paediatric age

儿科年龄
  • 文章类型: Journal Article
    对0-12岁儿童眼发病的先天性病因的分析很有意义。因此,这项研究是在2021年1月至2023年12月在RLJalappa医院和研究中心进行的,该中心隶属于SriDevarajUrs医学院,Tamaka,Kolar,卡纳塔克邦,印度。在56名患者中,57%为男性,43%为女性儿童。31名(55%)的母亲属于20-30岁之间的年龄组,而24名(43%)在31-40岁之间,1名(2%)在41-50岁之间。在56名患者中,14(25%)个中有阳性家族史。其中34人(61%)有近亲婚姻。34人中有14位父母(41%)与二级血缘关系(兄弟/姐妹/祖父母/孙子)结婚,有20位父母(59%)与三级血缘关系(姨妈/叔叔/侄女/侄女/侄子/曾祖父/曾孙子女)结婚。31例(55%)出现双边参与。发现鼻泪管异常是最常见的(32%),其次是先天性内斜视(14%)。教育,意识,咨询有关血缘关系的风险和其他风险因素,如产妇年龄,感染,怀孕期间的药物,疫苗接种必须是医疗保健机构的常规做法。这可以显著降低发病率并防止失明。
    An analysis of the congenital etiologies of ocular morbidity in children of age 0-12 years is of interest. Hence, this study was conducted over a period of 2 years from Jan 2021- Dec 2023 at RL Jalappa Hospital and Research center that is attached to Sri Devaraj Urs Medical College, Tamaka, Kolar, Karnataka, India. Out of 56 patients, 57% were male and 43% were female children. 31 (55%) of mothers belonged to age group between 20-30 years and 24 (43%) between 31-40 years and 1(2%) between 41-50 years. Out of 56 patients, 14 (25%) of them had positive family history. 34 (61%) of them had consanguious marriage. 14 parents (41%) out of 34 are married to second degree consanguinity (brother/sister/grandparent/grandchild) and 20 (59%) belonged to third degree consanguinity (aunt/uncle/niece/nephew/great-grandparent/great-grandchild). Bilateral involvement was seen in 31 (55%). Nasolacrimal duct anomalies were found to be the most common (32%) followed by congenital esotropia (14%). Education, awareness, counseling about risks of consanguinity and other risk factors such as maternal age, infections, medications during pregnancy, vaccination must be a routine practice in healthcare set up. This can significantly reduce morbidity and prevent blindness.
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  • 文章类型: Case Reports
    称为软骨瘤的良性软骨病变通常出现在四肢的长骨中。这个病例报告,然而,提请注意锁骨内侧端附近的内生软骨瘤的罕见和不寻常的外观。因为不寻常的位置,诊断过程非常复杂,这给医生带来了挑战。我们提供临床,放射学和组织学结果,最终允许一个准确的诊断。这个例子强调了需要考虑良性病变的非典型位置,并强调了在意外的临床环境中彻底诊断方法的必要性。由于锁骨软骨瘤的发生是一种罕见的实体,有时会误导临床医生,医疗保健提供者必须足够警惕,以保证及时准确的诊断,以便及时干预。
    Benign cartilaginous lesions called enchondromas usually appear in the long bones of the limbs. This case report, however, draws attention to an uncommon and unusual appearance of enchondroma near the medial end of clavicle. Because of the unusual location, the diagnostic process was very complex, which presented a challengefor the physicians. We provide the clinical, radiological and histological results that finally allowed for an accurate diagnosis. This example highlights the need of taking into account atypical location for benign lesions and highlights the necessity of a thorough diagnostic approach in unexpected clinical settings. Since the occurrence of clavicular enchondromas is a rare entity and can at times mislead the clinician, healthcare providers must be vigilant enough to guarantee a prompt and accurate diagnosis for timely intervention.
