Patient-reported outcome measures and value-based medicine in paediatrics: a timely review

Healthcare delivery is moving towards a more personalised and patient-centric approach. There is now an appropriate emphasis on providing value in our healthcare system. Patient-reported outcome measures (PROMs) assess our patients' perceptions of the status of their health and quality of life, measured over a period of time. PROM is an integral component of a value-driven and value-based healthcare system and is key if we want to practise value-based medicine. In paediatrics and child health, PROMs, if implemented well with appropriate measurement tools that are regularly updated and validated in a self-learning healthcare ecosystem, will help to enhance personalised healthcare delivery and collectively improve the health of the community at large. This review covers the role of PROMs in paediatrics, as well as their role in value-based medicine.

A nutrição no transtorno do espectro autista: benefícios de intervenções dietéticas na infância / Nutrition in autism spectrum disorder benefits of dietary interventions in childhood / Nutrición en el trastorno del espectro autista: beneficios de las intervenciones dietéticas en la infância

Objetivo: Conduzir uma revisão de literatura com foco na busca de intervenções nutricionais em pacientes pediátricos que apresentam transtorno do espectro autista (TEA). Método: Tratase de uma revisão narrativa realizada entre dezembro de 2022 a janeiro de 2023 nas bases de dados eletrônicas: nas bases de dados MEDLINE, SCIENCE DIRECT, PUBMED, SCIELO, LILACS e Google Acadêmico por meio dos descritores de assunto: transtorno do espectro autista, TEA, autismo, nutrição, terapia nutricional, pediatria, criança, adolescente. Resultados: O uso de dietas contendo nutrientes específicos, além da utilização de suplementos nutricionais, como vitaminas, minerais, ácidos graxos ômega-3 são abordados de forma fornecer evidências atualizadas sobre sua utilização. Conclusão: o uso de intervenções nutricionais pode melhorar algumas questões observadas em pacientes pediátricos com TEA e propiciar uma vida com maior qualidade, garantindo que as necessidades nutricionais da criança sejam atingidas para a promoção do crescimento e desenvolvimento.

Objective: To conduct a literature review focusing on the search for nutritional interventions in pediatric patients with autism spectrum disorder (ASD). Method: This is a narrative review conducted between December 2022 and January 2023 in the electronic databases: in the databases MEDLINE, SCIENCE DIRECT, PUBMED, SCIELO, LILACS and Google Scholar through the subject descriptors: autism spectrum disorder, ASD, autism, nutrition, nutritional therapy, pediatrics, child, adolescent. Results: The use of diets containing specific nutrients, in addition to the use of nutritional supplements such as vitamins, minerals, omega-3 fatty acids are addressed in order to provide updated evidence on their use. Conclusion: the use of nutritional interventions can improve some issues observed in pediatric patients with ASD and provide a higher quality of life, ensuring that the nutritional needs of the child are met to promote growth and development.

Objetivo: Realizar una revisión bibliográfica centrada en la búsqueda de intervenciones nutricionales en pacientes pediátricos con trastorno del espectro autista (TEA). Método: Se trata de una revisión narrativa realizada entre diciembre de 2022 y enero de 2023 en las bases de datos electrónicas: en las bases de datos MEDLINE, SCIENCE DIRECT, PUBMED, SCIELO, LILACS y Google Scholar a través de los descriptores temáticos: trastorno del espectro autista, TEA, autismo, nutrición, terapia nutricional, pediatría, niño, adolescente. Resultados: Se aborda el uso de dietas que contienen nutrientes específicos, además del uso de suplementos nutricionales como vitaminas, minerales, ácidos grasos omega-3 con el fin de proporcionar evidencia actualizada sobre su uso. Conclusión: el uso de intervenciones nutricionales puede mejorar algunos problemas observados en pacientes pediátricos con TEA y proporcionar una mayor calidad de vida, asegurando que se satisfagan las necesidades nutricionales del niño para promover el crecimiento y el desarrollo.

Antibiotic prescription patterns in paediatric wards of Rivers State university teaching hospital, southern Nigeria: a point prevalence survey

Antimicrobial resistance remains a threat to patient safety and healthcare outcomes and largely arises from inappropriate antimicrobial prescriptions. This study aimed to determine the pattern of antibiotic prescriptions in the Paediatrics department of Rivers State University Teaching Hospital, Port Harcourt.Method:A point prevalence survey was conducted in the Paediatric wards and Special Care Baby Unit (SCBU) on 13 November 2021. Records of all children admitted before or at 8:00a.m. on the day of the survey were descriptively analysed using the protocol and web-based management system of the Global Point Prevalence Survey of Antimicrobial Consumption and Resistance, University of Antwerp.Results: The antibiotic prevalence in this study was 77.4%. The most common indication(s) for antibiotic use in SCBU was infection prophylaxis (81.3%) and in paediatric wards: Pneumonia, Ear Nose Throat and Soft tissue infections accounted for (23.1%) each. Third-generation cephalosporins and aminoglycosides were predominantly used in all wards and were empirical-based prescriptions. Regarding antibiotic quality indicators of prescriptions: In SCBU: 19 (90.5%) had indication(s) for antibiotics documented, 10 (46.7%) were guideline compliant, and 1 (4.8%) had documented review/stop date. In the paediatric medical and surgical wards, 17(85.0%) vs. 4(100%) had indication(s) for antibiotics documented, 6(30.0%) vs. 0(0%) were guideline compliant, and 1(5.0%) vs. 4(100.0%) had a review/ stop date.Conclusion:High prevalence of antibiotic use, suboptimal antibiotic quality indicators and absence of laboratory evidence for antibiotic prescriptions were observed in the paediatric units. There is a need to reorientate prescribers and institute strategic measures to improve antimicrobial stewardship

