and any risks associated with, high community rates of cefalexin dispensing.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634936","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}
引用次数: 0
Phaeochromocytoma in a Child Presenting With Post-Streptococcal Glomerulonephritis and Hypertensive Encephalopathy—Case Report 以链球菌感染后肾小球肾炎和高血压性脑病为表现的儿童嗜铬细胞瘤1例报告。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-11-27 DOI: 10.1111/jpc.70242
J. Loh, C. Mincham, N. Larkins, T. Ford, M. Princehorn, C. Kiraly-Borri, N. Jain
{"title":"Phaeochromocytoma in a Child Presenting With Post-Streptococcal Glomerulonephritis and Hypertensive Encephalopathy—Case Report","authors":"J. Loh,&nbsp;C. Mincham,&nbsp;N. Larkins,&nbsp;T. Ford,&nbsp;M. Princehorn,&nbsp;C. Kiraly-Borri,&nbsp;N. Jain","doi":"10.1111/jpc.70242","DOIUrl":"10.1111/jpc.70242","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"138-142"},"PeriodicalIF":1.4,"publicationDate":"2025-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634911","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}
引用次数: 0
MIST/LISA in Portugal: National Survey of Clinical Practice. MIST/LISA在葡萄牙:临床实践的全国调查。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-11-27 DOI: 10.1111/jpc.70247
Sílvia Duarte Costa, Sofia Silva Faria, Cláudia Ferraz, Ana Azevedo

Background: Minimally/less invasive surfactant therapy/administration (MIST/LISA) is increasingly used for surfactant delivery in preterm infants with respiratory distress syndrome, aiming to reduce complications associated with invasive methods. Despite its growing adoption, variations in clinical practice remain.

Objective: To evaluate MIST/LISA use in Portuguese neonatal intensive care units (NICUs) and identify variations in practice.

Methods: A national, anonymous questionnaire was distributed to neonatologists. The survey explored surfactant administration techniques, experience with MIST/LISA, device preferences, premedication strategies and protocols. Responses were analysed descriptively.

Results: Fifty-two responses were collected. Most respondents reported using MIST/LISA (87%), preferring it over INSURE or standard intubation. Practices varied widely in pre-intervention drug use and catheter choice. Surfcath was the most used device (71%). Only one-third consistently used pre-sedation.

Conclusion: MIST/LISA is widely implemented in Portuguese NICUs, but substantial variability exists. Standardised guidelines are needed to optimise clinical outcomes and reduce practice heterogeneity.

背景:微创/微创表面活性剂治疗/给药(MIST/LISA)越来越多地用于呼吸窘迫综合征早产儿表面活性剂的给药,旨在减少与有创方法相关的并发症。尽管越来越多的人采用它,但在临床实践中仍然存在差异。目的:评估MIST/LISA在葡萄牙新生儿重症监护病房(NICUs)的使用,并确定实践中的变化。方法:对新生儿医师进行全国性匿名问卷调查。调查探讨了表面活性剂给药技术、MIST/LISA的使用经验、设备偏好、用药前策略和方案。对反应进行描述性分析。结果:共收集到52份问卷。大多数受访者报告使用MIST/LISA(87%),比INSURE或标准插管更喜欢它。在干预前药物使用和导管选择方面,实践差异很大。表面导管是使用最多的设备(71%)。只有三分之一的患者持续使用预镇静。结论:MIST/LISA在葡萄牙nicu广泛实施,但存在很大的差异。需要标准化的指南来优化临床结果并减少实践的异质性。
{"title":"MIST/LISA in Portugal: National Survey of Clinical Practice.","authors":"Sílvia Duarte Costa, Sofia Silva Faria, Cláudia Ferraz, Ana Azevedo","doi":"10.1111/jpc.70247","DOIUrl":"https://doi.org/10.1111/jpc.70247","url":null,"abstract":"<p><strong>Background: </strong>Minimally/less invasive surfactant therapy/administration (MIST/LISA) is increasingly used for surfactant delivery in preterm infants with respiratory distress syndrome, aiming to reduce complications associated with invasive methods. Despite its growing adoption, variations in clinical practice remain.</p><p><strong>Objective: </strong>To evaluate MIST/LISA use in Portuguese neonatal intensive care units (NICUs) and identify variations in practice.</p><p><strong>Methods: </strong>A national, anonymous questionnaire was distributed to neonatologists. The survey explored surfactant administration techniques, experience with MIST/LISA, device preferences, premedication strategies and protocols. Responses were analysed descriptively.</p><p><strong>Results: </strong>Fifty-two responses were collected. Most respondents reported using MIST/LISA (87%), preferring it over INSURE or standard intubation. Practices varied widely in pre-intervention drug use and catheter choice. Surfcath was the most used device (71%). Only one-third consistently used pre-sedation.</p><p><strong>Conclusion: </strong>MIST/LISA is widely implemented in Portuguese NICUs, but substantial variability exists. Standardised guidelines are needed to optimise clinical outcomes and reduce practice heterogeneity.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634946","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}
引用次数: 0
Clearing the Air: Nitrous Oxide in Australian Paediatric Practice 净化空气:一氧化二氮在澳大利亚儿科实践。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-11-26 DOI: 10.1111/jpc.70244
Isabella Natale, Shefali Jani, Eduardo Alcaino, Louisa Swain, Justin Skowno

