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Erythema multiforme major/Stevens-Johnson syndrome: a diagnostic challenge 重度多形红斑/史蒂文斯-约翰逊综合征:诊断难题。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.10.005
Rita Lages Pereira , Mariana Oliveira Pereira , Carmo Ferreira , Ivo Neves
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引用次数: 0
Coping with death: bereavement support for paediatricians 应对死亡:为儿科医生提供丧亲支持。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.07.019
Manuel Gijón Mediavilla , Pablo Rojo Conejo , Eduardo López Fernández , Sara Losantos Ucha , Sylvia Belda Hofheinz
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引用次数: 0
Recommendations of the Spanish Society of Neonatology for the prevention of severe respiratory syncytial virus infections with nirsevimab, for the 2024–2025 season 西班牙新生儿学会关于使用 nirsevimab 预防严重呼吸道合胞病毒感染的建议(2024-2025 年)。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.09.010
Manuel Sánchez Luna , M. Luz Couce Pico , Belén Fernández Colomer , en representación de la Junta Directiva de la Sociedad Española de Neonatología (SENEO) Comisión de Estándares de SENEO y Comisión de Infecciones de SENEO
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引用次数: 0
Primary adrenal insufficiency: case study IN 5 tertiary hospitals 原发性肾上腺功能不全:5 家三级医院的病例研究。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.10.008
María Teresa Ovejero García , Blanca Sáez Gallego , Ana Coral Barreda Bonís , Jesús Domínguez Riscart , María Beatriz Garnier Rodríguez , Ruth Molina Suárez , Antonio De Arriba Muñoz

Introduction

Primary adrenal insufficiency (PAI) in children is a rare condition characterized by deficient production of glucocorticoids and/or mineralocorticoids. The clinical manifestations are nonspecific and insidious. Providers need to know about this disorder to be able to make an early diagnosis, as appropriate management can be life-saving.

Methods

We conducted a multicentre retrospective study including every patient aged less than 18 years given a diagnosis of PAI in the last 30 years at 5 Spanish hospitals.

Objectives

The objective was to determine the aetiologies, signs, symptoms and laboratory findings of PAI in the paediatric age group.

Results

Twenty nine patients received a diagnosis of PAI at a median age of 5.6 years. An aetiological diagnosis was established in 23 patients (79.3%): X-linked adrenoleukodystrophy in 8 (27.6%), autoimmune adrenalitis in 6 (20.7%), X-linked adrenal hypoplasia congenita in 4 (13.8%), adrenocorticotropic hormone (ACTH) resistance syndrome in 2 (6.9%), Pearson syndrome in 2 (6.9%) and Allgrove syndrome in 1 (3.4%). In the remaining 6 patients, no clear aetiology was identified. Sixteen patients (55.2%) had onset with an adrenal crisis. Twenty patients (69%) needed combination therapy (hydrocortisone and fludrocortisone).