Prévalence et facteurs associés au syndrome d'épuisement professionnel chez le personnel de santé du service de pédiatrie du Centre Hospitalier Universitaire de Bouaké (Côte d'Ivoire) / Prevalence and factors associated with burnout syndrome among health personnel in the paediatric department of the university hospital of Bouaké (Côte d'Ivoire)

Contexte et objectif.: Le syndrome d'épuisement professionnel (SEP) est une pathologie particulièrement préoccupante en milieu hospitalier avec un impact négatif sur la qualité des soins. L'objectif de l'étude était de connaitre la prévalence et les principaux facteurs associés au SEP chez le personnel de santé en pédiatrie au CHU de Bouaké. Méthodes. Etude prospective, descriptive et analytique réalisée en pédiatrie au CHU de Bouaké du 05 octobre au 03 novembre 2022. Etaient inclus les agents dudit service, consentants, reconnus par la Direction des Ressources Humaines du CHU de Bouaké et présent durant la période de l'étude. Les variables étudiées étaient socioprofessionnelles et l'évaluation du SEP. Le SEP a été évalué à l'aide du Maslach Burnout Inventory (MBI). Résultats : Au total 70 participants dont 39 présentant un SEP (13 hommes, 26 femmes) soit une prévalence de 56%. Les facteurs signifi cativement associés au SEP étaient la situation matrimoniale (Célibataire) (p=0,004 ; OR 0,155 ; IC 0,043-0,563), et le statut d'agent journalier (p=0,024 ; OR 0,058 IC 0,005-687). Conclusion. Le SEP était très fréquent en pédiatrie du CHU de Bouaké. Il avait un lien étroit avec la situation matrimoniale et le statut d'agent journalier. Pour améliorer la situation nous recommandons un accompagnement psychologique du personnel ainsi que la revalorisation salariale et des conditions de travail des agents journaliers. Mots clés : -Syndrome d'épuisement professionnel; -Hôpital; -Pédiatrie, -Côte d'Ivoire. ABSTRACT Background and object

Background and objective. Burnout syndrome (BWS) is a particularly worrying pathology in the hospital environment with a negative impact on the quality of care. The objective of the study was to determine the prevalence and main factors associated with burnout among paediatric health care staff at the University Hospital of Bouaké. Methods. Prospective, descriptive and analytical study conducted in pediatrics at the University Hospital of Bouaké from October 5 to November 3, 2022. The study included consenting staff of the said department, recognised by the Human Resources Department of the University Hospital of Bouaké and present during the study period. The variables studied were socio-professional and SEP evaluation. The BWS was assessed using the Maslach Burnout Inventory (MBI). Results. A total of 70 participants, 39 of whom had MS (13 men, 26 women), representing a prevalence of 56%. The factors significantly associated with MS were marital status (single) (p=0.004; OR 0.155; CI 0.043-0.563), and day worker status (p=0.024; OR 0.058 CI 0.005-687). Conclusion. The SEP is very frequent in the paediatric ward of the University Hospital of Bouaké. It is closely related to marital status and day labourer status. To improve the situation, we recommend psychological support for the staff as well as an increase in the salary and working conditions of day workers.

Antibiotic safety among neonates and paediatrics in a public hospital: KwaZulu-Natal

Background: The World Health Organization (WHO) guidelines recommend the empiric treatment of infections before definitive treatment begins. However, ethical concerns limit the availability of clinical trials in neonates and paediatrics to fully ascertain the safety profile of antibiotics in these populations. Aim: This study aimed to quantify the use of antibiotics among neonates and paediatrics and commented on the use, rationale and appropriateness of antibiotics prescribed. Setting: A secondary level public sector hospital located in Durban, KwaZulu-Natal. Methods: Demographic and treatment information of neonates and paediatrics were collected retrospectively from January 2022 to June 2022. Data were obtained from patient files and extracted for analysis using Microsoft Excel®. Analytical and descriptive statistics were used to analyse patient demographics and treatment variables. Results: A total of 568 antibiotics, issued to 389 patients, were reviewed. Penicillins (40.1%), aminoglycosides (24.3%) and combination penicillin-beta-lactam inhibitors (23.3%) were identified as the most frequently prescribed antibiotics for inpatients. Most antibiotics prescribed to inpatients were for complications associated with pre-term birth (66.9%). Combination penicillin-beta-lactam inhibitors (34.7%), penicillins (29.5%) and cephalosporins (29.5%) were the most frequently prescribed antibiotics to outpatients. A correlation was found between the route of administration and the duration of therapy; the intravenous route (63.6%) was preferred over the oral route (36.4%) for administration. Conclusion: Many broad-spectrum antibiotics were prescribed, thus increasing the risk of resistance. Antibiotics were being prescribed according to the guidelines; however, there is still a need for therapeutic drug monitoring to ensure the continuation of rational drug use. Contribution: There was evidence of rational use of antibiotics in the public hospital (KwaZulu-Natal), in keeping with economic and availability factors.