Aims

Nitrous oxide is a commonly used anaesthetic gas, particularly in paediatric practice; however there are growing concerns about its environmental impact as both a greenhouse gas and ozone-depleting substance. Whilst the nitrous oxide emissions related to healthcare are small compared to other industries, the majority of the nitrous oxide purchased by the healthcare sector is attributed to non-clinical purposes such as leaks from central supply systems. This review aims to contextualise the provision, use, and processing of nitrous oxide in Australian paediatric practice, understand issues associated with its use, and consider whether the clinical utility of nitrous oxide outweighs its environmental impact.

Methods

This review specifically addresses the breadth of paediatric practice including non-anaesthesia use through consideration of pre-existing primary and secondary research and specialist input from paediatric emergency, anaesthesia, and dental experts.

Results

Key findings include the need to measure the nitrous oxide ‘gap’ to determine emissions targets, strategies to facilitate the transition from centralised pipeline systems, and a consideration of alternative approaches throughout and beyond the nitrous lifecycle.

Conclusions

Ultimately, modifications to nitrous oxide supply, efficient clinical use, and viable alternatives to nitrous oxide where appropriate will be necessary to continue providing responsible, sustainable healthcare.

目的:氧化亚氮是一种常用的麻醉气体,特别是在儿科实践中;然而,作为一种温室气体和消耗臭氧的物质,人们越来越担心它对环境的影响。虽然与其他行业相比,与医疗保健相关的一氧化二氮排放量很小,但医疗保健部门购买的大部分一氧化二氮都是出于非临床目的,例如中央供应系统的泄漏。本综述旨在概述澳大利亚儿科实践中氧化亚氮的提供、使用和处理,了解与氧化亚氮使用相关的问题,并考虑氧化亚氮的临床应用是否超过其对环境的影响。方法:本综述通过考虑已有的初级和二级研究以及儿科急诊、麻醉和牙科专家的专家意见,专门讨论了儿科实践的广度,包括非麻醉使用。结果:主要发现包括需要测量一氧化二氮的“差距”,以确定排放目标,促进从集中式管道系统过渡的战略,以及考虑整个和超越氮氧化物生命周期的替代方法。结论:最终,修改一氧化二氮的供应,有效的临床使用,以及在适当情况下可行的一氧化二氮替代品将是继续提供负责任的、可持续的医疗保健所必需的。
{"title":"Clearing the Air: Nitrous Oxide in Australian Paediatric Practice","authors":"Isabella Natale,&nbsp;Shefali Jani,&nbsp;Eduardo Alcaino,&nbsp;Louisa Swain,&nbsp;Justin Skowno","doi":"10.1111/jpc.70244","DOIUrl":"10.1111/jpc.70244","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>Nitrous oxide is a commonly used anaesthetic gas, particularly in paediatric practice; however there are growing concerns about its environmental impact as both a greenhouse gas and ozone-depleting substance. Whilst the nitrous oxide emissions related to healthcare are small compared to other industries, the majority of the nitrous oxide purchased by the healthcare sector is attributed to non-clinical purposes such as leaks from central supply systems. This review aims to contextualise the provision, use, and processing of nitrous oxide in Australian paediatric practice, understand issues associated with its use, and consider whether the clinical utility of nitrous oxide outweighs its environmental impact.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This review specifically addresses the breadth of paediatric practice including non-anaesthesia use through consideration of pre-existing primary and secondary research and specialist input from paediatric emergency, anaesthesia, and dental experts.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Key findings include the need to measure the nitrous oxide ‘gap’ to determine emissions targets, strategies to facilitate the transition from centralised pipeline systems, and a consideration of alternative approaches throughout and beyond the nitrous lifecycle.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Ultimately, modifications to nitrous oxide supply, efficient clinical use, and viable alternatives to nitrous oxide where appropriate will be necessary to continue providing responsible, sustainable healthcare.</p>\u0000 </section>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"6-15"},"PeriodicalIF":1.4,"publicationDate":"2025-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145634906","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}
引用次数: 0
Early Infant Sleep Environments: An Observational Study of New Zealand Electronic Health Data. 婴儿早期睡眠环境:新西兰电子健康数据的观察性研究。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-11-25 DOI: 10.1111/jpc.70245
Lisa Daniels, Barry J Taylor, Jillian J Haszard, Justine Camp, Karen L Magrath, Zoe K Tipa, Victoria Bryant, Sheree J Gibb

Aim: New Zealand's post-neonatal mortality rate has remained steady at around 1.4 per 1000 live births since 2015, with Sudden Unexpected Death in Infancy (SUDI) accounting for approximately 60% of these deaths. Safe sleep guidelines are a key element of SUDI prevention but there is limited information on how these guidelines are applied in New Zealand homes. This work aims to examine safe sleep practices among New Zealand infants and explore variations based on demographic, infant and family characteristics.

