Abstract Asthma symptoms can be controlled through proper self-management, medication adherence, and avoidance of triggers, thus avoiding high costs for multiple hospitalizations, emergency or outpatient visits. This article seeks to develop a conceptual framework of the factors that influence asthma self-management in a pediatric population living in the Andean urban area of Cuenca-Ecuador. A phenomenological study was carried out (February-April 2021) in 10 children and adolescents from 7 to 17 years old with asthma attending public and private hospitals. In-depth interviews were applied using open questions created after reviewing the literature. Factors influencing asthma self-management include individual (attitudes, self-education, seeking medical attention, managing crisis triggers) and environmental (health system, school/family support). The interviews were audio-recorded, transcribed, and analyzed by two independent researchers. Our results show that the pediatric population identifies individual and environmental influences that affect asthma self-management, with age and socioeconomic status moderating the influential factors. The participants described the beliefs, attitudes, practices, perceptions, and specific experiences in the family, school, and health system contexts as relevant to comply with asthma self-management. In addition, problems or barriers that hinder the self-management of the disease, mainly related to adherence to treatment, were identified. In conclusion, our analysis highlights the need to empower the pediatric population in asthma self-management. When delivering patient-centered education for effective asthma management, future interventions must consider children´s age and socioeconomic status influences in individual and environmental factors.

RESUMEN La neurodegeneración con acumulación de hierro en el cerebro es un grupo raro de enfermedades neurodegenerativas que presenta síntomas extrapiramidales, trastornos del estado de ánimo y deterioro cognitivo. Se estima una prevalencia de 1 a 2 casos por millón de habitantes. En América Latina no existe una prevalencia estimada, aunque existen algunos casos reportados con presentación típica. Se presenta el primer reporte de caso de esta enfermedad en el Ecuador, en una presentación atípica, bajo el descarte de diferenciales y cumplimiento de criterios diagnósticos.

ABSTRACT Neurodegeneration with brain iron accumulation is a rare group of neurodegenerative diseases that present extrapyramidal symptoms, mood disorders, and cognitive impairment. A prevalence of 1 to 2 cases per million inhabitants is estimated. In Latin America, there is no estimated prevalence, although there are some reported cases with typical presentation. The first case report of this disease in Ecuador is presented, in an atypical presentation, under the ruling out of differentials and compliance with diagnostic criteria.

Resumo Este estudo tem como objetivo analisar a associação entre a adesão à alimentação escolar e a coocorrência do consumo regular de marcadores de alimentação saudável e não saudável entre adolescentes brasileiros. Foram avaliados 67.881 adolescentes de escolas públicas brasileiras participantes da Pesquisa Nacional de Saúde do Escolar (PeNSE) de 2015. A partir do QFA de sete dias, construiu-se a variável dependente, coocorrência do consumo regular (≥ 5x/semana) de marcadores de alimentação saudável e não saudável, que foi categorizada em consumo regular de nenhum; um ou dois; ou três marcadores de alimentação. Realizou-se regressão logística ordinal com ajuste para variáveis sociodemográficas, hábitos alimentares fora da escola e características da escola. A prevalência da coocorrência do consumo regular de três marcadores de alimentação saudável foi de 14,5%, e de três marcadores de alimentação não saudável foi de 4,9%. A alta adesão à alimentação escolar (todos os dias) foi positivamente associada ao consumo regular de marcadores de alimentação saudável e inversamente associada ao consumo regular de marcadores de alimentação não saudável. A alimentação escolar fornecida pelo PNAE contribui para a promoção de hábitos alimentares saudáveis entre os adolescentes brasileiros.