Characteristics of Paediatric Death Cases at a Malaysian University Hospital

@#Introduction: Auditing of paediatric deaths has rarely been evaluated in hospitals especially in low and middle-income countries. The aim of the study is to describe the characteristics of paediatric death in hospitalised children and determine the predictors of death location in Hospital Universiti Sains Malaysia (USM). Method: Paediatric mortality cases for children aged between 29 days and 18 years old at the time of death were analyzed. These were documented cases between the year 2013 and 2020 at Hospital USM. Case notes were reviewed, and data were captured retrospectively. Multiple Logistic Regression was used to assess predictors of the location of death especially in the ward setting.Results: Out of 841 paediatric deaths identified during the period of the study, 544 cases were enrolled. The male gender has a higher proportion (1.4:1). The median age was 56 months, with infancy being the predominant age group. The median length of hospital stay was 10.5 days. More than half of the cases (58.3%) had underlying life-limiting illnesses. The majority of the patients had “Do Not Resuscitate” orders. A longer length of stay (prevalence odds ratio (POR 0.99, 95% CI 0.98, 0.99), being on ventilator (POR 3.42,95% CI 1.95, 6.01), being terminally ill (POR 0.40,95% CI 0.23, 0.70) and having underlying life limiting illness (POR 0.50, 95% CI 0.33, 0.75) were the significant predictors for the ward death. Conclusion: Understanding the characteristics of child death and the factors associated with death location is critical for improving paediatric care and treatment.

PANS-PANDAS, reporte de caso / PANS-PANDAS, Case Report

RESUMEN Introducción: Desde 1980 se conocen casos de síndromes neuropsiquiátricos infantiles en el mundo y su concepto ha evolucionado con cambios en las definiciones de 1995 (PITANDS: trastornos neuropsiquiátricos pediátricos autoinmunes precipitados por infección), 1998 (PANDAS: síndrome neuropsiquiátrico autoinmune pediátrico asociado con la infección por estreptococos), 2010 (PANS: síndrome pediátrico neuropsiquiátrico de inicio agudo) y 2012 (CANS: síndromes neuropsiquiátricos agudos de los niños). A pesar de que se conoce desde hace más de 20 años, aún es una enfermedad que suele pasar inadvertida para muchos profesionales de la salud. Objetivo: Sensibilizar a la comunidad médica acerca de la identificación de la enfermedad y disminuir la morbilidad asociada con un diagnóstico tardío. Caso clínico: Una niña de 6 años consultó a urgencias por trastorno de rechazo alimentario. En el tratamiento hospitalario se identificó historia clínica con criterios diagnósticos de PANS-PANDAS. Mostraba un curso clínico recurrente-remitente, tal y como describe la literatura, con pobre respuesta a los tratamientos de primera línea. Conclusiones: En todo niño en edad escolar que se presente con trastorno obsesivo compulsivo o trastornos alimentarios, con sin otros síntomas, se debe evaluar y descartar su asociación con PANS-CANS.

ABSTRACT Introduction: Since 1980, there have been known cases of childhood neuropsychiatric syndromes in the world and its concept has evolved with changes in the definitions in 1995 (PITANDs - paediatric infection-triggered autoimmune neuropsychiatric disorders), 1998 (PANDAS - paediatric autoimmune neuropsychiatric syndrome associated with streptococci infection), 2010 (PANS - paediatric acute-onset neuropsychiatric syndrome) and 2012 (CANS - childhood acute neuropsychiatric syndrome). Despite being known for more than 20 years, it is still an illness that often goes unnoticed by many health professionals. Objective: To sensitise the medical community about the identification of the disease and reduce the morbidity associated with a late diagnosis. Clinical case: A 6-year-old schoolgirl brought to the emergency department due to her refusal to eat. In the hospital treatment, a clinical history was identified with PANS-PANDAS diagnostic criteria. She exhibited a relapsing-remitting clinical course, as described in the literature, with poor response to first-line treatments. Conclusions: In all school-age child presenting with obsessive compulsive disorder or eating disorders, with other symptoms or not, a possible link to PANS-CANS should be evaluated and ruled out.

Study of Prevalence of Iron Deficiency Anemia in Hospitalised Children in Tertiary Care Centre

Background: Anemia prevalence in young children con?nues to remain over 70% in most parts of India and Asia. In developing countries like India,anemia is a significant cause of mortality and morbidity in children under 5 years of age. In public health terms, Iron deficiency anemia (IDA) is a very important causa?ve factor for childhood anemia. Materials & Methods: A prospec?ve observa?onal study was conducted on hospitalized children in ter?ary care center. All children between 1 month to 18 years of age were admi?ed in Pediatric ward were taken to the study for 1.5 years study period. Results: Total number pa?ents admi?ed in pediatric ward were 1280 out of which 812 were enrolled for the study, out of which 522 pa?ents have been studied in detail as they had iron deficiency anemia. The total prevalence in our study period is 65.64%. Our analysis showed that males outnumbered females with sex ra?o of Male: Female is1.76:1. This study shows that the children of 1 month to 5 years is 361 which are 69.1%, children of age 5-12years are 123 which is 23.6%, children of age 12-18years are 38 which is 7.3%. We observed that the most common age group involved is less than 5 years which is significant. The e?ology of the hospitaliza?on has been studied, out of which Lower respiratory infec?ons were more common. Conclusion: This study revealed that the prevalence of IDA in under 5 years is more common than in other age groups. Children with anemia and those with iron deficiency anemia were found to be more suscep?ble to lower respiratory tract infec?ons.

