Factores relacionados con la supervivencia en una cohorte de pacientes con enfermedad pulmonar obstructiva crónica grave a largo plazo: relación con la capacidad aeróbica / Related Factors for Survival in a Cohort of Patients with Severe Chronic Obstructive Pulmonary Disease in a Long-Term of Follow-Up: Relationship with Aerobic Capacity

Introducción: Sólo Oga et al. (AJRCCM 2003) relacionaron supervivencia y capacidad aeróbica en pacientes EPOC, pero en hombres y seguimiento a 5 años. Objetivos: Evaluar la supervivencia de una cohorte de pacientes EPOC grave según el consumo de oxígeno pico (VO2p) ajustado al peso. Material y Métodos: Se evaluó la supervivencia a largo plazo desde el diagnóstico de pacientes con EPOC (GOLD). Se midió el VO2p ajustado por peso en prueba cicloergo- métrica máxima (gases espirados). Se usaron técnicas estadísticas convencionales y análisis de supervivencia de LogRank (Mantel-Cox). Resultados: 70 pacientes (27% femenino); edad 68 años (RIQ 63-73); %FEV1 postBD: 39,95±2,09; VO2p: 9,25 ± 3,17 ml/kg/min. GOLD D/B/A 84,3/14,2/1,5%; GOLD II/III/IV: 15,7/61,4/22,9%. A 14 años de seguimiento, 75% había fallecido. Supervivencia: primer cuartilo de VO2p (ml/kg/min) fue 38,5 meses (RIQ 18,25-58,5) y para el cuarto cuartilo 68 meses (RIQ 48-93). A 103 meses, la diferencia en supervivencia fue: primer cuartilo vs. cuarto cuartilo de VO2p (p<0,01) y segundo vs. cuarto cuartilo (p<0,03); a 145 meses entre segundo vs. cuarto cuartilo (p=0,049). En el análisis multivariado, el VO2p alto es un factor protector sobre la mortalidad. En cambio, otras variables independientes como sexo masculino, edad >70, grado de obstrucción bronquial severo y fenotipo exacerbador frecuente se asociaron a mortalidad. Conclusión: A largo plazo, en una cohorte de pacientes hombres y mujeres EPOC grave, en análisis multivariado, el VO2p alto es factor protector sobre la mortalidad. En cambio, otras variables independientes como sexo masculino, edad >70, grado de obstrucción bronquial severo y exacerbador frecuente se asociaron a mortalidad.

Introduction: Only Oga et al. (AJRCCM 2003) related survival and aerobic capacity, but only in chronic obstructive pulmonary disease (COPD) men with 5 years of follow-up. Objective: To determine survival in a cohort of patients with severe COPD due to aerobic capacity (VO2max) adjusted by weight. Methods: Survival of COPD patients was evaluated to long-term (GOLD definition). Patients performed maximal exercise test in cicloergometry (expired gases) evaluating (VO2max). Conventional statistics and Log-Rank survival analysis (Mantel-Cox) were used. Results: We included 70 patients (27% female) followed up 60.77 months (RIQ 29- 87.85); age 68 years (RIQ 63-73); %FEV1 postBD: 39.95±2.09; VO2p: 9.25± 3.17 ml/kg/ min. GOLD D/B/A 84.3/14.2/1.5%; GOLD II/III/IV: 15.7/61.4/22.9%. After 14 years of follow-up, 75% of patients died. Survival: VO2p (ml/kg/min) first quartil was 38.5 months (RIQ 18,25-58,5); second quartil 66 months (RIQ 35-84.5); third quartil 70 months (RIQ 15-96) and fourth quartil 68 months (RIQ 48-93). After 103 months of follow-up, survival was compared: 1st vs 4rd quartil of VO2p (p<0.01) and 2nd vs. 4rd quartil (p<0.03); comparing at 145 months: 2nd vs. 4rd quartil (p=0.049). In a multivariate analysis, high VO2p is a protective factor on mortality, nevertheless other independent variables as male gender, age >70, severe airway obstruction and frequent exacerbators were associated to mortality. Conclusion: At long term of follow-up, a cohort of severe COPD patients (males and fe- males), in multivariate analysis, high VO2p is a protective factor of mortality, nevertheless other independent variables as male gender, age >70, severe airway obstruction and frequent exacerbators were associated to mortality.