Abstract The aim of this study was to analyze the association between adherence to school meals and the co-occurrence of the regular consumption of healthy and unhealthy eating markers among Brazilian adolescents. Data from 67,881 adolescents in Brazilian public schools who participated in the 2015 National School Health Survey, were used. From the 7-day FFQ, the dependent variable was constructed, co-occurrence of regular consumption (≥ 5x/week) of healthy and unhealthy food markers, which was categorized as regular consumption of none, one or two, or three eating markers. We performed an ordinal logistic regression with adjustment for sociodemographic, eating habits outside of school, and school characteristics variables. The prevalence of the co-occurrence of the regular consumption of three healthy eating markers was 14.5%, and that of three unhealthy markers was 4.9%. High adherence to school meals (every day) was positively associated with regular consumption of healthy eating markers and inversely associated with regular consumption of unhealthy eating markers. The school meals provided by PNAE contribute to the promotion of healthy eating habits among Brazilian adolescents.

Abstract The aim of this study was to analyze the association between adherence to school meals and the co-occurrence of the regular consumption of healthy and unhealthy eating markers among Brazilian adolescents. Data from 67,881 adolescents in Brazilian public schools who participated in the 2015 National School Health Survey, were used. From the 7-day FFQ, the dependent variable was constructed, co-occurrence of regular consumption (≥ 5x/week) of healthy and unhealthy food markers, which was categorized as regular consumption of none, one or two, or three eating markers. We performed an ordinal logistic regression with adjustment for sociodemographic, eating habits outside of school, and school characteristics variables. The prevalence of the co-occurrence of the regular consumption of three healthy eating markers was 14.5%, and that of three unhealthy markers was 4.9%. High adherence to school meals (every day) was positively associated with regular consumption of healthy eating markers and inversely associated with regular consumption of unhealthy eating markers. The school meals provided by PNAE contribute to the promotion of healthy eating habits among Brazilian adolescents.

Resumo Este estudo tem como objetivo analisar a associação entre a adesão à alimentação escolar e a coocorrência do consumo regular de marcadores de alimentação saudável e não saudável entre adolescentes brasileiros. Foram avaliados 67.881 adolescentes de escolas públicas brasileiras participantes da Pesquisa Nacional de Saúde do Escolar (PeNSE) de 2015. A partir do QFA de sete dias, construiu-se a variável dependente, coocorrência do consumo regular (≥ 5x/semana) de marcadores de alimentação saudável e não saudável, que foi categorizada em consumo regular de nenhum; um ou dois; ou três marcadores de alimentação. Realizou-se regressão logística ordinal com ajuste para variáveis sociodemográficas, hábitos alimentares fora da escola e características da escola. A prevalência da coocorrência do consumo regular de três marcadores de alimentação saudável foi de 14,5%, e de três marcadores de alimentação não saudável foi de 4,9%. A alta adesão à alimentação escolar (todos os dias) foi positivamente associada ao consumo regular de marcadores de alimentação saudável e inversamente associada ao consumo regular de marcadores de alimentação não saudável. A alimentação escolar fornecida pelo PNAE contribui para a promoção de hábitos alimentares saudáveis entre os adolescentes brasileiros.

Resumen Introducción: La enfermedad de Gorham-Stout es un trastorno poco frecuente caracterizado por malformaciones linfáticas localizadas sobre estructuras óseas que causan osteólisis progresiva. La proliferación de células endoteliales linfáticas depende de factores de crecimiento que utilizan la vía de la fosfoinositida-3 cinasa (PI3K)/Akt y convergen en la vía de la molécula diana de rapamicina de los mamíferos (mTOR). Este conocimiento ha permitido el tratamiento de esta enfermedad con inhibidores de esta vía como sirolimus o everolimus. Caso clínico: Se presenta el caso de una paciente de sexo femenino de un año referida a nuestra institución tras presentar fractura de fémur derecho y aumento de volumen de dicha extremidad posterior a un traumatismo. Después de diversos episodios de infecciones de tejidos blandos se realizaron estudios de imagen que mostraron pseudoartrosis, lesiones líticas y ausencia total del fémur derecho, así como una biopsia de fémur que mostró estructuras vasculares positivas con tinción D2-40, diagnosticándose enfermedad de Gorham-Stout. Durante su abordaje, se realizó la desarticulación de la extremidad a nivel de la cadera y se inició tratamiento con sirolimus oral, presentando una mejoría clínica y radiológica con menores lesiones líticas y evidencia de osificación posterior a 20 meses de tratamiento. Conclusiones: El tratamiento con sirolimus oral para la enfermedad de Gorham-Stout inhibe la actividad osteoclástica y la angiogénesis, estimulando el anabolismo óseo que resulta en la detención de la progresión de la osteólisis y una mejoría en la osificación, la calidad de vida y el pronóstico del paciente. Por tal motivo, el sirolimus debe considerarse como una opción terapéutica para esta enfermedad.