Childhood aplastic anaemia with paroxysmal nocturnal haemoglobinuria clones: A retrospective single-centre study in South Africa

Background: Paroxysmal nocturnal haemoglobinuria (PNH) clones in children are rare but commonly associated with aplastic anaemia (AA) and myelodysplasia.Objective: This study aimed to determine the prevalence of PNH clones in paediatric patients with idiopathic AA, identify differences in clinical and laboratory features and outcomes, and determine the impact of clone size on clinical presentation.Methods: Patients with confirmed idiopathic AA who were tested for PNH between September 2013 and January 2018 at the Inkosi Albert Luthuli Central Hospital, Durban, KwaZulu-Natal, South Africa, were included. PNH clones were detected in neutrophils and monocytes by flow cytometry using fluorescent aerolysin, CD24, CD66b and CD14. Results: Twenty-nine children with AA were identified and 11 were excluded. Ten patients (10/18, 55.6%) had PNH clones ranging from 0.11% to 24%. Compared to the PNH-negative group, these children were older (median: 10 years vs 4 years, p= 0.02) and had significantly lower total white cell counts (median 1.7 × 109/L vs 3.2 × 109/L; p= 0.04). There was no difference in median absolute neutrophil count or haemoglobin concentration. Four patients in each group received immunosuppressive therapy (IST). At six months, all four patients with PNH clones had responded, compared to one in the PNH-negative group. Conclusion: More than half of children with AA had a PNH clone. The size of the clone did not impact clinical severity; however, IST use may positively impact prognosis. We recommend early initiation of IST in patients with AA to avoid delays associated with human leukocyte antigen typing.

Síndrome inflamatorio multisistémico pediátrico vinculado a SARS-CoV-2, una entidad severa e impredecible / Pediatric inflammatory multisystemic syndrome temporally associated with SARS-CoV-2 infection, a severe and unpredictable entity

La infección por COVID-19 en la población pediátrica tiene un curso leve en la mayoría de los casos. Sin embargo, en abril de 2020 se reportan por primera vez casos de niños que presentan un cuadro clínico compatible con un síndrome inflamatorio multisistémico de expresividad clínica variable y que se vincula a una infección reciente o activa por SARS-CoV-2. En este reporte se presenta un caso de síndrome inflamatorio multisistémico pediátrico vinculado a SARS-CoV-2 (SIM-PedS) cuyo diagnóstico fue dificultoso dada la variabilidad clínica del mismo. Se muestra el diagnóstico diferencial llevado a cabo y se pone de manifiesto la necesidad de tener en cuenta todos los signos y síntomas para poder llevar a cabo el diagnóstico de forma precoz, un manejo óptimo y disminuir la morbimortalidad secundaria. Este síndrome comparte rasgos clínicos y analíticos similares a la enfermedad de Kawasaki y al síndrome de shock tóxico o de activación macrofágica. Esto dificulta llegar al diagnóstico de forma precoz, precisando una valoración multidisciplinar por parte de diferentes especialistas pediátricos, así como seguir unas recomendaciones y protocolos para el diagnóstico, estabilización y tratamiento con el objetivo de homogeneizar el manejo en los diferentes centros de urgencia y hospitalización

In children, COVID-19 infection has usually a mild course. However, cases of children with multisystemic inflammatory syndrome presenting a variable clinical expression linked to a recent or active infection by SARS-CoV-2 were reported in April 2020. This report presents a case of paediatric inflammatory multisystemic syndrome temporally associated with SARS-CoV-2 infection (PIMS-TS), whose diagnosis was difficult given the variability in clinical expression. The differential diagnosis is shown and highlights the need to take into account all the signs and symptoms to get an early diagnosis, optimal management and reduce secondary morbidity and mortality. This variability in clinical manifestations with clinical and analytical features similar to Kawasaki syndrome, toxic shock syndrome or macrophage activation makes it difficult to reach an early diagnosis. It shows the need for a multidisciplinary assessment of different paediatric specialists, as well as to follow recommendations and protocols for the diagnosis, stabilization and treatment in order to homogenize the management in the emergency and hospitalization departments.

Randomized trial: inflammatory response to corticoids versus placebo in moderate and severe bronchiolitis. COTHEB study

ABSTRACT Introduction: The use of corticosteroids has been shown not to improve the prognosis of patients with bronchiolitis, but it could be assumed that steroids will reduce inflammation. Objective: This study aimed to assess whether corticoid therapy influenced the inflammatory and clinical response of critically ill infants. Methods: Prospective, randomized, double blind placebo-controlled trial of glucocorticoids (GCT) in infants of less than 12 months with severe or moderate bronchiolitis. Patients were randomized to receive systemic corticoid therapy (low dose for 7 days) or placebo. The main outcomes were: a) levels of lymphocyte subsets; b) levels of IL-2, IL-12, and IFNγ as pro-inflammatory factors, and c) levels of IL-4 and IL-10 as anti-inflammatory response. Secondary outcomes related with the clinical response were also analyzed. Results: 97 patients were randomized. Evolution of lymphocyte subsets was similar in both groups. Pro-inflammatory interleukins and interferon decreased, but without differences. Anti-inflammatory interleukins showed a significant decrease from baseline to the end of the study, and IL-10 values were significantly lower (p = 0.046) in the GCT group [1.82 pg/ml (1.2-3.5)] vs non-GCT [4 pg/ml (1.5-6.3)]. GCT group showed a lower time of mechanical ventilation and of hospitalization, but without statistically significant differences. No cases of severe adverse reaction to steroids were detected. Conclusions: Administration of systemic GCT did not modify the inflammatory nor the clinical response of patients with severe bronchiolitis, except for IL-10 levels that were significantly lower in the GCT group. This can open a line of investigation about the relation of IL-10 and response to bronchiolitis.