Methods: This cross-sectional observational study used electronic health data for 45 969 New Zealand infants at approximately 6 weeks of age. Data came from 'Well Child/Tamariki Ora' visits conducted in 2023 by Whānau Āwhina Plunket, a provider of free health assessment and parenting support services for almost 80% of families in New Zealand. 'Safer sleep space' was defined as the exclusive use of recommended spaces (e.g., bassinet, cot, Pēpi-Pod or wahakura) and 'safer sleep position' referred to the supine (back) sleep position.

Results: Most infants were placed in safe sleep spaces (89% day, 93% night) and positions (95% day, 96% night). The prevalence of safe sleep practices varied between regions, ethnic groups and sociodemographic groups. Higher prevalences of safe sleep practices were seen with lower socioeconomic deprivation, adequate housing, non-smoking and employed caregivers, and caregivers with greater knowledge of health and support services.

Conclusions: There was a high prevalence of safe sleep practices with significant variations between sociodemographic, regional and ethnic groups, providing opportunities for targeted interventions to improve infant sleep safety.

目的:自2015年以来,新西兰的新生儿后死亡率一直稳定在每1000例活产1.4例左右,其中婴儿猝死(SUDI)约占这些死亡人数的60%。安全睡眠指南是预防SUDI的关键要素,但关于这些指南如何在新西兰家庭中应用的信息有限。这项工作旨在研究新西兰婴儿的安全睡眠习惯,并探索基于人口统计、婴儿和家庭特征的变化。方法:这项横断面观察性研究使用了45969名新西兰约6周龄婴儿的电子健康数据。数据来自Whānau Āwhina Plunket于2023年进行的“Well Child/Tamariki Ora”访问,该机构为新西兰近80%的家庭提供免费健康评估和育儿支持服务。“更安全的睡眠空间”被定义为只使用推荐的空间(例如,摇篮、婴儿床、Pēpi-Pod或wahakura),“更安全的睡眠姿势”是指仰卧(仰卧)的睡眠姿势。结果:大多数婴儿被放置在安全的睡眠空间(89%白天,93%夜间)和姿势(95%白天,96%夜间)。安全睡眠习惯的流行程度因地区、种族和社会人口群体而异。在社会经济剥夺程度较低、住房适足、不吸烟和雇用照顾者以及照顾者对保健和支助服务了解较多的情况下,安全睡眠习惯的流行率较高。结论:在不同的社会人口、地区和种族群体之间,安全睡眠习惯的患病率很高,存在显著差异,这为有针对性的干预措施提供了改善婴儿睡眠安全的机会。
{"title":"Early Infant Sleep Environments: An Observational Study of New Zealand Electronic Health Data.","authors":"Lisa Daniels, Barry J Taylor, Jillian J Haszard, Justine Camp, Karen L Magrath, Zoe K Tipa, Victoria Bryant, Sheree J Gibb","doi":"10.1111/jpc.70245","DOIUrl":"https://doi.org/10.1111/jpc.70245","url":null,"abstract":"<p><strong>Aim: </strong>New Zealand's post-neonatal mortality rate has remained steady at around 1.4 per 1000 live births since 2015, with Sudden Unexpected Death in Infancy (SUDI) accounting for approximately 60% of these deaths. Safe sleep guidelines are a key element of SUDI prevention but there is limited information on how these guidelines are applied in New Zealand homes. This work aims to examine safe sleep practices among New Zealand infants and explore variations based on demographic, infant and family characteristics.</p><p><strong>Methods: </strong>This cross-sectional observational study used electronic health data for 45 969 New Zealand infants at approximately 6 weeks of age. Data came from 'Well Child/Tamariki Ora' visits conducted in 2023 by Whānau Āwhina Plunket, a provider of free health assessment and parenting support services for almost 80% of families in New Zealand. 'Safer sleep space' was defined as the exclusive use of recommended spaces (e.g., bassinet, cot, Pēpi-Pod or wahakura) and 'safer sleep position' referred to the supine (back) sleep position.</p><p><strong>Results: </strong>Most infants were placed in safe sleep spaces (89% day, 93% night) and positions (95% day, 96% night). The prevalence of safe sleep practices varied between regions, ethnic groups and sociodemographic groups. Higher prevalences of safe sleep practices were seen with lower socioeconomic deprivation, adequate housing, non-smoking and employed caregivers, and caregivers with greater knowledge of health and support services.</p><p><strong>Conclusions: </strong>There was a high prevalence of safe sleep practices with significant variations between sociodemographic, regional and ethnic groups, providing opportunities for targeted interventions to improve infant sleep safety.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145604642","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}
引用次数: 0
Diagnosis and Management of Children With Tuberous Sclerosis Complex. 儿童结节性硬化症的诊断和治疗。
IF 1.4 4区 医学 Q2 PEDIATRICS Pub Date : 2025-11-21 DOI: 10.1111/jpc.70243
Celia Dewell, Denise L Chan, Vanessa Sarkozy, Eleanor Farley, Jacqueline Russell, David Mowat, Sean E Kennedy, Clara W T Chung