¿Cómo llega el paciente asmático al servicio de emergencias? Datos del estudio multicéntrico SABINA EMERGENCIAS / How Does the Asthmatic Patient Arrive at the Emergency Department? Data from the SABINA EMERGENCIAS Multicenter Study

Introducción: Los objetivos del control del asma son prevenir la aparición de síntomas y reducir el riesgo de exacerbaciones y mortalidad mediante educación médica, técnica inhalatoria, adherencia a medicación controladora e indicación de planes de acción (PA); pero los pacientes enfrentan exacerbaciones de diversa gravedad. Objetivos: El objetivo principal del estudio SABINA EMERGENCIAS fue describir la forma en que los pacientes concurren al servicio de emergencias (SE), considerando la frecuencia y uso de medicación de rescate. Objetivos secundarios: consultas al SE; uso de corticoides sistémicos (CS), agonistas beta-2 de acción corta (SABA) y tratamiento controlador; disponibilidad de PA. Material y Métodos: Estudio transversal, observacional, descriptivo, en cuatro hospitales del área metropolitana de Buenos Aires en adultos con asma. Resultados: n=323 (edad: 43,7±16,8 años; mujeres: 66,6%): 61,3% no eran seguidos por especialistas; 90,1% utilizaron SABA como rescate (mediana:10 inhalaciones; rango 0-100) la semana previa; 75,9% tuvieron ≥1 consulta al SE el año previo (mediana:2 [0-100]); 29,4% habían sido hospitalizados; 59,1% recibieron ≥1 ciclo de CS; mediana de consumo de SABA: 3 envases/año (0-23); 51,7% habían utilizado ≥3 envases; 30% no empleaban tratamiento de mantenimiento (23% usaba SABA); 75,9% no efectuaban terapia regular de mantenimiento; 77,1% no contaban con PA. Conclusión: Una reducida proporción de pacientes asmáticos que concurren al SE son seguidos por médicos especialistas, con alto consumo y elevada frecuencia de aplicación de SABA como rescate y baja adherencia al tratamiento de mantenimiento. Se remarca la necesidad de optimizar el manejo, con énfasis en la derivación al especialista, adherencia al tratamiento y prescripción de PA.

Introduction: The objectives of asthma control are to prevent the onset of symptoms and reduce the risk of exacerbations and mortality through medical education, inhaler technique, adherence to controller medication and indication of action plans (AP); but patients experience exacerbations of varying severity. Objective: The main objective of the SABINA EMERGENCIAS study was to describe how patients attend the emergency department (ED), considering the frequency and use of rescue medication. Secondary objectives: ED visits; use of systemic corticosteroids (SC), short-acting beta-2 agonists (SABA) and controller therapy; availability of AP. Methods: Cross-sectional, observational, descriptive study in 4 hospitals in the metropolitan area of Buenos Aires in adults with asthma. Results: n=323 (age:43.7±16.8 years; women:66.6%): 61.3% were not followed by specialists; 90.1% used SABA as rescue medication (median:10 puffs; range 0-100) the previous week; 75.9% had ≥1 visit to the ES the previous year (median: 2 [0-100]); 29.4% had been hospitalized; 59.1% received ≥1 cycle of CS; median SABA consumption: 3 cannisters/year (0-23); 51.7% had used ≥3 cannisters; 30% did not use maintenance therapy (23% used SABA); 75.9% did not perform regular maintenance therapy; 77.1% did not have an AP. Conclusion: A small proportion of asthmatic patients attending the ES are followed by specialist physicians, with high consumption and high frequency of SABA application as rescue medication and low adherence to maintenance treatment. The need to optimize management is highlighted, with emphasis on referral to specialists, adherence to treatment and prescription of APs.

Sarcoma de Ewing extraesquelético: reporte de caso / Extraskeletal Ewing's Sarcoma: a Case Report

Introducción: El sarcoma de Ewing es un tumor maligno de alto grado con localización principalmente ósea; se han reportado aproximadamente 12% con presentación extra-esquelética. Actualmente, existen alrededor de 20 casos descritos en la literatura con origen mediastinal y 10 casos con origen pulmonar. Caso clínico: Se presenta el caso de una mujer de 25 años con un mes de disnea y dolor torácico, con el hallazgo de derrame pleural masivo y tumoración mediastinal en hemitórax derecho. Se le realiza toracotomía anterior bilateral con esternotomía transversa de Clamshell, con resección parcial que demuestra, por patología, sarcoma monomórfico de alto grado e inmunohistoquímica concluyente de sarcoma de Ewing. Conclusión: Este caso es una entidad rara y conlleva un reto diagnóstico para el clínico; sin embargo, debe sospecharse considerando la presentación clínica y radiológica del paciente, buscando incrementar la tasa de supervivencia mediante el diagnóstico y tratamiento oportuno.