RESUMEN Introducción: Se ha demostrado que los glucocorticoides no mejoran el pronóstico de pacientes con bronquiolitis, pero se podría suponer que reducen la inflamación. Objetivo: Evaluar si los glucocorticoides influyen en la respuesta inflamatoria y clínica de los lactantes críticos. Métodos: Ensayo prospectivo, aleatorizado, doble ciego, controlado con placebo en lactantes < 12 meses con bronquiolitis grave y administración de glucocorticoides sistémicos (dosis bajas 7 días). Se examinaron: a) concentraciones de subconjuntos de linfocitos; b) concentraciones de IL-2, IL-12 e IFNγ como factores proinflamatorios, y c) concentraciones de IL-4 e IL-10 como respuesta antiinflamatoria. También se analizaron los resultados relacionados con la respuesta clínica. Resultados: Se aleatorizaron 97 pacientes. La evolución de los subconjuntos de linfocitos fue similar en ambos grupos. Disminuyeron las interleucinas proinflamatorias y el interferón, pero sin diferencias. Las interleucinas antiinflamatorias mostraron una disminución significativa desde el inicio hasta el final del estudio, y los valores de IL-10 fueron significativamente más bajos (p= 0,046) en el grupo de glucocorticoides [1,82 pg/ml (1,2-3,5)] frente a los no glucocorticoides [4 pg/ml (1,5 - 6,3)]. El grupo glucocorticoides mostró menor tiempo de ventilación mecánica y de hospitalización, pero sin diferencias significativas. No se detectaron reacciones adversas graves a glucocorticoides. Conclusiones: La administración de glucocorticoides sistémicos no modificó la respuesta inflamatoria ni clínica de los pacientes con bronquiolitis severa, excepto las concentraciones de IL-10 que fueron significativamente menores en el grupo de glucocorticoides. Esto puede abrir una línea de investigación sobre la relación de IL-10 y la respuesta a la bronquiolitis.

Terapias dirigidas con inhibidores del braf en gliomas pediátricos de alto y bajo grado con la mutación brafv600e. Experiencia del hospital Prof. Dr. Juan P. Garrahan / Targeted therapies with BRAF inhibitors for pediatric low- and high-grade gliomas with BRAF v600e mutation. Prof. Dr. Juan P. Garrahan Hospital experience

Resumen La mutación puntual V600E del gen BRAF juega un papel fundamental en la tumorigénesis de muchos gliomas. La inhibición de su producto forma parte de terapias innovadoras emergentes en los últimos años. Conocer el rol de estos tratamientos resulta imprescindible. El objetivo del trabajo fue describir la respuesta clínico-radiológica en niños con gliomas BRAF V600E mutado tratados con inhibidores BRAF. Para ello se realizó un estudio descriptivo y retrospectivo en pacientes menores de 16 años con gliomas BRAF V600E mu tado que recibieron vemurafenib o dabrafenib en el Hospital Garrahan. Trece pacientes tratados en los últimos 7 años fueron incluidos: 9 gliomas de bajo grado y 4 de alto grado. La mediana de edad al diagnóstico fue 8.6 años (0.89-14.04) y del comienzo del inhibidor 11.62 años (3.64-15.42). Inicialmente, todos habían realizado tratamiento quirúrgico, y 12/13 recibieron previamente otra terapia: 11 quimioterapia (eventualmente hasta 4 líneas distintas) y 4 radioterapia. Con la terapia dirigida, 10 pacientes tuvieron una disminución tumoral mayor o igual al 25%, quedando evidenciada en 7 niños la mejor respuesta dentro de los 6 meses del inicio. Hubo 4 progresados intratratamiento (todos alto grado), y 2 progresados prontamente luego de suspender el inhibidor (ambos bajo grado). Cinco presentaron efectos adversos grado 3-4, con recuperación ad-integrum. Se describe una buena y sostenida respuesta clínico-radiológica, con tolerancia aceptable, en pacientes con gliomas de bajo grado BRAF V600E mutado tratados con inhibidores BRAF V600E . En contraste, la respuesta en pacientes con gliomas de alto grado fue intermedia y de poca duración, con progresión tumoral precoz.

Abstract The BRAF V600E point mutation plays a key role in the tumorigenesis of many gliomas. Inhibiting its product is part of the innovative therapies emerging in recent years. Knowing the role of these treatments is essential. The aim of this experience was to describe the clinical-radiological response of pediatric BRAF V600E mutated gliomas treated with BRAF inhibitors. To this end, a descriptive and retrospective study was performed in patients under 16 years of age with BRAF V600E gliomas, who received vemurafenib or dabrafenib at Hospital Garrahan. Thirteen patients treated in the last 7 years were included: 9 were low-grade and 4 high-grade gliomas. The median age at diagnosis was 8.6 years (0.89-14.04) and at start of targeted therapy was 11.62 years (3.64-15.42). All patients had previously a surgical procedure, and 12/13 had received another therapy prior BRAF inhibition: 11 chemotherapy (in one case, up to 4 different protocols) and 4 radiotherapy. Under targeted therapy, tumour response was obtained in 10 patients (size reduction equal to or greater than 25%), and best response was observed in the first 6 months of treatment in 7 children. Four patients progressed under treatment (all high-grade gliomas) and 2 progressed shortly after stopping the inhibitor (both low-grade gliomas). Five patients had grade 3-4 toxicity, with subsequent full recovery. A good and sustained clinical-radiological response, with acceptable tolerance, is described in patients with BRAF V600E mutated low-grade gliomas treated with BRAF V600E inhibitors. In contrast, the response in patients with high-grade gliomas was intermediate and of short duration, with early tumour progression.