Abstract Background: Gorham-Stout disease (GSD) is a rare syndrome characterized by lymphatic malformations, mainly in bone structures, causing progressive osteolysis. Lymphatic endothelial cell proliferation depends on several growth factors that use the phosphoinositide-3 kinase (PI3K)/Akt pathway and converge on the mammalian target molecule of the rapamycin (mTOR) pathway. These findings have allowed treating GSD with mTOR pathway inhibitors such as sirolimus or everolimus. Case report: We present the case of a one-year-old female patient referred to our institution after a right femur fracture and progressive limb volume increase, disproportionately to the trauma. After several episodes of soft tissue infections, imaging studies showed pseudarthrosis, lytic lesions, and progressive loss of the right femur that ended in total absence. A femur biopsy showed lymphatic structures positive with D2-40 staining, diagnosing GSD. After six months of non-response to traditional treatments, the limb was disarticulated at the hip level, and oral sirolimus treatment was initiated, showing clinical and radiological improvement with minor lytic lesions and evidence of ossification after 20 months of treatment. Conclusions: Oral sirolimus treatment for GSD inhibits angiogenesis and osteoclastic activity, stimulating bone anabolism and leading to arrested osteolysis progression and improved ossification, quality of life, and patient prognosis. Therefore, sirolimus should be considered a therapeutic option for this rare disease.

ABSTRACT Objectives: Congenital hypothyroidism (CH) can be permanent (PCH) or transient (TCH). While the importance of thyroxine in myelination of the brain is undisputed, the benefits to neurodevelopmental outcomes of TCH treatment are controversial. Our objectives were to determine predictive factors for PCH and verify its prevalence changes over time. Subjects and methods: A total of 165 children were evaluated at 3 years of age to verify the diagnosis of PCH. 130 were submitted to a two-step cluster analysis, with the aim of grouping them into homogeneous clusters. The mean incidence of PCH and TCH was calculated from 2004 to 2010 and 2011 to 2015. Results: Sixty-six children were diagnosed with PCH, and 99 were diagnosed with TCH. Eighty-one percent of PCH children and all TCH children with thyroid imaging had glands in situ. Eighty children (61.5%) were in Cluster 1, 8 children (6.2%) were in Cluster 2 and 42 children (32.3%) were in Cluster 3. No children had PCH in Cluster 1, while 87.5% of children in Cluster 2 and all children in Cluster 3 had PCH. The most important predictor for PCH was the initial serum TSH, which was marginally higher in importance than the blood spot TSH, followed by the initial serum free T4. The mean incidence of PCH (odds ratio: 1.95, 95% CI 1.36 to 2.95, p < 0.0001) and TCH (odds ratio 1.33, 95%, CI 1.02 to 1.77, p = 0,038) increased over time. Conclusions: The most important PCH predictors are the initial serum TSH and the blood spot TSH. The mean incidence of both PCH and TCH in our series increased.