Introduction: Ewing's sarcoma is a high-grade malignant tumor with mainly bony lo-calization; approximately 12% have been reported with extraskeletal presentation. Currently, there are about 20 cases described in the literature with mediastinal origin and 10 pulmonary cases. Case Report: We present the case of a 25-year-old woman with one month of dysp-nea and chest pain, with massive pleural effusion and mediastinal tumor in the right hemithorax who underwent bilateral anterior thoracotomy with Clamshell transverse sternotomy, with partial resection demonstrating, by pathology, high-grade monomorphic sarcoma and conclusive immunohistochemistry of Ewing's sarcoma. Conclusion: This case is a rare entity and involves a diagnostic challenge for the clinician; however, it should be suspected considering the clinical and radiological presentation of the patient, seeking to increase the survival rate through timely diagnosis and treatment.

Bronquitis plástica. Caso clínico interesante / Plastic Bronchitis. Interesting Case Report

La bronquitis plástica es una enfermedad infrecuente y poco estudiada. Se caracteriza por la obstrucción parcial o total de la vía aérea inferior por moldes o yesos gomosos y firmes, compuestos por múltiples sustancias como fibrina, mucina y otros, que se acumulan en la luz bronquial. En la actualidad, no hay un consenso de la fisiopatología real. Puede presentarse con síntomas leves como tos, sibilancias y disnea, hasta eventos fatales de insuficiencia respiratoria. Se clasifican en tipo I (inflamatorios) y tipo II (acelulares). La presencia de la bronquitis plástica es una complicación de varias enfermedades y está relacionada con procedimientos correctivos de cardiopatías congénitas (procedimiento de Fontan). El diagnóstico se hace a través de la identificación de los yesos bronquiales, ya sea cuando el paciente los expectora o por broncoscopía. Se han utilizado múltiples terapias que solo tienen evidencias anecdóticas. En los últimos años se han observado buenos resultados con el uso de heparinas, así como el alteplasa nebulizado e instilado por broncoscopia.

Plastic bronchitis is a rare and little-studied disease. It is characterized by partial or total obstruction of the lower airway by rubbery and firm molds or plasters, made up of multiple substances that accumulate in the bronchial lumen. Currently, there is no consensus on real pathophysiology. It can present itself with mild symptoms such as cough, wheezing and dyspnea, to fatal events of respiratory failure. They are classified into type I (inflammatory) and type II (acellular). The presence of plastic bronchitis is a complication of several diseases and in corrective procedures for congenital heart disease (Fontan procedure). Diagnosis is made by identifying bronchial casts, either by the patient expectorating them or by bronchoscopy. Multiple therapies have been used that only have anecdotal evidence. In recent years, good results have been observed with the use of heparins and tPA nebulized and instilled by bronchoscop.

Liposarcoma gigante mediastinal. Cirugía radical con abordaje Clamshell y manejo en la Unidad de Cuidados Críticos / Giant Mediastinal Liposarcoma. Radical Surgery with Clamshell Approach and Management in Critical Care Unit

Se presenta a un paciente con liposarcoma mediastinal gigante con dolor torácico, disnea, cuyos estudios por imágenes revelaban la presencia de una gran tumoración de 42 cm en su diámetro mayor que abarcaba todo el mediastino, comprometía ambas cavidades torácicas, rechazaba los pulmones, corazón y grandes vasos. La biopsia con aguja cortante bajo guía ecográfica fue informada como liposarcoma. El paciente tuvo resección completa del tumor mediante la incisión Clamshell. En el post operatorio inmediato, presentó shock circulatorio más disfunción multiorgánica (DOMS): plaquetopenia, insuficiencia renal aguda con necesidad de soporte dialítico, injuria hepática. El soporte y monitoreo especializado en la Unidad de Cuidados Intensivos (UCI) permitió mejoría clínica y buena evolución. Salió de alta en buenas condiciones.