Tuberous sclerosis complex (TSC) is a multi-system autosomal dominant condition associated with disease-causing variants in either of the TSC1 or TSC2 genes. It can be associated with variable benign tumours (hamartomas) in different organs, most notably the brain, kidneys, skin and heart with different ages of onset. Affected children may have early-onset epilepsy, refractory epilepsy, varying degrees of developmental disability, ranging from normal cognition and learning difficulties to moderate-severe intellectual disability, neurodevelopmental disorders and mental health disorders. Multidisciplinary TSC-specific clinical care is recommended. A precision medicine (mechanistic target of rapamycin (mTOR) inhibitors) aimed at the early treatment of epilepsy and TSC-specific therapy for tumours can reduce the burden of the disease and improve clinical outcomes. We describe a multidisciplinary model of care for TSC, with an emphasis on the early detection and diagnosis of TSC and active surveillance/management of electrical seizures and clinical seizures. This review article will cover the following: clinical presentation and diagnosis of TSC, its genetic basis, the role of genetic counselling, pre-seizure surveillance of infants and TSC-specific (neurological, neurodevelopmental, renal, dermatological and pulmonary) management guidelines throughout life.

结节性硬化症(TSC)是一种多系统常染色体显性遗传病,与TSC1或TSC2基因的致病变异有关。它可能与不同器官的可变良性肿瘤(错构瘤)有关,最明显的是大脑、肾脏、皮肤和心脏,发病年龄不同。受影响的儿童可能患有早发性癫痫、难治性癫痫、不同程度的发育障碍,从正常的认知和学习困难到中重度智力残疾、神经发育障碍和精神健康障碍。推荐多学科tsc特异性临床护理。针对癫痫早期治疗的精准医学(雷帕霉素(mTOR)抑制剂的机制靶点)和针对肿瘤的tsc特异性治疗可以减轻疾病负担并改善临床结果。我们描述了一种多学科的TSC护理模式,重点是TSC的早期发现和诊断以及电性癫痫发作和临床癫痫发作的主动监测/管理。这篇综述文章将涵盖以下内容:TSC的临床表现和诊断,其遗传基础,遗传咨询的作用,婴儿癫痫前监测和TSC特异性(神经、神经发育、肾脏、皮肤和肺部)终生管理指南。
{"title":"Diagnosis and Management of Children With Tuberous Sclerosis Complex.","authors":"Celia Dewell, Denise L Chan, Vanessa Sarkozy, Eleanor Farley, Jacqueline Russell, David Mowat, Sean E Kennedy, Clara W T Chung","doi":"10.1111/jpc.70243","DOIUrl":"https://doi.org/10.1111/jpc.70243","url":null,"abstract":"<p><p>Tuberous sclerosis complex (TSC) is a multi-system autosomal dominant condition associated with disease-causing variants in either of the TSC1 or TSC2 genes. It can be associated with variable benign tumours (hamartomas) in different organs, most notably the brain, kidneys, skin and heart with different ages of onset. Affected children may have early-onset epilepsy, refractory epilepsy, varying degrees of developmental disability, ranging from normal cognition and learning difficulties to moderate-severe intellectual disability, neurodevelopmental disorders and mental health disorders. Multidisciplinary TSC-specific clinical care is recommended. A precision medicine (mechanistic target of rapamycin (mTOR) inhibitors) aimed at the early treatment of epilepsy and TSC-specific therapy for tumours can reduce the burden of the disease and improve clinical outcomes. We describe a multidisciplinary model of care for TSC, with an emphasis on the early detection and diagnosis of TSC and active surveillance/management of electrical seizures and clinical seizures. This review article will cover the following: clinical presentation and diagnosis of TSC, its genetic basis, the role of genetic counselling, pre-seizure surveillance of infants and TSC-specific (neurological, neurodevelopmental, renal, dermatological and pulmonary) management guidelines throughout life.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145573918","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}
引用次数: 0
期刊
Journal of paediatrics and child health
全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1