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  • 文章类型: Journal Article
    目的:在先天性矫正的大动脉转位(CCTGA)中,右心室(RV)是全身性的。经常观察到房室传导阻滞(AVB)和收缩功能障碍。肺动脉下左心室(LV)的永久起搏可能会使RV功能障碍恶化。这项研究的目的是寻找在三维电解剖标测系统(3D-EAM)指导下的LV传导系统起搏(LVCSP)是否可以保留小儿CCTGAAVB患者的RV收缩功能。
    结果:对接受3D-EAM引导的LVCSP的CCTGA患者进行回顾性分析。三维起搏图引导导联植入至间隔部位,QRS波起搏较窄。心电图(ECG),超声心动图,和导线参数(阈值,传感,和阻抗)在基线(植入前)和1年随访时进行比较。通过3D射血分数(EF)评估右心室功能,分数面积变化(FAC),RV全局纵向应变(GLS)。数据报告为中位数(25-75百分位数)。7名15岁(9-17岁)的CCTGA患者,完整/高级AVB(4例先前心外膜起搏),接受3D引导LVCSP(5DDD,2VVIR)。大多数患者的基线超声心动图参数受损。无急性/慢性并发症发生。心室起搏>90%。在1年随访时,QRS持续时间与基线相比无显著变化;然而,与先前的心外膜起搏相比,QRS持续时间缩短。尽管心室阈值增加,但导线参数仍可接受。系统性RV功能得到保留:FAC和GLS明显改善,所有患者的RVEF均正常(>45%)。
    结论:三维EAM引导的LVCSP可在短期随访后保留儿童CCTGA和AVB患者的RV收缩功能。
    In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB.
    Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th-75th centiles). Seven CCTGA patients aged 15 (9-17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%).
    Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.
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  • 文章类型: Journal Article
    背景:股骨骨干骨折常见于儿科年龄组,因为道路交通事故的发生率有所增加。钛弹性钉系统(TENS)和钢板是儿科长骨骨折固定的常用方法。我们研究的目的是评估和比较使用锁定加压钢板和TENS治疗的小儿股骨骨干骨折的功能和放射学结果。
    方法:我们的研究包括59例诊断为股骨干骨折的患者。第一组28例患者接受了切开复位和锁定加压钢板内固定,第二组31例患者接受了切开复位/闭合复位和髓内TENS。所有术后患者在4岁时进行评估,八,10、12、16、20、24和36周。根据Flynn评分系统评估功能结果,并根据X射线上的骨折愈合评估放射学愈合。
    结果:我们使用Flynn评分系统分析了我们的数据。在第一组中,28例采用锁定加压钢板治疗,25(89%)是优秀的,两名(7.5%)令人满意,其中一人(3.5%)较差。在第二组中,在31例接受髓内TENS治疗的病例中,26(83.8%)是优秀的,5(16.2%)是令人满意的。在我们的研究中,第一组的平均愈合时间为11.4周,第二组为14.41周。两组骨折愈合率为100%。
    结论:在我们的研究中,我们注意到,股骨轴的联合是早期使用锁定加压板。在TENS中,术中失血较少,术后疤痕极小,减少软组织损伤。此外,与锁定加压钢板相比,植入物的移除更容易。
    BACKGROUND:  Diaphyseal femur fractures are commonly seen in the paediatric age group as there is an increase in the incidence of road traffic accidents. Titanium elastic nailing system (TENS) and plating are the common methods used for paediatric long bone fracture fixation. The purpose of our study was to evaluate and compare functional and radiological outcomes of paediatric femur diaphyseal fractures treated with locking compression plates and with TENS.
    METHODS:  Our study included 59 patients diagnosed with femur shaft fracture. Twenty-eight patients included in group one underwent open reduction and internal fixation with locking compression plates and 31 patients in group two underwent open reduction/closed reduction with intramedullary TENS. All post-operation patients were evaluated at four, eight, 10, 12, 16, 20, 24, and 36 weeks. The functional outcome was assessed based on the Flynn scoring system and radiological union based on fracture union on X-ray.