Diagnostic challenges in paediatric foreign body aspiration: a case report / Desafío diagnóstico en la aspiración de cuerpo extraño en pediatría: reporte de un caso

Abstract Introduction Foreign body aspiration (FBA) is a potentially fatal paediatric emergency. Our objective was to highlight the importance of a multidisciplinary approach to difficult/doubtful diagnosis. Case report 34-month-old girl referred for urgent rigid bronchoscopy after suspected metallic blade ingestion (found chewing on it). She had a previous recurrent history of wheezing. The physical examination revealed face/lip wounds, traces of powder on her teeth but no breathing difficulty. The plain X-Ray revealed radiopaque images of the upper pulmonary field and gastric chamber. In the absence of FBA clinical signs but considering a previous history of bronchial hyperresponsiveness, a direct digital radiographic study was performed. There were no images compatible with foreign bodies: the results were interpreted as artefacts and no bronchoscopy was performed. Conclusions A careful pre-anaesthetic evaluation, a high level of suspicion and excellent multidisciplinary communication led to the recognition of false radiologic findings. A conservative approach was followed and invasive procedures in a remote location, with high anaesthetic risk for the paediatric population were avoided.

Resumen Introducción La aspiración de cuerpo extraño (ACE) es una emergencia pediátrica potencialmente fatal. La intención del presente artículo es resaltar la importancia de un abordaje multidisciplinario en caso de un diagnóstico difícil/dudoso. Reporte de caso Se trata de una paciente de 4 meses de edad remitida para broncoscopia rígida de urgencia, luego de la sospecha de ingestión de una hojilla metálica (se encontró a la bebé mordiéndola). La paciente tenía antecedentes de sibilancia. Al examen físico se encontraron heridas en la cara y los labios, rastros de polvo en los dientes, pero no había dificultad respiratoria. El examen de rayos-x mostraba imágenes radio opacas en el cuadrante superior derecho del pulmón y en la cámara gástrica. En virtud de la ausencia de signos clínicos de ACE en una paciente con antecedentes de hiperresponsividad bronquial, se realizó un estudio radiográfico digital. No hubo imágenes compatibles con cuerpos extraños: los resultados se interpretaron como artefactos y no se realizó la broncoscopia. Conclusiones Un cuidadoso examen pre-anestesia, el alto grado de sospecha y una excelente comunicación multidisciplinaria, permitieron el reconocimiento de hallazgos radiológicos falsos. Se siguió un abordaje conservador, evitando así procedimientos invasivos en lugares remotos con alto grado de riesgo para la población pediátrica.

Psicopatología en lupus eritematoso sistêmico pediátrico / Psychopathology in paediatric systemic lupus erythematosus

RESUMEN Introducción: El lupus eritematoso sistémico (LES) es más frecuente en adultos; sin embargo, cuando se diagnostica antes de los 16 años, es de peor pronóstico. Los síntomas depresivos y ansiosos son frecuentes en esta población, pero pocos estudios examinan la ansiedad o su relación con manifestaciones de la enfermedad o el tipo de tratamiento recibido. Objetivo: Determinar la frecuencia de aparición de síntomas ansiosos y depresivos en niños y adolescentes con LES, así como su relación con la actividad de la enfermedad y el tratamiento inmunosupresor utilizado. Métodos: Estudio de corte trasversal en el que se incluyó a pacientes pediátricos con LES de 9 a 17 años de un centro de referencia pediátrico de la ciudad de Bogotá. Se entrevistó a cada paciente y se exploraron el tratamiento inmunosupresor, el tiempo hasta el diagnóstico y la actividad de la enfermedad. Se midieron con cuestionarios validados la frecuencia de síntomas depresivos y ansiosos y la calidad de vida. Se buscó la relación entre los síntomas ansiosos o depresivos y la actividad de la enfermedad y el tratamiento inmunosupresor. Resultados: Se entrevistó a 40 pacientes, con una media de edad de 14±2 años. El 52% de los casos tenían enfermedad activa. El 60% presentó puntuaciones que indicaban ansiedad y 1 paciente, de depresión. El 37% informó de ideas suicidas tras el diagnóstico de enfermedad lúpica. No se encontró relación entre la actividad de la enfermedad y las manifestaciones psiquiátricas. De los diferentes medicamentos utilizados, solo la prednisona se asoció con puntuaciones más altas de síntomas de ansiedad (p = 0,002). Conclusiones: La ansiedad fue más frecuente que la depresión en los pacientes con LES pediátrico. Este trastorno y la utilización de corticoides fueron factores de vulnerabilidad a sufrir psicopatología. La actividad lúpica no se relaciona con síntomas psiquiátricos. El LES tiene un gran impacto en la calidad de vida de los pacientes.

