Anales de pediatria最新文献

{"title":"Oral blistering mucositis in adolescent","authors":"Jana Codina Barbosa , Alicia Suñé Aznarez , Carla Donado-Mazarrón Garcia , José Martínez Hernando","doi":"10.1016/j.anpede.2024.11.002","DOIUrl":"10.1016/j.anpede.2024.11.002","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 419-420"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142741629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute cholecystitis as an adverse effect of treatment with elexacaftor/tezacaftor/ivacaftor in a patient with cystic fibrosis","authors":"Carlos Martín de Vicente , Silvia Jade Cáceres Barrera , Celia Fuentes Sánchez , Cristina Gutiérrez Alonso , Ruth García Romero","doi":"10.1016/j.anpede.2024.11.006","DOIUrl":"10.1016/j.anpede.2024.11.006","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 413-414"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142775617","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Expert consensus for the management of patients with achondroplasia in treatment with vosoritide","authors":"Ana-Coral Barreda-Bonis ,&nbsp;Josep Maria de Bergua Domingo ,&nbsp;Enrique Galán-Gómez ,&nbsp;Encarna Guillén-Navarro ,&nbsp;Isabel Leiva-Gea ,&nbsp;Isolina Riaño-Galán","doi":"10.1016/j.anpede.2024.09.012","DOIUrl":"10.1016/j.anpede.2024.09.012","url":null,"abstract":"<div><h3>Introduction</h3><div>Patients with achondroplasia present, in addition to disproportionate short stature, multiple manifestations that require a comprehensive approach. The present consensus of experts in Spain responds to the need to establish clear guidelines for the management of achondroplasia with the introduction of a new treatment, vosoritide.</div></div><div><h3>Material and methods</h3><div>A panel of six experts in achondroplasia participated in the development of the consensus. They developed a narrative review of the recommendations on achondroplasia and vosoritide treatment, which were agreed upon and adapted to the Spanish context in two subsequent meetings with a structured discussion format.</div></div><div><h3>Results</h3><div>This protocol underscores that achondroplasia requires specialised and multidisciplinary management involving expert paediatricians and specialists in paediatric endocrinology or medical genetics, in collaboration with specialists in neurology, neurosurgery, pneumology, otorhinolaryngology, rehabilitation, and orthopaedics or psychology, among others, with adequate coordination of care. This interdisciplinary team should be involved in the planning of vosoritide treatment (including the education of patients and caregivers, with management of their expectations, and their training in the practical aspects of vosoritide administration), treatment initiation and close monitoring with regular assessment of anthropometric, biochemical, functional or patient-reported variables.</div></div><div><h3>Conclusions</h3><div>This protocol for the administration of vosoritide will allow standardised implementation and optimization of treatment outcomes. It also offers an opportunity to improve the management of achondroplasia in Spain through a comprehensive and interdisciplinary approach.</div></div>","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 401-410"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792681","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Implementation of the mini-CEX in a paediatric emergency department","authors":"Iris Alonso Sánchez ,&nbsp;Sandra Morán Moya ,&nbsp;Gemma Claret Teruel ,&nbsp;Roser Garrido Romero ,&nbsp;David Muñoz Santanach ,&nbsp;Victoria Trenchs Sainz de la Maza ,&nbsp;Carles Luaces Cubells","doi":"10.1016/j.anpede.2024.11.005","DOIUrl":"10.1016/j.anpede.2024.11.005","url":null,"abstract":"<div><h3>Introduction</h3><div>The mini-CEX is a direct observation of care delivery that provides feedback to the medical resident; and previous studies have confirmed its validity, reliability, feasibility and user satisfaction. Its application in the paediatric emergency care setting is scarce.</div></div><div><h3>Objective</h3><div>To assess trends in the acquisition of emergency care skills in paediatrics residents through the mini-CEX and determine the satisfaction of participants after its implementation.