Isidro Robredo García , Paula Grattarola , Patricia Correcher Medina , Fátima Abu-Sharif Bohigas , Verónica Vélez García , Isidro Vitoria Miñana , Cecilia Martínez Costa
{"title":"Nutritional status in patients with protein metabolism disorders. Case-control study","authors":"Isidro Robredo García , Paula Grattarola , Patricia Correcher Medina , Fátima Abu-Sharif Bohigas , Verónica Vélez García , Isidro Vitoria Miñana , Cecilia Martínez Costa","doi":"10.1016/j.anpede.2024.10.012","DOIUrl":"10.1016/j.anpede.2024.10.012","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":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 331-336"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606202","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":"The minor in Spanish legislation: an ethics perspective","authors":"María Alija , Isolina Riaño , Montserrat Esquerda","doi":"10.1016/j.anpede.2024.08.007","DOIUrl":"10.1016/j.anpede.2024.08.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Legislation embodies and regulates the common values by which a society is governed. Today’s society is multicultural and dynamic, a consensus must be reached and values consolidated on which different ideological groups can agree, an approach known as the ethics of minima. The concept of the minor has also been changing over the years; from being perceived as passive to being considered an autonomous individual engaged in the development of his or her life project and the bearer of duties and rights to be progressively exercised according to his or her capacity. This approach entails emerging problems. Both aspects need to be regulated in our legislation.</div></div><div><h3>Objective</h3><div>The objective of this work was to consider the different laws that concern minors from an ethical perspective and from the point of view of the practicing paediatrician.</div></div><div><h3>Material and methods</h3><div>We reviewed legislation concerning minors from the 1990s to the present day. For the purpose of discussion, we grouped it into 4 sections: the minor as a patient, protection of the minor, education and new social realities.</div></div><div><h3>Conclusions</h3><div>Current laws reflect a plural society and focus on protecting minority groups. They recognize the minor as the bearer of rights and duties, and consider the acquisition of autonomy to be progressive, guaranteeing equal opportunity and respecting the child’s own pace in achieving his or her potential. Current legislation also stresses the need for a stable environment and states that all forms of violence are preventable. Furthermore, it respects different moral, social and cultural perspectives.</div></div>","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 337-343"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142634330","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}
Justo Santiago , Jorge Alvarado , Michell Guarin , María I. Diaz , Alexandra Hurtado
{"title":"Utility of the Amplatzer Piccolo™ occluder in the endovascular treatment of pulmonary sequestration in infants","authors":"Justo Santiago , Jorge Alvarado , Michell Guarin , María I. Diaz , Alexandra Hurtado","doi":"10.1016/j.anpede.2024.10.011","DOIUrl":"10.1016/j.anpede.2024.10.011","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 355-357"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142559694","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":"Are adverse childhood experiences the hidden iceberg of emotional distress in children and adolescents?","authors":"Ana Rosa Sánchez Vázquez","doi":"10.1016/j.anpede.2024.10.013","DOIUrl":"10.1016/j.anpede.2024.10.013","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 299-302"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142605599","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}
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
{"title":"Recommendations of the Spanish Society of Neonatology for the prevention of severe respiratory syncytial virus infections with nirsevimab, for the 2024–2025 season","authors":"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","doi":"10.1016/j.anpede.2024.09.010","DOIUrl":"10.1016/j.anpede.2024.09.010","url":null,"abstract":"","PeriodicalId":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 364-366"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142514477","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}
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
{"title":"Primary adrenal insufficiency: case study IN 5 tertiary hospitals","authors":"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","doi":"10.1016/j.anpede.2024.10.008","DOIUrl":"10.1016/j.anpede.2024.10.008","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":93868,"journal":{"name":"Anales de pediatria","volume":"101 5","pages":"Pages 303-309"},"PeriodicalIF":0.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606311","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}