ABSTRACT Introduction: Although systemic lupus erythematosus (SLE) is more common in adults, when it is diagnosed before the age of 16, it has a worse prognosis. Depressive and anxious symptoms are common in this population but few studies have examined anxiety or its relationship with manifestations of the disease or type of treatment received. Objective: To determine the frequency of anxious and depressive symptoms in children and adolescents with SLE, as well as their relationship with disease activity and the immuno-suppressive treatment used. Methods: A cross-sectional study in which paediatric patients with SLE from 9 to 17 years of age from a paediatric referral centre in the city of Bogota were included. Each patient was interviewed and immunosuppressive treatment, time to diagnosis and disease activity were recorded. The frequency of depressive and anxious symptoms and quality of life were measured with validated questionnaires. The relationship between anxious or depressive symptoms and disease activity and immunosuppressive treatment was sought. Results: 40 patients with a mean age of 14 ± 2 years were interviewed. 52% of cases had active disease. 60% presented scores indicating anxiety and one patient a score suggesting depression. 37% reported suicidal thoughts following the diagnosis of SLE. No relationship was found between disease activity and psychiatric manifestations. Of the different drugs used, only prednisone was associated with higher anxiety symptom scores (p =0.002). Conclusions: Anxiety was more prevalent than depression in patients with paediatric SLE. This disorder and the use of corticosteroids were factors of vulnerability to manifest psychopathology. Lupus activity is not related to psychiatric symptoms. SLE has a significant impact on the quality of life of patients.

Pleasure and suffering in the work of nurses at the oncopediatric hospital unit: qualitative research / Placer y sufrimiento en el trabajo de los enfermeros de la unidad de internación oncopediátrica: investigación cualitativa / Prazer e sofrimento no trabalho dos enfermeiros da unidade de internação oncopediátrica: pesquisa qualitativa

ABSTRACT Objective: To analyze the experiences that generate pleasure and suffering in the work of nurses in an oncopediatric inpatient unit. Methods: Qualitative, exploratory, descriptive study. Data collection took place from March to May 2018, through interviews with eight nurses in an oncopediatric inpatient unit. Results: The verbalized experiences of pleasure were the work developed with children, participation in the treatment and cure of patients and the recognition of family members in relation to the work performed. The suffering experiences were palliative care and death of patients, performing invasive procedures and organizing work. Final considerations: It was possible to analyze the experiences of pleasure and suffering of nurses in an oncopediatric unit through the psychodynamics of work, allowing to reinvent prevention and intervention measures by managers in health/mental illness processes at work.

RESUMEN Objetivo: Analizar las vivencias generadoras de placer y sufrimiento en el trabajo de enfermeros en una unidad de internación oncopediátrica. Método: Estudio cualitativo, del tipo exploratorio-descriptivo. La recogida de datos ocurrió de marzo a mayo de 2018, por medio de entrevistas con ocho enfermeros en una unidad de internación oncopediátrica. Resultados: Las vivencias de placer verbalizadas han sido el trabajo desarrollado con niños, la participación en el tratamiento y cura de los pacientes y el reconocimiento de los familiares en relación al trabajo realizado. Las vivencias de sufrimiento han sido los cuidados paliativos y muerte de los pacientes, realización de procedimientos invasivos y organización del trabajo. Consideraciones finales: Ha sido posible analizar las vivencias de placer y sufrimiento de enfermeros en una unidad oncopediátrica por medio de la psicodinámica del trabajo, permitiendo reinventar medidas de prevención e intervención de los gestores en los procesos de salud/enfermedad mental en el trabajo.

RESUMO Objetivo: Analisar as vivências geradoras de prazer e sofrimento no trabalho de enfermeiros em uma unidade de internação oncopediátrica. Método: Estudo qualitativo, do tipo exploratório-descritivo. A coleta de dados ocorreu de março a maio de 2018, por meio de entrevistas com oito enfermeiros em uma unidade de internação oncopediátrica. Resultados: As vivências de prazer verbalizadas foram o trabalho desenvolvido com crianças, a participação no tratamento e cura dos pacientes e o reconhecimento dos familiares em relação ao trabalho realizado. As vivências de sofrimento foram os cuidados paliativos e morte dos pacientes, realização de procedimentos invasivos e organização do trabalho. Considerações finais: Foi possível analisar as vivências de prazer e sofrimento de enfermeiros em uma unidade oncopediátrica por meio da psicodinâmica do trabalho, permitindo reinventar medidas prevenção e intervenção dos gestores nos processos de saúde/doença mental no trabalho.

Construction of competency model for outpatient nurses in pediatrics / 中国实用护理杂志

Objective:To explore the scientific and practical modeling method and construct the post competency model of pediatric outpatient nurses to provide reference for the recruitment, training and selection of pediatric outpatient nurses.Methods:Competency factors were extracted by literature review, behavioral event interview, exploratory common factor molecular method and Delphi method, and the weight of each factor was determined by AHP and expert scoring method.Results:6 items of differential competence were obtained, and the final competency model of pediatric outpatient post was formed, including personal characteristics, professional accomplishment, development ability, professional knowledge, professional technology and management ability. The weight range was 0.107 4-0.210 1, 39 secondary indexes, the weight range was 0.086 4-0.256 5, and the combined weight range was 0.009 9-0.053 9.Conclusion:The modeling method is scientific and reasonable, the model is reliable and practical, which provides the basis for the admission, training and performance appraisal of pediatric outpatient nurses, and has theoretical and practical significance.