</div></div><div><h3>Material and methods</h3><div>Inclusion criteria: paediatrics residents in the first (MIR1) and second (MIR2) year of residency. Setting: emergency department. Study period: 4 years (May 2019–May 2023). Evaluated competencies: anamnesis, physical examination, professionalism, clinical judgment, communication, organization-efficiency and global assessment. Number of evaluations: maximum of 6 per resident per year; each evaluation assessed 2–3 competencies (each competency could be evaluated twice per year). The evaluators were adjunct physicians in the emergency department; medical residents were assessed for training purposes. Satisfaction was rated on a scale from 1 to 9.</div></div><div><h3>Results</h3><div>A total of 217 evaluations were performed on 54 residents (1–9 per resident). The median observation time was 16 min per visit (IQR, 14–25) and the feedback time was 10 min per visit (IQR, 7–15). The median anamnesis, physical examination, clinical judgment, organization-efficacy and global scores were 7 for MR1 and 8 for MRI2 residents (<em>P =</em> .015; <em>P =</em> .001, <em>P =</em> .076, <em>P =</em> .009 and <em>P =</em> .010, respectively). We did not find significant differences in the remaining competencies. The average satisfaction score was 9 for both evaluators and residents.</div></div><div><h3>Conclusions</h3><div>The acquisition of skills was high; with favourable outcomes observed in MRI2 relative to MRI1. The implementation of the mini-CEX was well accepted among the involved professionals.</div></div>","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 388-392"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe hypertransaminasaemia during the acute hepatitis of unknown origin alert of 2022","authors":"Ana Martín Costa ,&nbsp;Ignacio Ros Arnal ,&nbsp;Ruth García Romero ,&nbsp;Gerardo Rodríguez Martínez ,&nbsp;Beatriz García Rodríguez","doi":"10.1016/j.anpede.2024.11.007","DOIUrl":"10.1016/j.anpede.2024.11.007","url":null,"abstract":"<div><h3>Introduction</h3><div>The impact of acute severe hepatitis of unknown origin in children (SHIC) subject to a medical alert in 2022 medical alert is poorly understood.</div></div><div><h3>Materials and methods</h3><div>Observational study of the incidence, aetiology and clinical presentation of acute hypertransaminasaemia (HTRA) with laboratory values in the severe range (ALT and/or AST ≥ 500 U/L) in paediatric patients (age 0 to 16 years) in one health care zone from 2012 to 2022, comparing the periods of the SHIC alert and the SARS-CoV2 pandemic with previous years.</div></div><div><h3>Results</h3><div>The incidence of severe HTRA of any cause was 195.28 per 100 000 blood tests, with an incidence of 181.38 in the SHIC alert period and 166.09 during the SARS-CoV-2 pandemic, without statistically significant differences.</div><div>Hepatitis of unknown origin accounted for 7.42% of total cases and transaminase levels normalised in 126 days (SD, 99.4). During the SHIC alert period there was a nonsignificant trend towards a higher incidence, as occurred in 2012 and 2018. In this group of cases, there was a significant increase in the presence of fever, vomiting and upper respiratory symptoms and lower levels of albumin and alkaline phosphatase. One patient required a liver transplant.</div></div><div><h3>Conclusions</h3><div>In our setting, there was no significant increase in the incidence of severe HTRA of any aetiology or of unknown source during either the SHIC alert or the SARS-CoV2 pandemic. In the SHIC alert period, a clinical pattern emerged characterised by an increase in nonspecific infectious symptoms, so we cannot rule out a higher prevalence of an infectious agent different from the usual involved pathogens, but it did not cause a significant change in epidemiological trends.