Conclusions

Asthenia, hyperpigmentation and hyponatraemia were the most prevalent sign, symptom and electrolyte abnormality at onset of PAI, although their absence does not rule out this disease. The elevation of ACTH persists despite adequate glucocorticoid replacement therapy.
简介:儿童原发性肾上腺功能不全(PAI)是一种以糖皮质激素和/或矿物质皮质激素分泌不足为特征的罕见疾病。其临床表现为非特异性和隐匿性。医务人员需要了解这种疾病,以便及早做出诊断,因为适当的治疗可以挽救生命:我们进行了一项多中心回顾性研究,包括西班牙 5 家医院在过去 30 年中诊断出的所有 PAI 患者,患者年龄均小于 18 岁:目的:确定儿科 PAI 的病因、体征、症状和实验室检查结果:结果:29 名患者被诊断为 PAI,中位年龄为 5.6 岁。23名患者(79.3%)的病因诊断已经确定:8例(27.6%)为X连锁肾上腺白质营养不良,6例(20.7%)为自身免疫性肾上腺炎,4例(13.8%)为X连锁先天性肾上腺发育不全,2例(6.9%)为促肾上腺皮质激素(ACTH)抵抗综合征,2例(6.9%)为皮尔逊综合征,1例(3.4%)为阿尔格罗夫综合征。其余 6 名患者没有明确的病因。16名患者(55.2%)发病时出现肾上腺危象。20名患者(69%)需要联合治疗(氢化可的松和氟氢可的松):结论:气喘、色素沉着和低钠血症是 PAI 发病时最常见的体征、症状和电解质异常,尽管没有这些症状并不能排除该病。尽管进行了充分的糖皮质激素替代治疗,但促肾上腺皮质激素的升高仍然存在。
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引用次数: 0
Trichothiodystrophy, playing with optical microscope 毛细血管营养不良症,玩光学显微镜。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.10.009
Elisabeth Gómez Moyano, Jose Antonio Llamas Carmona, Irene López Riquelme, Leandro Martínez Pilar
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引用次数: 0
Risk factors and profiles of reattempted suicide in children aged less than 12 years 12 岁以下儿童再次企图自杀的风险因素和特征。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.10.002
César D. González , Carlos Fabian Martínez-Cárdenas

Objective

Suicide attempt (SA) repetition is considered one of the main risk factors for completed suicide. In spite of this, there is no previous research on this variable in children. The objective was to determine the factors and profiles associated with reattempted suicide in children aged less than 12 years.

Methods

Cross-sectional study that included 120 reports (event code INS.356) of SA in children aged less than 12 years between 2018 and 2023 in the SIVIGILA surveillance system of Colombia. We carried out a bivariate logistic regression analysis to generate the best fitting risk factor model and a multiple correspondence analysis (MCA) to establish the possible SA recurrence profiles using the SPSS software, version 26.

Results

Of the 120 cases analysed, 43 (35.8%) were reports of reattempted suicide. The best model for predicting risk factors for repeated SA included child maltreatment (OR, 6.22; P < .05), persistent suicidal ideation (PSI) (OR, 30.91; P < .001), a history of violence or sexual abuse (OR, 13.10; P < .05) and the use of sharp objects (OR, 46.45; P < .001). The MCA identified 3 profiles: “first SA” with poisoning as the attempt method and in absence of PSI, violence and abuse; “one previous SA” with the use of sharp objects and presence of PSI ; “two or more previous SAs” with a history of violence or sexual abuse, child maltreatment and hanging as the method.