Tangled Throughout Life: A Case of Paediatric Ruptured Cerebral Arteriovenous Malformation

@#Arteriovenous malformation (AVM) in children is uncommon occurrence defined as presence of arteriovenous shunting through coiled and tortuous vascular connections. We discussed a case of a 3-year old girl presented with acute left facial asymmetry and right-sided limb weakness. Neurological examination revealed MRC scale of 0 out of 5 for power on her right side. Magnetic Resonance Angiography (MRA) revealed bilateral thalamic AVM. Surgical resection was not advisable in view of deep-seated location. Paediatric AVM most often become apparent following rupture with majority presents with headache. Ruptured paediatric AVM carries high burden of morbidity and mortality. Paediatric intracranial haemorrhage posed tremendous concern regarding its long-term outcome. Treatment would be more appropriate sooner rather than later especially for those presented with ruptured AVM. Surgical resection remains the gold standard treatment for all accessible paediatric AVMs with embolization and radiosurgery as adjunctive therapies. AVM in paediatric population is rare but carries grim prognosis.

Clinical Characteristic of Paediatric Palliative Care Cases in North Eastern of Peninsular Malaysia

@#Introduction: Paediatric Palliative care has been a relatively new specialty in Malaysia. It serves to cater hospital and community support for children with life-limiting illnesses (LLI). The aim of the study is to describe the clinical characteristic of Paediatric Palliative Care (PPC) cases in the North East of Peninsular Malaysia. Methods: Children with LLI were identified through 2 databases captured by the ‘Sayangku’ Homecare team and Paediatric Palliative Care (PPC) team. These databases include the details on clinical diagnosis, time of referral and outcome. Children included were those referred from November 2012 to March 2020 to PPC service. The number of new cases referred per annum (2019) was compared to the crude estimation of prevalence. Results: A total of 216 children were included. About half of the children with LLI (50.5%) were from group 4 and about a quarter from group 1 (27%) of the ACT/ RCPCH classification. Approximately one third of our cohort (31%) had died, majority from oncology causes. There were 116 neurology cases, 60 oncology cases and further 48 cases were the remaining cases in our databases. More than one third (37.5%) of the patients were in the age range of 11-18-year-old. The estimated cases under PPC was only 7.7% from the estimated annual prevalence of LLI. Conclusion: The current data showed that many of the LLI cases in our area have gone underreported. There is a need to disseminate information and awareness among the medical fraternity on the patient’s eligibility for the PPC service.

Subcapital Femoral Neck Tension Stress Fracture - A Rare Injury in A Child: A Case Report

@#Femoral neck stress fractures are rare in children. To the best of our knowledge, the tension type stress fracture has been reported only twice in the English language literature. We report on a five years follow-up of a 10-year-old boy with this injury which was initially missed. The fracture healed after screw fixation. We highlight the importance of considering stress fracture as a differential diagnosis in a child with chronic hip pain. A careful physical examination and the appropriate imaging will avoid missing the diagnosis.

Faulty traditional practices in infant care / Prácticas tradicionales defectuosas en el cuidado infantil

Proper advice regarding home-based care of normal infants is no less important than managing critical diseases. Otherwise parents tend to follow traditional practices running in the family which are often deleterious. Clinicians must also be aware of the faulty traditional practices so that they can actively enquire them in the child's history.The author enlisted various such faulty traditional practices as experienced by him during his OPD practice in various public and corporate hospitals over a period of five years. Topics relating mainly to the infant age group have been chosen for this article. Relevant search was done on these topics in standard paediatric textbooks, journals, and websites (like WHO, NICE, AAP) to find the validity of these practices as well as the standard recommendations on these topics.It was found that the standard recommendations often differ from the traditional practices. Detailed text was included from various authentic sources regarding few dubious topics related to home-based infant care.Clinicians should be aware of the correct recommended methods regarding the day to day home-based infant care. They should counsel the parents accordingly so as to avoid any harm caused by faulty traditional practices and social beliefs.

El asesoramiento adecuado sobre la atención domiciliaria de los bebés normales no es menos importante que el manejo de enfermedades críticas. De lo contrario, los padres tienden a seguir las prácticas tradicionales de la familia que a menudo son perjudiciales. Los médicos también deben ser conscientes de las prácticas tradicionales defectuosas para poder investigarlas activamente en la historia del niño.El autor reclutó varias prácticas tradicionales defectuosas que experimentó durante su práctica en OPD en varios hospitales públicos y corporativos durante un período de cinco años. Para este artículo se han elegido temas relacionados principalmente con el grupo de edad infantil. Se realizaron búsquedas relevantes sobre estos temas en libros de texto, revistas y sitios web estándar de pediatría (como OMS, NICE, AAP) para encontrar la validez de estas prácticas, así como las recomendaciones estándar sobre estos temas.Se encontró que las recomendaciones estándar a menudo difieren de las prácticas tradicionales. Se incluyó texto detallado de varias fuentes auténticas con respecto a algunos temas dudosos relacionados con el cuidado infantil en el hogar.Los médicos deben conocer los métodos correctos recomendados con respecto al cuidado infantil diario en el hogar. Deben asesorar a los padres en consecuencia para evitar cualquier daño causado por prácticas tradicionales y creencias sociales defectuosas.