</div></div>","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 370-377"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neutrophil CD64 expression increases in infants aged less than 3 months with fever without source: pilot study in the paediatric emergency care setting","authors":"Valeria Antoñanzas Bernar ,&nbsp;Pilar Storch de Gracia-Calvo ,&nbsp;Inés Leoz Gordillo ,&nbsp;Ana Castillo Robleda ,&nbsp;Alberto García-Salido","doi":"10.1016/j.anpede.2024.08.006","DOIUrl":"10.1016/j.anpede.2024.08.006","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 416-418"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142514476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paediatric endoscopy in Spain: current situation and recent developments","authors":"María Navalón Rubio ,&nbsp;Carmen Jovani Casano ,&nbsp;Marta Soria López ,&nbsp;Alfonso Rodríguez Herrera ,&nbsp;Miguel Ángel López Casado ,&nbsp;Víctor Manuel Navas-López ,&nbsp;Víctor Vila Miravet ,&nbsp;en representación del Grupo de trabajo de Endoscopia pediátrica de la Sociedad Española de Gastroenterología Hepatología y Nutrición Pediátrica (SEGHNP)","doi":"10.1016/j.anpede.2024.11.004","DOIUrl":"10.1016/j.anpede.2024.11.004","url":null,"abstract":"<div><h3>Introduction</h3><div>Paediatric gastrointestinal endoscopy (pGIE) has advanced significantly over the last decade, with increased diagnostic and therapeutic applications.</div></div><div><h3>Objectives</h3><div>This study examines the current state of pGIE in Spain, changes in the field over 5 years, and the involvement of paediatric gastroenterologists (pGEs).</div></div><div><h3>Materials and methods</h3><div>A structured self-administered questionnaire was distributed by the Endoscopy Working Group of the Spanish Society of Paediatric Gastroenterology, Hepatology, and Nutrition (SEGHNP) through the REDCap platform. The questionnaire included questions concerning techniques, sedation, indications, and organizational barriers. We compared the results with data from 2015.</div></div><div><h3>Results</h3><div>Eighty-one of the 103 invited hospitals participated (66.4%). Paediatric gastroenterologists performed 71% of scheduled pGIEs and 24% of emergency pGIEs. Therapeutic endoscopies accounted for 72% of procedures, including foreign body removal (50%) and polypectomy (36%), and were performed by pGEs in 61% and 63% of cases, respectively. When it came to other procedures, pGEs performed 24% in the case of hydrostatic dilation, 20% in the case of variceal sclerotherapy and of haemoclip placement, and 18% in the case of endoscopic coagulation. None of the endoscopic ultrasound, endoscopic retrograde cholangiopancreatography or enteroscopy procedures were performed by pGEs. Sedation was administered by anaesthesiologists in 70% of cases. Compared to 2015, there was an increase in the scheduled endoscopies performed by pGEs (71% vs 54%), the performance of gastroscopies (&gt;180 per year in 61% vs 24%) and colonoscopies (60−120 per year in 31% vs 12%) and the use of picosulfate for colonoscopy preparation (44% vs 10%).</div></div><div><h3>Conclusions</h3><div>There has been a significant increase in the involvement of pGEs in pGIE in Spain, although of lesser magnitude in emergency and therapeutic endoscopy procedures.</div></div>","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 393-400"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792894","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Metabolopathy with characteristic clinical triad: Sengers syndrome","authors":"Laura Naqui Xicota,&nbsp;Elisenda Cortès-Saladelafont,&nbsp;Elena Berrocal Acevedo,&nbsp;Andrea Ros Peña,&nbsp;Berta Brussosa Ventura,&nbsp;Roser Porta Ribera","doi":"10.1016/j.anpede.2024.11.001","DOIUrl":"10.1016/j.anpede.2024.11.001","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 415-416"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142752647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Childhood desmoplastic ganglioglioma in an infant, radiological aspects to know","authors":"Juan Ramón y Cajal Calvo ,&nbsp;Laura Pérez Abad ,&nbsp;Patricia Morte Coscolin","doi":"10.1016/j.anpede.2024.11.003","DOIUrl":"10.1016/j.anpede.2024.11.003","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 6","pages":"Pages 421-422"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of respiratory distress syndrome in moderate/late preterm neonates: A Delphi consensus","authors":"Segundo Rite Gracia ,&nbsp;Juan José Agüera Arenas ,&nbsp;Gemma Ginovart Galiana ,&nbsp;María Jesús Rodríguez Revuelta","doi":"10.1016/j.anpede.2024.10.003","DOIUrl":"10.1016/j.anpede.2024.10.003","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":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 319-330"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142565066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}