Conclusion

Our findings demonstrate the impact of early life adversity (ELA) on children's mental health, so it is necessary to detect and prevent these types of abuse in order to reduce childhood suicide.
目的:自杀未遂(SA)重复被认为是导致完全自杀的主要风险因素之一。尽管如此,以前却没有针对儿童这一变量的研究。本研究旨在确定与 12 岁以下儿童再次自杀未遂相关的因素和特征:横断面研究纳入了哥伦比亚 SIVIGILA 监控系统中 2018 年至 2023 年间关于 12 岁以下儿童 SA 的 120 份报告(事件代码 INS.356)。我们使用 SPSS 软件 26 版进行了双变量逻辑回归分析,以生成最佳拟合风险因素模型,并进行了多重对应分析(MCA),以确定可能的 SA 复发情况:在分析的 120 个病例中,有 43 例(35.8%)是自杀未遂报告。预测重复自杀风险因素的最佳模型包括儿童虐待(OR,6.22;P 结论:我们的研究结果表明,早年生活对自杀有影响:我们的研究结果表明了早期生活逆境(ELA)对儿童心理健康的影响,因此有必要发现并预防这类虐待行为,以减少儿童自杀。
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引用次数: 0
A holistic perspective of the comorbidities in childhood obesity 从整体角度看儿童肥胖症的并发症。
Pub Date : 2024-11-01 DOI: 10.1016/j.anpede.2024.10.001
Rosa Herrera , Empar Lurbe
Childhood obesity is associated with comorbidities that affect almost all body systems, including, among others, the endocrine, gastrointestinal, pulmonary, cardiovascular and musculoskeletal systems, as well as medical and surgical procedures that may be required due to different clinical situations. The objective of this article is to describe the classic and emerging comorbidities associated with obesity and the complications of procedures that involve invasive manoeuvres. Although some of the problems associated with obesity during childhood are widely known, such as musculoskeletal and cutaneous disorders or apnoea–hypopnoea syndrome, others, such as changes in kidney function, non-alcoholic fatty liver and cardiometabolic risk, have received less attention due to their insidious development, as they may not manifest until adulthood. In contrast, there is another group of comorbidities that may have a greater impact due to their frequency and consequences, which are psychosocial problems. Finally, in the context of invasive medico-surgical interventions, obesity can complicate airway management. The recognition of these pathologies in association with childhood obesity is of vital importance not only in childhood but also due to their ramifications in adulthood.
儿童肥胖症与影响几乎所有身体系统的合并症有关,其中包括内分泌、胃肠道、肺部、心血管和肌肉骨骼系统,以及因不同临床情况而可能需要进行的内科和外科手术。本文旨在介绍与肥胖相关的传统和新出现的合并症,以及涉及侵入性操作的手术并发症。虽然与儿童期肥胖相关的一些问题已广为人知,如肌肉骨骼和皮肤疾病或呼吸暂停-低通气综合征,但其他问题,如肾功能变化、非酒精性脂肪肝和心血管代谢风险,由于其发展隐匿,可能要到成年后才会显现出来,因此受到的关注较少。与此相反,另一类合并症由于其发生频率和后果可能会产生更大的影响,这就是社会心理问题。最后,在进行侵入性医疗手术干预时,肥胖会使气道管理复杂化。认识这些与儿童肥胖症相关的病症不仅对儿童时期至关重要,而且对成年后的影响也很大。
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引用次数: 0
Vascular anomalies in childhood. Review and update 儿童血管异常。回顾与更新。
Pub Date : 2024-10-01 DOI: 10.1016/j.anpede.2024.09.009
Juan Carlos López Gutiérrez , Jesús Pozo Losada , Manuel Gómez Tellado
Vascular anomalies are changes in vascularization that usually appear in the foetal stage, at birth or in early childhood. They can cause chronic pain, motor impairment, cosmetic changes or coagulopathy and may be fatal in some cases, but in every case they have a negative impact on the quality of life of the child and the family. Up to 150 different subtypes have been described. They can involve arteries, capillaries, veins, lymphatic vessels or a combination thereof. They may be associated with additional malformations and frequently cause musculoskeletal and soft tissue hypertrophy or hypotrophy. They can develop anywhere in the body, invade any tissue and affect the function of various organs. The prevalence of the different subtypes varies greatly, from 1/20 to 1/1 000 000. Subtypes considered rare diseases (incidence <1/2000) continue to affect more than 500 000 people in the European Union.
Differentiating between vascular tumours and vascular malformations is critical, especially in paediatric patients. They are completely different diseases, although they are often grouped under the umbrella term of vascular anomalies.
The diagnostic algorithm used in the clinical evaluation of vascular anomalies should be based on a thorough history-taking and detailed physical examination.
Future knowledge in this field will be based above all on genetic findings and therapeutic innovations. New molecules and their indications are being explored with the aim of reducing the aggressiveness of previous treatments and increasing the life expectancy and quality of life of patients who do not respond to conventional treatments.
血管异常是指血管发生变化,通常出现在胎儿期、出生时或幼儿期。血管畸形可导致慢性疼痛、运动障碍、外观变化或凝血功能障碍,在某些情况下可能致命,但在任何情况下都会对儿童和家庭的生活质量造成负面影响。已描述的亚型多达 150 种。它们可能涉及动脉、毛细血管、静脉、淋巴管或它们的组合。它们可能伴有其他畸形,并经常导致肌肉骨骼和软组织肥大或萎缩。它们可在身体任何部位发病,侵犯任何组织,并影响各种器官的功能。不同亚型的发病率差别很大,从 1/20 到 1/1 000 000。被视为罕见病的亚型(发病率
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引用次数: 0
Role of fractional exhaled nitric oxide in patients with sickle cell disease 镰状细胞病患者呼出一氧化氮分数的作用。
Pub Date : 2024-10-01 DOI: 10.1016/j.anpede.2024.09.003
Agustín Muñoz-Cutillas , Sara Bellón-Alonso , Eduardo Bardón-Cancho , Santiago Rodríguez-Tubío-Dapena , Lucía Díez-Llamazares , Rosa Rodríguez-Fernández , Juan Luis Rodríguez-Cimadevilla