    RESULTS: We analyzed our data using the Flynn scoring system. In group one, out of 28 cases treated with locking compression plates, 25 (89%) were excellent, two (7.5%) were satisfactory, and one (3.5%) was poor. In group two, out of 31 cases treated with intramedullary TENS, 26 (83.8%) were excellent and five (16.2%) were satisfactory. In our study, the average union time in group one was 11.4 weeks and in group two was 14.41 weeks. Fracture union was 100% in both groups.
    CONCLUSIONS: In our study, we noted that the union of the femur shaft was early with the use of locking compression plates. In TENS, there was less intraoperative blood loss, very minimal postoperative scar, and less soft tissue damage. Also, implant removal was easier compared to locking compression plates.
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  • 文章类型: Journal Article
    目的:皮下植入心脏除颤器(S-ICD)越来越多地用于预防年轻患者的心源性猝死。这项研究是为了深入了解S-ICD的适应症,可能的并发症,以及它们的预测因素和后续结果。
    结果:多中心,观察,回顾性,非随机化,关于年轻先天性心脏病(CHD)患者S-ICD结局的护理标准注册表,遗传性心律失常(IAs),特发性心室纤颤(IVF),和心肌病(CMP)。注册了人体测量学以及植入技术,中期器械相关并发症,以及适当/不适当电击(IAS)的发生率。数据报告为中值(四分位间距)或平均值±标准偏差。81名患者(47%的CMPs,20%CHD,21%IVF,和12%IA),15岁(14-17岁),体重指数(BMI)21.8±3.8kg/m2,接受了S-ICD植入(59%的一级预防)。81%的患者采用双切口技术,59%的患者采用皮下口袋。冲击和条件区编程为250(200-250)和210(180-240)b.p.m.,分别。术中无并发症发生。随访19(6~35)个月:无除颤失败,17%的患者接受了适当的电击,13%的患者接受了IAS(室上性心动过速40%,T波过感知40%,和非心脏过度感知20%)。重新编程,适当的药物治疗,和手术翻修避免了进一步的IAS。需要手术翻修的并发症发生在9%的患者中,三切口手术患者的风险较高[风险比(HR)4.3,95%置信区间(95%CI)0.5-34,P=0.038]和BMI<20(HR5.1,95%CI1-24,P=0.031)。
    结论:这项多中心欧洲儿科注册在年轻患者中显示出良好的S-ICD疗效和安全性。较新的植入技术和BMI>20显示出更好的结果。
    Subcutaneous-implantable cardiac defibrillators (S-ICDs) are used increasingly to prevent sudden cardiac death in young patients. This study was set up to gain insight in the indications for S-ICD, possible complications, and their predictors and follow-up results.
    A multicentre, observational, retrospective, non-randomized, standard-of-care registry on S-ICD outcome in young patients with congenital heart diseases (CHDs), inherited arrhythmias (IAs), idiopathic ventricular fibrillation (IVF), and cardiomyopathies (CMPs). Anthropometry was registered as well as implantation technique, mid-term device-related complications, and incidence of appropriate/inappropriate shocks (IASs). Data are reported as median (interquartile range) or mean ± standard deviation. Eighty-one patients (47% CMPs, 20% CHD, 21% IVF, and 12% IA), aged 15 (14-17) years, with body mass index (BMI) 21.8 ± 3.8 kg/m2, underwent S-ICD implantation (primary prevention in 59%). This was performed with two-incision technique in 81% and with a subcutaneous pocket in 59%. Shock and conditional zones were programmed at 250 (200-250) and 210 (180-240) b.p.m., respectively. No intraoperative complications occurred. Follow up was 19 (6-35) months: no defibrillation failure occurred, 17% of patients received appropriate shocks, 13% of patients received IAS (supraventricular tachycardias 40%, T-wave oversensing 40%, and non-cardiac oversensing 20%). Reprogramming, proper drug therapy, and surgical revision avoided further IAS. Complications requiring surgical revision occurred in 9% of patients, with higher risks in patients with three-incision procedures [hazard ratio (HR) 4.3, 95% confidence interval (95% CI) 0.5-34, P = 0.038] and BMI < 20 (HR 5.1, 95% CI 1-24, P = 0.031).