Abstract Introduction Post-COVID-19 syndrome is characterized by chronic fatigue and myalgia, among other symptoms, which can limit activities of daily living. Physical therapy protocols with multicomponent exercises combine strength, resistance, balance and gait, producing sig-nificant improvements in functional mobility. Objective Evaluate whether multicomponent rehabilitation is effective in improving functional mobility and quality of life in individuals with post-COVID-19 syndrome. Methods Randomized controlled trial with 59 volunteers, divided into an intervention (IG) and control group (CG), all of whom underwent initial assessment (T0). The IG performed 24 sessions over 12 weeks and the CG did not undergo training. Functional mobility was analyzed using the 6-minute walk test (6MWT) and the Berg balance scale, while the SF-36 questionnaire assessed quality of life. Results In the 6MWT, the IG covered 464.40 + 81.26 meters (T0) and 518.60 + 82.68 meters (T4). The IG Berg scale scores were 48.00 + 4.00 (T0) and 51.90 + 4.26 (T4). In terms of quality of life, the IG obtained mean scores of 96.26 + 10.14 (T0) and 102.60 + 5.53 (T4). None of these measures was statistically significant. Conclusion Individuals who underwent the multicomponent phys-iotherapy protocol showed statistically nonsignificant increases in the variables studied.

Resumo Introdução A síndrome pós-COVID-19 caracteriza-se por fadiga crônica, mialgia, entre outros sintomas que podem causar limitações na realização das atividades de vida diária. Protocolos de fisioterapia com exercício de multicomponentes combinam força, resistência, equi-líbrio e marcha, e produzem melhorias significativas na mobilidade funcional. Objetivo Avaliar se a reabilitação de multicomponentes é eficaz em promover melhora na mobilidade funcional e qualidade de vida em indivíduos com síndrome pós-COVID-19. Métodos Ensaio clínico randomizado, controlado, realizado com 59 voluntários, divididos em grupo intervenção (GI) e grupo controle (GC). Todos fizeram avaliação inicial (T0); GI realizou 24 sessões durante 12 semanas e GC não realizou treinamento. A análise da mobilidade funcional foi realizada através do teste de caminhada de 6 minutos (TC6m) e da escala de equilíbrio de Berg. O questionário SF-36 avaliou a qualidade de vida. Resultados A distância percorrida no TC6m pelo GI foi de 464,40 + 81,26 metros (T0) e de 518,60 + 82,68 metros (T4). A pontuação na escala de Berg do GI foi de 48,00 + 4,00 (T0) e de 51,90 + 4,26 (T4). Na qualidade de vida, GI apresentou escore com média de 96,26 + 10,14 (T0) e 102,60 + 5,53 (T4). Nenhuma destas medidas foram estatisticamente significantes. Conclusão Os indivíduos que foram submetidos ao protocolo fisioterapêutico de multicomponentes apresentaram incrementos nas variáveis estudadas sem significância estatística.

Abstract: In the literature Informed consent (IC) assumptions is well established. However, the different stages and the conditions under which the IC for anesthetic practices is obtained, is scarce. The aim of the present study is to explore the phases and conditions of IC in anesthesiology. Anonymized clinical records of 325 patients submitted to anesthetic procedures at the Institute of Oncology of Porto were analyzed. A total agreement between the anesthetic techniques established in the IC and those performed, was reach with 270 patients. The importance of IC in clinical practice is discussed and an ideal process for IC is argued.

Resumen: El consentimiento informado (CI) está bien establecido en la literatura. Sin embargo, la información sobre las diferentes fases y condiciones en las que se obtiene el CI para las prácticas anestésicas es escasa. El objetivo del presente estudio es explorar las fases y condiciones de obtención de la CI en anestesiología. Se analizaron las historias clínicas anónimas de 325 pacientes sometidos a procedimientos anestésicos en el Instituto de Oncología de Oporto. Se alcanzó una concordancia total entre las técnicas de anestesia establecidas en el CI y las realizadas con 270 pacientes. Se defiende la importancia del CI en la práctica clínica y se discute un proceso ideal para obtenerlo.

Resumo: Na literatura o Consentimento Informado (CI) é bem estabelecido. Contudo, a informação sobre as diferentes fases e as condições em que o CI para práticas anestésicas é obtido, é escassa. O objetivo do presente estudo é explorar as fases e condições da obtenção do CI em anestesiologia. Foram analisados os registos clínicos anónimos de 325 pacientes submetidos a procedimentos anestésicos no Instituto de Oncologia do Porto. Foi alcançado um acordo total entre as técnicas anestésicas estabelecidas no CI e as realizadas, com 270 pacientes. A importância do CI na prática clínica é defendida e discute-se um processo ideal para a obtenção do CI.