We present a patient with giant mediastinal liposarcoma with chest pain, dyspnea, whose imaging studies revealed the presence of a large tumor measuring 42 cm in its greatest diameter that covered the entire mediastinum, involved both thoracic cavities, rejected the lungs, heart and big glasses. The sharp needle biopsy under ultrasound guidance was reported as liposarcoma. The patient had complete resection of the tumor through the Clamshell incision. In the immediate postoperative period, he presented circulatory shock plus multiple organ dysfunction (DOMS): plateletopenia, acute renal failure with the need for dialytic support, liver injury. Specialized support and monitoring in the Intensive Care Unit (ICU) allowed clinical improvement and good evolution. He was discharged in good condition.

Descripción de las intervenciones broncoscópicas en estenosis traqueales posterior a infección por COVID-19 / Description of Bronchoscopic Interventions in Post-COVID Tracheal Stenosis

Introducción: La infección por SARS-CoV-2 puede presentar síndrome de distrés res-piratorio agudo con requerimiento de ventilación mecánica prolongada y retraso en la realización de traqueostomía. Esto trae como consecuencia un incremento en casos de estenosis traqueal y la necesidad de métodos menos invasivos para su abordaje. Métodos: Estudio descriptivo de corte transversal, desde marzo 2020 hasta diciem-bre 2021 en el Hospital Universitario Nacional de Colombia, en adultos con estenosis traqueal postintubación asociado SARS-CoV-2. Se realizó análisis univariado entre los grupos con infección o no por SARS-CoV-2 como control, y reintervención, grado de estenosis, uso de inyección intramucosa con dexametasona intratraqueal o múltiples estenosis como desenlaces de importancia. Se usó test exacto de Fisher, t Student y Man-Whitney según la naturaleza de variables. Se consideró p estadísticamente significativo menor a 0.05.Resultados: Se identificaron 26 pacientes, 20 tenían COVID-19 y 6 no. Se encontraron diferencias en edad (p=0,002), epilepsia (p=0,007) y estenosis múltiple (p= 0,04). En 85% de los casos se utilizó láser blue más dilatación con balón pulmonar, en 35% inyección intramucosa con dexametasona intratraqueal y reintervención en 35%, sin diferencias significativas entre grupos. Conclusiones: Se observó un incremento tres veces mayor de pacientes con estenosis múltiple en el grupo de infección por COVID-19, así mismo se encontró que el método más utilizado en este grupo para la recanalización fue el uso de láser blue más dilatación con balón pulmonar y la innovación en el uso de inyección intramucosa.

Introduction: SARS-CoV-2 infection can lead to acute respiratory distress syndrome with a prolonged need for mechanical ventilation and delayed tracheostomy, resulting in an increase in cases of tracheal stenosis and the necessity for less invasive approaches.Methods: A descriptive cross-sectional study was conducted from March 2020 to December 2021 at the Hospital Universitario Nacional de Colombia, focusing on adults with post-intubation tracheal stenosis associated with SARS-CoV-2. Univariate analysis was performed between groups with or without SARS-CoV-2 infection as a control, considering reintervention, degree of stenosis, use of intratracheal steroids, or multiple stenoses as important outcomes. Fisher's exact test, Student's t-test, and Mann-Whit-ney test were employed based on the nature of variables. A p-value less than 0.05 was considered statistically significant.Results: A total of 26 patients were included, with 20 having COVID-19 and 6 without. Significant differences were found in age (p=0.002), epilepsy (p=0.007), and multiple stenosis (p=0.04). In 85% of cases, laser blue plus balloon pulmonary dilation was used, intratracheal dexamethasone in 35%, and reintervention in 35%, with no significant differences between groups.Conclusions: A threefold increase in subglottic stenosis was observed during the SARS-CoV-2 pandemic, with more instances of multiple stenosis and predominantly the use of laser blue plus balloon pulmonary dilation as a successful recanalization technique. There was a higher use of intratracheal dexamethasone in this group compared to oth-er pathologies causing tracheal stenosis.

Un caso pediátrico de telangiectasia hemorrágica hereditaria (Síndrome de Rendu-Osler-Weber) con compromiso pulmonar / A pediatric case of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber Syndrome) with pulmonary involvement

La telangiectasia hemorrágica hereditaria (HHT), o síndrome de Rendu-Osler-Weber, se considera dentro del grupo de las enfermedades raras, pues afecta a 1 de cada 3.000 a 8.000 individuos. Tiene un patrón de herencia autosómica dominante y la mayor parte de los casos se debe a una alteración en la codificación de los genes endoglina (ENG) y activina receptor-like kinase 1 (ALK1), dando origen a los tipos 1 y 2, respectivamente. Esta alteración genética se traduce en una displasia del endotelio de la pared vascular debido a haploinsuficiencia para endoglina y se manifiesta clínicamente con epistaxis a repetición, telangiectasias mucocutáneas y malformaciones arteriovenosas (MAV) viscerales. A continuación, se presenta el caso clínico de una paciente pediátrica de 11 años de edad que se manifestó por disnea e hipoxemia severa debido a múltiples fístulas arteriovenosas pulmonares.