    This multicentre European paediatric registry showed good S-ICD efficacy and safety in young patients. Newer implantation techniques and BMI > 20 showed better outcome.
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  • 文章类型: Journal Article
    儿童肛门直肠畸形(ARM)的治疗和长期临床结果一直是儿科外科医生关注的焦点。这项研究旨在报告我们在儿童ARM长期随访方面的经验。
    我们招募了1999年至2019年接受治疗的患者,并建立了选择标准以选择合适的受试者。使用经过验证的问卷来确定长期生活质量结果。
    在研究期间接受治疗的48名患者中,28人参加了这项研究。在后者中,超过35%的人至少有一次长期并发症,90%以上的人有良好的生活方式。在使用医疗设备的患者中,超过95%的患者实现了尿失禁和大便失禁。
    这项研究旨在提出新的概念;考虑到ARM患者生活的各个方面,从学校生活到性,同时评估粪便和尿失禁。这对于提高参与这些患者管理的不同专家的技能至关重要,以及实施可以改善术后功能的策略。尤其是,这也将有助于改善医生之间的沟通,以确保这些儿童充分过渡到成人生活。
    UNASSIGNED: The treatment and long term clinical outcomes of anorectal malformations (ARM) in children have always been the focus of pediatric surgeons. This study aimed at reporting our experience as far as long-term follow-up of ARM in children is concern.
    UNASSIGNED: We enrolled patients treated between 1999 and 2019, and established selection criteria to choose appropriate subjects. A validated questionnaire was used to determine long-term quality of life outcomes.
    UNASSIGNED: Out of a total of 48 patients treated within the study period, 28 were enrolled in this study. Among the latter, more than 35% had at least one long-time complication, and more than 90% had a good lifestyle. Urinary and fecal continence was achieved in more than 95% of the patients using medical devices.
    UNASSIGNED: This study aimed to bring up new concepts; taking into consideration all aspects of life in patients with ARM, from school life to sexuality, while evaluating fecal and urinary continence. This is essential for the improvement of the skills of the different specialists involved in the management of these patients, and for the implementation of strategies that can improve postoperative function. Most especially, it will also help improve communication between doctors to ensure an adequate transition of these children into adult life.
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  • 文章类型: English Abstract
    未经评估:这项研究的目的是确定在2020年期间,是否与COVID-19大流行相吻合,与前2年相比,儿童糖尿病的发病率有所增加。这也是为了找出封锁和在卫生系统中提供面对面护理的困难是否导致儿童在诊断时表现出更严重的症状。
    UNASSIGNED:塔拉戈纳省的回顾性观察多中心研究,其中数据是从2020年15岁以下患者的1型糖尿病新诊断中收集的,并与2018年和2019年进行比较。
    UNASSIGNED:2020年1型糖尿病新诊断病例为37例,而2019年和2018年分别为23例和29例。发病年龄中位数为9岁,54%的男性。10至14岁儿童的新诊断有所增加,在0到4岁的范围内减少。2020年,马格里布地区家庭患者组的发病率从2019年的每100,000人口/年52.2例(c/105p-y)上升到2020年的135.8例。与前一年相比,2020年显示酮症酸中毒在发病时显著减少。所有患者在入院期间均未被诊断为COVID-19。
    未经批准:在与COVID-19大流行一致的2020年,儿科新诊断1型糖尿病的数量有所增加.与预期相反,发病时酮症酸中毒的比例降低并没有使临床表现恶化.这些数据表明,尽管不同医疗机构的出勤率在2020年大幅下降,但以虚拟咨询为代价,卫生系统和家庭能够早期发现疾病的症状。
    UNASSIGNED: The aim of this study is to determine whether during the year 2020, coinciding with the COVID-19 pandemic, there has been an increase in the incidence of diabetes mellitus in children compared to the previous 2 years. It is also to find out if lockdowns and the difficulty providing face-to-face care in the health system have led to children showing more severe symptoms at the time of diagnosis.