Abstract Lawsuits due to patient perception of inappropriate medical actions are a growing reality in medical practice, which entails widespread concern in the medical community. Lawsuits often entail additional circumstances beyond the primary concern of preventing or sanctioning acts of medical negligence. CETREMI proposes various recommendations aimed at legal and medical professionals to improve this circumstance and avoid harming the doctor-patient relationship.

Resumen Las demandas judiciales por la percepción del paciente de una actuación médica inadecuada son una realidad creciente en la práctica médica, la cual entraña una preocupación extendida en el gremio médico. Las demandas judiciales frecuentemente conllevan circunstancias adicionales a la primaria preocupación de prevenir o sancionar actos de negligencia médica. CETREMI emite algunas recomendaciones a los profesionales jurídicos y médicos para mejorar esta situación y evitar daños en la relación médico-paciente.

ABSTRACT Coronary artery fistula draining into the coronary sinus is a rare vascular malformation, and its diagnosis and clinical manifestations usually occur late. We describe the case of a 72-year-old female patient with dyspnea on exertion (New York Heart Association Class III) associated with palpitations. The transthoracic echocardiogram showed significant tricuspid insufficiency. Cardiac catheterization showed aneurysm of the circumflex coronary artery and fistula of this artery draining into the coronary sinus. The patient underwent fistula ligation and tricuspid valve repair, with excellent surgical results.

Abstract: The local anesthesics are defined as medicaments that block the generation and spread of impulses in excitable fabrics, from bony marrow, nervous roots, peripheral nerves or other excitable fabrics as cardiac muscle, smooth muscle and brain. The systemic intoxication takes place due to the plasmatic concentrations raised after high doses or the intravenous inadvertent administration. The increasing use of technologies of anesthesia locorregional forces to bear in mind the systemic intoxication for local anesthesics as an anesthesic complication of low incident, but high morbi-mortality, beside constituting one of the more frequent reasons of cardiorespiratory unemployment of anesthesic origin. The clinical presentation of this complication is very variable and includes a great spectrum of symptoms related principally to the neurological and cardiovascular toxicity. Though infrequent, the reactions can be very serious, and to result in irreversible hurt or death of the patient. The prevention seems to have diminished the poisoning of the local anesthesics and is more effective than the treatment. The managing is based on measures of resuscitation cardiopulmonar advanced, the pharmacological treatment and the precocious employment of the lipid emulsions. I present a case of systemic intoxication using lidocaine simply as anesthesic local only one during the accomplishment of blockade of peripheral nerve in elective surgery.

Resumen: Los anestésicos locales se definen como fármacos que bloquean la generación y propagación de impulsos en tejidos excitables desde médula ósea, raíces nerviosas, nervios periféricos hasta otros tejidos excitables como músculo cardíaco, músculo liso y cerebro. La intoxicación sistémica se produce debido a las concentraciones plasmáticas elevadas después de altas dosis o la administración intravenosa inadvertida. El creciente uso de técnicas de anestesia locorregional obliga a tener presente la intoxicación sistémica por anestésicos locales como una complicación anestésica de baja incidencia, pero alta morbimortalidad, además de constituir una de las causas de paro cardiorrespiratorio de origen anestésico más frecuentes. La presentación clínica de esta complicación es muy variable y abarca un gran espectro de síntomas relacionados principalmente con la toxicidad neurológica y cardiovascular. Aunque infrecuentes, las reacciones pueden ser muy graves, y resultar en daño irreversible o muerte del paciente. La prevención parece haber disminuido la intoxicación de los anestésicos locales y es más efectiva que el tratamiento. El manejo se basa en medidas de reanimación cardiopulmonar avanzada, el tratamiento farmacológico y el empleo precoz de las emulsiones lipídicas. Se presenta un caso de intoxicación sistémica utilizando lidocaína simple como único anestésico local durante la realización de bloqueo de nervio periférico en cirugía electiva.