Anales de pediatria最新文献

{"title":"Afrontando la muerte: reuniones de duelo para profesionales en el cuidado pediátrico","authors":"Manuel Gijón Mediavilla , Pablo Rojo Conejo , Eduardo López Fernández , Sara Losantos Ucha , Sylvia Belda Hofheinz","doi":"10.1016/j.anpedi.2024.07.002","DOIUrl":"10.1016/j.anpedi.2024.07.002","url":null,"abstract":"","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 351-352"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142572496","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":"Discinesia ciliar primaria: diagnóstico adicional en una paciente con síndrome cardioespondilocarpofacial","authors":"Patricia Morte Coscolín , Sandra Rovira Amigo , Carlos Martín de Vicente","doi":"10.1016/j.anpedi.2024.08.002","DOIUrl":"10.1016/j.anpedi.2024.08.002","url":null,"abstract":"","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 357-359"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142572539","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":"Estado nutricional en errores innatos del metabolismo de las proteínas. Estudio caso-control","authors":"Isidro Robredo García ,&nbsp;Paula Grattarola ,&nbsp;Patricia Correcher Medina ,&nbsp;Fátima Abu-Sharif Bohigas ,&nbsp;Verónica Vélez García ,&nbsp;Isidro Vitoria Miñana ,&nbsp;Cecilia Martínez Costa","doi":"10.1016/j.anpedi.2024.08.005","DOIUrl":"10.1016/j.anpedi.2024.08.005","url":null,"abstract":"<div><h3>Introduction</h3><div>Patients with protein metabolism disorders (PMDs) require a diet with strict protein control that can affect their growth and development. The aim of the study was to assess nutritional status and growth in patients with PMDs undergoing dietary treatment.</div></div><div><h3>Patients and methods</h3><div>Prospective observational case-control study in 63 patients with PMDs and 63 healthy controls matched for age and sex. We collected data for anthropometric variables (weight, height, BMI, tricipital and subscapular skinfolds, arm and waist circumference) and calculated the corresponding <em>z</em> scores. We also estimated the body fat mass and classified patients into nutritional status categories.</div></div><div><h3>Results</h3><div>The overall analysis found a lower height <em>z</em> score in patients with PMDs and an equal proportion of overweight and obesity with respect to controls (30.2%). When we divided patients with PMDs into 2 groups (phenylketonuria and other aminoacidopathies), we found that patients with phenylketonuria had a height that was similar to the height of controls and significantly higher compared to patients with other aminoacidopathies. When it came to nutritional status, the prevalence of overweight and obesity was greater in the phenylketonuria group (45.5%), while underweight and short stature were more frequent in the group with other aminoacidopathies.</div></div><div><h3>Conclusion</h3><div>Not all patients with PMDs follow the same growth pattern, and their body composition is variable. In our sample, the group of patients with phenylketonuria had an adequate height but also a higher prevalence of overweight and obesity. On the other hand, patients with other aminoacidopathies had a higher prevalence of underweight and lower <em>z</em> scores for height and arm circumference.</div></div>","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 331-336"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142572544","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":"Eritema multiforme mayor/síndrome de Stevens-Johnson: un desafío diagnóstico","authors":"Rita Lages Pereira ,&nbsp;Mariana Oliveira Pereira ,&nbsp;Carmo Ferreira ,&nbsp;Ivo Neves","doi":"10.1016/j.anpedi.2024.06.011","DOIUrl":"10.1016/j.anpedi.2024.06.011","url":null,"abstract":"","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 361-363"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141841015","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":"Insuficiencia suprarrenal primaria: casuística de cinco hospitales terciarios","authors":"María Teresa Ovejero García ,&nbsp;Blanca Sáez Gallego ,&nbsp;Ana Coral Barreda Bonís ,&nbsp;Jesús Domínguez Riscart ,&nbsp;María Beatriz Garnier Rodríguez ,&nbsp;Ruth Molina Suárez ,&nbsp;Antonio De Arriba Muñoz","doi":"10.1016/j.anpedi.2024.08.001","DOIUrl":"10.1016/j.anpedi.2024.08.001","url":null,"abstract":"<div><h3>Introduction</h3><div>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.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Objectives</h3><div>The objective was to determine the aetiologies, signs, symptoms and laboratory findings of PAI in the paediatric age group.</div></div><div><h3>Results</h3><div>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).</div></div><div><h3>Conclusions</h3><div>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.</div></div>","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 303-309"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142572463","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":"Manejo del síndrome de distrés respiratorio en recién nacidos prematuros moderados/tardíos: consenso Delphi","authors":"Segundo Rite Gracia ,&nbsp;Juan José Agüera Arenas ,&nbsp;Gemma Ginovart Galiana ,&nbsp;María Jesús Rodríguez Revuelta","doi":"10.1016/j.anpedi.2024.08.011","DOIUrl":"10.1016/j.anpedi.2024.08.011","url":null,"abstract":"<div><h3>Introduction</h3><div>Respiratory distress syndrome (RDS) is the most frequent cause of respiratory distress in preterm neonates. In the management of RDS, surfactant plays a pivotal role, but there are no evidence-based recommendations for moderate/late preterm neonates (32-36 weeks).