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is considered among rare diseases, as it affects 1 in every 3,000 to 8,000 individuals. It follows an autosomal dominant inheritance pattern, and most cases are due to alterations in the coding of the endoglin (ENG) and activin receptor-like kinase 1 (ALK1) genes, leading to types 1 and 2, respectively. This genetic alteration results in vascular endothelial dysplasia due to haploinsufficiency for endoglin and clinically manifests with recurrent epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations (AVMs). Herein, we present the clinical case of an 11-year-old pediatric patient who exhibited severe dyspnea and hypoxemia due to multiple pulmonary arteriovenous fistulas.

Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.

A case of neonatal Netherton syndrome / 中国当代儿科杂志

A male infant, aged 6 days, was admitted to the hospital due to respiratory distress and systemic desquamative rash after birth. The infant presented with erythema and desquamative rash, respiratory failure, recurrent infections, chronic diarrhea, hypernatremic dehydration, and growth retardation. Comprehensive treatment, including anti-infection therapy, intravenous immunoglobulin administration, and skin care, resulted in improvement of the rash, but recurrent infections persisted. Second-generation sequencing revealed a homozygous mutation in the SPINK5 gene, consistent with the pathogenic variation of Netherton syndrome. The family opted for palliative care, and the infant died at the age of 2 months after discharge. This report documents a case of Netherton syndrome caused by the SPINK5 gene mutation in the neonatal period, and highlights multidisciplinary diagnosis and therapy for this condition.

A fase terminal do filho com câncer: percepções dos profissionais hospitalares / The terminal phase of children with cancer: perceptions of hospital professionals / La fase terminal del hijo con cáncer: percepciones de los profesionales del hospital

O confronto com o câncer de um filho e a percepção da sua morte como inevitável dão lugar a experiências parentais relevantes para a pesquisa científica. Este estudo teve como objetivo investigar, por meio da percepção dos profissionais hospitalares, o modo como os pais experienciam a fase terminal e fim de vida do filho com câncer para melhor compreender os processos psicoemocionais experienciados por esses pais diante da cronicidade da doença e da morte do filho. No sentido de alcançar esse objetivo, realizou-se um estudo qualitativo de tipo fenomenológico envolvendo 17 profissionais de dois hospitais portugueses de referência em oncologia pediátrica. Os dados foram recolhidos com recurso a um guia de entrevista semiestruturada. Na percepção dos profissionais hospitalares, os resultados evidenciam que esses pais experienciam múltiplas dificuldades e preocupações na fase terminal da doença do filho e no pós-morte, bem como um sofrimento extremo e desestruturação biopsicossocial e espiritual na família. O conhecimento aprofundado da fenomenologia desses processos é essencial para o desenho e a implementação de intervenções emocionais, cognitivas, comportamentais e sociais mais ajustadas às dificuldades e preocupações parentais vividas no fim de vida e pós-morte.(AU)

Coping with children's cancer and the perception of their inevitable death give rise to parental experiences that are important to study. This study aimed to investigate, based on hospital professionals' perspectives, how parents experience the terminal phase and end of life of their children suffering from cancer to better understand the psycho-emotional processes these parents experienced in face of the chronicity of the disease and their children's death. To achieve this objective, a qualitative phenomenological study was carried out involving 17 professionals of two Portuguese hospitals that are reference in pediatric oncology. Data were collected using a semi-structured interview guide. From the perspective of hospital professionals, results show that these parents experience multiple difficulties and concerns in the terminal phase of their children's disease and postmortem, as well as the extreme suffering and biopsychosocial and spiritual disruption of the family. A deeper understanding of the phenomenology of these processes is essential to design and implement better adjusted emotional, cognitive, behavioral, and social interventions aimed at the parental difficulties and concerns experienced at the end of life and after death.(AU)