Introduction

Patients with sickle cell disease exhibit different patterns in pulmonary function tests. In particular, there is little evidence on the fractional exhaled nitric oxide (FeNO) test, and its value ranges and its interpretation in these patients have been under debate in recent years.

Methods

We conduced a cross-sectional, observational and descriptive study between November 2021 and January 2023 including patients aged 6–18 years with sickle cell disease able to perform the FeNO test. We applied the GLI-2012 reference values and the ERS/ATS standards. We defined statistical significance as P < 0.05.

Results

The sample included 43 patients with a median age of 12 years (IQR, 10−15). We did not find an association between significantly elevated FeNO (≥25 ppb) and the diagnosis of asthma (P = 0.37), an obstructive pattern in spirometry (P = 0.67), a positive bronchodilator test (P = 0.53), clinical bronchial hyperreactivity in the context of cold or flu-like symptoms (P = 0.48), cough with exercise (P = 0.42) or nocturnal cough (P = 1.0), but found an association with peripheral eosinophilia (P < 0.01).

Conclusions

We found no association between FeNO values and the classic features of asthma (clinical or spirometric) in patients with sickle cell disease. Therefore, airway inflammation mechanisms are probably different in these patients.
简介镰状细胞病患者的肺功能测试表现出不同的模式。特别是,关于分数呼出一氧化氮(FeNO)测试的证据很少,近年来对其在这些患者中的价值范围和解释一直存在争议:我们在 2021 年 11 月至 2023 年 1 月期间开展了一项横断面、观察性和描述性研究,研究对象包括能够进行 FeNO 测试的 6-18 岁镰状细胞病患者。我们采用了 GLI-2012 参考值和 ERS/ATS 标准。我们将 P < 0.05 定义为统计学意义:样本包括 43 名患者,中位年龄为 12 岁(IQR,10-15 岁)。我们没有发现明显升高的 FeNO(≥25 ppb)与哮喘诊断(P = 0.37)、肺活量测定的阻塞模式(P = 0.67)、支气管扩张剂试验阳性(P = 0.53)、感冒或流感样症状背景下的临床支气管高反应性(P = 0.48)、运动时咳嗽(P = 0.42)或夜间咳嗽(P = 1.0),但发现与外周嗜酸性粒细胞增多有关(P < 0.01):我们发现镰状细胞病患者的 FeNO 值与哮喘的典型特征(临床或肺活量)之间没有关联。因此,这些患者的气道炎症机制可能有所不同。
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引用次数: 0
Home teleassistance using augmented reality glasses: A proof of concept 使用增强现实眼镜的家庭远程协助:概念验证
Pub Date : 2024-10-01 DOI: 10.1016/j.anpede.2024.09.006
Carmen Agra-Tuñas , Silvia Aranda-García , Graciela Gómez-Silva , Verónica Izquierdo , Antonio Rodríguez-Núñez
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引用次数: 0
期刊
Anales de pediatria
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