</div></div><div><h3>Methods</h3><div>A scientific committee developed a questionnaire with 53 questions addressing diagnosis, treatment, potential complications and future trends in RDS specifically focused on moderate and late preterm neonates. This was followed by the performance of a Delphi survey of expert neonatologists.</div></div><div><h3>Results</h3><div>Consensus was reached on 98 of the 109 items. The recommendations for the diagnosis of RDS included performing a lung ultrasound and including mild respiratory distress, transient tachypnoea of the newborn, congenital pneumonia and primary pulmonary hypertension in the differential diagnosis. Most panellists agreed on the need for studies that determine the benefit/harm balance, clinical profile and methods of surfactant administration in moderate/late preterm neonates. All respondents would use the MIST approach with devices specifically designed for surfactant administration. Regarding sedation measures during MIST, most participants agreed on the use of nonpharmacological interventions and, if these proved ineffective, an opioid. All respondents agreed that moderate/late preterm neonates are at increased risk of neonatal morbidity and mortality, particularly respiratory problems, and considered the need for more specialised monitoring in hospital follow-up visits in neonates with associated risk factors or a history of complications in the neonatal period. Finally, all respondents agreed that there is a lack of studies identifying risk factors and medium-term adverse outcomes in moderate/late preterm neonates.</div></div><div><h3>Conclusion</h3><div>This expert consensus will help with the diagnosis and management of RDS and guide decision-making about surfactant administration in moderate/late preterm neonates.</div></div>","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 319-330"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142572466","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":"Utilidad del oclusor Amplatzer Piccolo™ en el tratamiento endovascular del secuestro pulmonar en lactantes","authors":"Justo Santiago ,&nbsp;Jorge Alvarado ,&nbsp;Michell Guarin ,&nbsp;María I. Diaz ,&nbsp;Alexandra Hurtado","doi":"10.1016/j.anpedi.2024.07.011","DOIUrl":"10.1016/j.anpedi.2024.07.011","url":null,"abstract":"","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 355-357"},"PeriodicalIF":1.5,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142572538","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":"Reemergencia de la tosferina: estrategias y retos en su control en España","authors":"Fernando Moraga-Llop ,&nbsp;María Garcés-Sánchez ,&nbsp;Juan José González-López","doi":"10.1016/j.anpedi.2024.07.012","DOIUrl":"10.1016/j.anpedi.2024.07.012","url":null,"abstract":"","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 4","pages":"Pages 227-229"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142418877","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":"Uso de antibioterapia tópica en otitis media aguda con otorrea: resultados de una encuesta nacional","authors":"Maria Garcia de Oteyza ,&nbsp;Miguel Ángel Molina Gutiérrez ,&nbsp;Carlota Marín Córdoba ,&nbsp;María de Ceano-Vivas","doi":"10.1016/j.anpedi.2024.07.001","DOIUrl":"10.1016/j.anpedi.2024.07.001","url":null,"abstract":"","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 4","pages":"Pages 291-292"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142418880","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":"Sufrimiento infantojuvenil en cuidados paliativos pediátricos en España: propiedades psicométricas de la escala qESNA","authors":"Daniel Toro-Pérez ,&nbsp;Joaquín T. Limonero ,&nbsp;Catalina Bolancé ,&nbsp;Montserrat Guillen ,&nbsp;Sergi Navarro Vilarrubí ,&nbsp;Ester Camprodon-Rosanas","doi":"10.1016/j.anpedi.2024.07.005","DOIUrl":"10.1016/j.anpedi.2024.07.005","url":null,"abstract":"<div><h3>Objective</h3><div>To analyze the psychometric properties of the qESNA scale and its usefulness to assess the suffering of paediatric patients with life-limiting and/or life-threatening diseases (children with LLTC) in clinical practice.</div></div><div><h3>Methods</h3><div>Cross-sectional and longitudinal study in 58 patients in Spain (female, 32.8%; mean age, 15.6 [SD, 4.5]; age range, 8-23 years), with administration of the qESNA scale along with other scales to assess anxiety, depression, emotional changes and psychosocial functioning. We performed exploratory and confirmatory factor analyses and calculated goodness-of-fit indices were calculated; we assessed reliability by means of the Cronbach alpha and temporal stability and convergent validity through the intraclass correlation coefficient with scales used to assess psychological disorders and the specificity and sensitivity through ROC curves.</div></div><div><h3>Results</h3><div>The factor analysis identified a 14-item scale with 3 factors, a comparative fit index of 0.93, a Tucker-Lewis index of 0.91 and a root mean square error of approximation of 0.07. the Cronbach alpha was 0.85 and the intraclass correlation coefficient was 0.66. The convergent validity was high for the correlation to the risk of depression (−0.69) and of anxiety (−0.60) and emotional changes (−0.59). The analysis of the ROC curves showed that a score of less than 81 would be indicative of suffering, with a sensitivity of 83.33% and a specificity of 93.48%.</div></div><div><h3>Conclusions</h3><div>This study confirmed that the psychometric properties of the qESNA scale are good and the scale's usefulness as an instrument to detect emotional suffering in children with life-limiting or life-threatening illnesses in clinical practice.</div></div>","PeriodicalId":7783,"journal":{"name":"Anales de pediatria","volume":"101 4","pages":"Pages 238-248"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142418879","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}