    UNASSIGNED: Retrospective observational multicenter study of the province of Tarragona where data is collected from new diagnoses of type 1 diabetes mellitus in patients under the age of 15 during the year 2020 and compared with years 2018 and 2019.
    UNASSIGNED: The number of new diagnoses of type 1 diabetes during 2020 was 37 cases compared to 2019 and 2018 which was 23 and 29 respectively. The median age at onset was 9 years, 54% males. There was an increase in new diagnoses in the range of 10 to14-year-olds, with a decrease in the range of 0 to 4 year-olds. In 2020, the incidence in the group of patients with families from the Maghreb area rose from 52.2 cases per 100,000 population/year (c/105 p-y) in 2019 to 135.8 in 2020. Compared to the previous year, 2020 showed a significant decrease of ketoacidosis at the onset. None of the patients was diagnosed with COVID-19 during admission.
    UNASSIGNED: During the year 2020 concurring with the COVID-19 pandemic, there was an increase in the number of new diagnoses of type 1 diabetes mellitus in pediatrics. Contrary to expectations, the presentation did not worsen by decreasing the proportions of ketoacidosis at onset. This data would suggest that, although attendance in the different health facilities dropped drastically during the year 2020 at the expense of virtual consultations, health systems and families were able to detect the symptoms of the disease early.
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  • 文章类型: Case Reports
    罕见继发于颈部肿块的小儿气管偏离的急性表现。差异很大,孩子可能会受到损害。稳定和复苏儿童是主要目标。这个病例描述了一个有神经发育迟缓和进行性吞咽困难病史的6岁男孩,具有急性软食推注嵌塞史,明显的气管偏移和一个坚固的颈部肿块。我们讨论演示文稿的诊断困难,在英国一所大学医院的背景下,这一罕见病例的处理和管理,现场没有儿科重症监护。
    Acute presentations of paediatric tracheal deviation secondary to neck masses are rare. The differentials are broad and the child may be compromised. Stabilising and resuscitating the child are the primary aims. This case describes a six-year-old boy with a history of neurodevelopmental delay and progressive dysphagia, presenting with an acute history of soft food bolus impaction, significant tracheal deviation and a firm neck lump. We discuss the diagnostic difficulties of the presentation, the work-up and the management of this rare case in the setting of a university hospital in the United Kingdom, with no paediatric intensive care on site.
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  • 文章类型: Observational Study
    目的:SARS-CoV-2mRNA疫苗在患有儿童期风湿性疾病(cRD)的青少年和年轻人(AYA)中的免疫原性未知。我们旨在用cRD评估我们的AYA疫苗的体液免疫原性和安全性。
    方法:采用159AYA(50.3%为女性,70.4%为中国人)进行单中心观察研究。在第一次和第二次接种后2-3和4-6周通过cPass™SARS-CoV-2中和抗体测定评估体液免疫原性。≥30%的抑制信号定义了SARS-CoV-2中和抗体阳性检测的截止值。在第二次疫苗接种后6周内评估疫苗安全性和疾病活动性。
    结果:159例患者中有64.9%和99.1%(中位年龄16.9岁,IQR:14.7-19.5)在第一次和第二次接种疫苗后出现了积极的SARS-CoV-2中和反应,分别。大多数患者(89.8%)在第二次疫苗接种后具有≥90%的抑制信号。甲氨蝶呤和霉酚酸酯增加了首次接种后与cPass阴性中和反应相关的风险。每次接种后保持两种药物不会影响免疫原性。没有症状性COVID-19感染。局部反应仍然是最常见的(23.3-25.2%)不良事件,无严重并发症。第一次和第二次接种疫苗后,有两名和七名患者爆发,分别。12-18岁队列的亚组分析显示疫苗效力没有任何差异,反应差和一般安全性的预测因素,但是疾病的比例更高。
    结论:SARS-CoV-2mRNA疫苗在两剂量方案后在几乎所有的AYA和cRD中都是有效的,没有严重的不良事件。在第二次mRNA疫苗剂量后观察到的疾病爆发率为4.4%。
    Immunogenicity to the SARS-CoV-2 mRNA vaccines in adolescents and young adults (AYA) with childhood-onset rheumatic diseases (cRD) is unknown. We aimed to evaluate the humoral immunogenicity and safety of the vaccines in our AYA with cRD.
    A monocentric observational study with 159 AYA (50.3% female and 70.4% Chinese). Humoral immunogenicity was assessed at 2-3 and 4-6 weeks following first and second vaccination by cPass™ SARS-CoV-2 Neutralization Antibody Assay. Inhibition signal of ≥30% defined the cut-off for positive detection of the SARS-CoV-2 neutralizing antibodies. Vaccine safety and disease activity were assessed within 6 weeks after second vaccination.
    A total of 64.9% and 99.1% of 159 patients (median age: 16.9, IQR: 14.7-19.5) mounted positive SARS-CoV-2 neutralizing responses after first and second vaccination, respectively. Most patients (89.8%) had ≥90% inhibition signal after second vaccination. Methotrexate and mycophenolate mofetil increased the risk associated with negative cPass neutralization responses following the first vaccination. Holding both medications after each vaccination did not affect immunogenicity. There was no symptomatic COVID-19 infection. Local reaction remained the most common (23.3-25.2%) adverse event, without serious complication. Two and seven patients flared following the first and second vaccination, respectively. Subgroup analyses of the 12-18-year-old cohort did not show any differences in vaccine efficacy, predictors of poor response and general safety, but higher proportion of disease flares.
    SARS-CoV-2 mRNA vaccines were efficacious after the two-dose regimen in almost all AYA with cRD without serious adverse event. The rate of disease flare observed is 4.4% after the second mRNA vaccine dose.
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  • 文章类型: Journal Article
    为了评估在一组10例接受过颅内脑电图(iEEG)的小儿癫痫手术患者中自动检测脑磁图(MEG)记录中的高频振荡(HFO)的可行性。
    使用波束形成源分析方法构建虚拟传感器,并应用自动算法检测HFO(80-250Hz)。我们评估了MEG发现与iEEGHFO来源的一致性,临床定义的癫痫发作区(SOZ),切除的大脑结构的位置,以及术后结果。
    在8/9患者中,MEGHFOs和iEEGHFOs的来源与SOZ之间具有良好的一致性。相对于MEGt(71)=2.85,p<0.05,iEEG中检测到更多的HFO。结果良好和不良的患者MEGHFOs来源与切除面积之间有很好的一致性。然而,在预后较差的患者中,也在切除区域之外检测到HFOs.
    我们的研究结果证明了在儿科患者的MEG记录中自动检测HFO的可行性,并确认结果与侵入性记录的兼容性。
    这种方法为HFOs的非侵入性检测提供了支持,以帮助进行手术计划,并可能减少对侵入性监测的需求。这与儿科患者有关。
    To assess the feasibility of automatically detecting high frequency oscillations (HFOs) in magnetoencephalography (MEG) recordings in a group of ten paediatric epilepsy surgery patients who had undergone intracranial electroencephalography (iEEG).
    A beamforming source-analysis method was used to construct virtual sensors and an automatic algorithm was applied to detect HFOs (80-250 Hz). We evaluated the concordance of MEG findings with the sources of iEEG HFOs, the clinically defined seizure onset zone (SOZ), the location of resected brain structures, and with post-operative outcome.
    In 8/9 patients there was good concordance between the sources of MEG HFOs and iEEG HFOs and the SOZ. Significantly more HFOs were detected in iEEG relative to MEG t(71) = 2.85, p < .05. There was good concordance between sources of MEG HFOs and the resected area in patients with good and poor outcome, however HFOs were also detected outside of the resected area in patients with poor outcome.
    Our findings demonstrate the feasibility of automatically detecting HFOs non-invasively in MEG recordings in paediatric patients, and confirm compatibility of results with invasive recordings.
    This approach provides support for the non-invasive detection of HFOs to aid surgical planning and potentially reduce the need for invasive monitoring, which is pertinent to paediatric patients.
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