El enfrentamiento del cáncer de un hijo y la percepción de su muerte como inevitable dan lugar a experiencias parentales importantes que deben ser estudiadas. Este estudio pretende identificar desde la percepción de los profesionales del hospital cómo los padres viven la fase terminal y el final de la vida de su hijo con cáncer con el fin de comprender mejor los procesos psicoemocionales que viven estos padres ante la cronicidad de la enfermedad y la muerte de su hijo. Para ello, se realizó un estudio cualitativo, con enfoque fenomenológico, en el que participaron 17 profesionales de dos hospitales portugueses de referencia en oncología pediátrica. Para recoger los datos se aplicó un guion de entrevista semiestructurada. En cuanto a la percepción de los profesionales del hospital, estos padres experimentaron múltiples dificultades y preocupaciones en la fase terminal de la enfermedad de su hijo y postmuerte, así como un sufrimiento extremo y una desestructuración biopsicosocial y espiritual en la familia. El conocimiento en profundidad de la fenomenología de estos procesos es esencial para elaborar e implementar intervenciones emocionales, cognitivas, conductuales y sociales más acordes a las dificultades y preocupaciones parentales que se experimentan al final de la vida y la postmuerte.(AU)

Series en disnea. Parte 2. Análisis de sus mecanismos según pasan los años / Series on dyspnea. Part 2. Analysis of its mechanisms as the years go by

Este artículo está dedicado al análisis detallado de los mecanismos de disnea. Se tratarán el control químico de la respiración, los reflejos neurales, la mecánica respiratoria, el costo de oxígeno para respirar y la inadecuación entre tensión y longitud de la fibra muscular. En general, las diferentes explicaciones estuvieron asociadas al desarrollo de aparatos y metodologías de estudio de los laboratorios pulmonares. Todas las teorías tuvieron defensores y detractores e, interesantemente, con el desarrollo de sofisticadas técnicas neurofisiológicas y de imágenes funcionales ha sido posible jerarquizar cada uno de los mecanismos. Todas han sobrevivido al paso del tiempo y ninguna puede explicar de manera unicista la disnea en todas las situaciones clínicas, lo cual habla de la naturaleza compleja y multifactorial del fenómeno.

This article is devoted to a detailed analysis of the mechanisms of dyspnea. Chemical control of respiration, neural reflexes, respiratory mechanics, the cost of oxygen to breathe, and the mismatch between tension and muscle fiber length will be discussed. In general, the different explanations were associated with the development of apparatus and study methodologies in pulmonary laboratories. All the theories had defenders and detractors and, interestingly, with the development of sophisticated neurophysiological techniques and functional imaging it has been possible to prioritize each of the mecha nisms. All have survived the passage of time and none can explain dyspnea in all clinical situations, which speaks of the complex and multifactorial nature of the phenomenon.

Analysis of pulmonary function in post-Covid-19 patients at a university hospital / Análise da função pulmonar de pacientes com síndrome pós-covid-19 em um hospital universitário

Objective: Evaluate the spirometry pattern of patients who persisted with respiratory symptoms after infection with SARS-Cov-2. Methods: Cross-sectional, observational, retrospective study in a single center, approved by the local Ethics Committee (registration number: 5,120,720). Patients who underwent spirometry due to Post-Covid Syndrome were evaluated to analyze the spirometric pattern presented. The following were collected: exam identification data, sex, age, symptom time, the need for mechanical ventilation, and quality of spirometry, in addition to the following exam parameters: FVC, FEV1, FEV1/FVC, FEV 25-75/FVC, and FEV 75, evaluating the Lower Limit of Normality, pre-bronchodilator and post-bronchodilator values. Results: Data from 72 patients were collected. Of these, 55.5% of patients had spirometry results within normal limits. The most frequent respiratory alteration was obstructive respiratory disorder, present in 29.2% of the patients. Conclusions: The presence of dyspnea in patients with normal spirometry may indicate further evaluation of lung function and other etiologies for dyspnea (AU).

Objetivo: Avaliar o padrão de espirometria de pacientes que persistiram com sintomas respiratórios após a infecção pelo SARS-CoV-2. Métodos: Estudo transversal, observacional e retrospectivo realizado em um único centro, aprovado pelo Comitê de Ética local (número do parecer: 5.120.720). Foram avaliados pacientes submetidos a espirometria devido à Síndrome Pós-Covid, a fim de analisar o padrão espirométrico apresentado. Os seguintes dados foram coletados: identificação do exame, sexo, idade, tempo de sintomas, necessidade de ventilação mecânica, qualidade da espirometria, além dos seguintes parâmetros do exame: CVF, VEF1, VEF1/CVF, VEF 25-75/CVF e VEF 75, avaliando o Limite Inferior da Normalidade, valores pré-broncodilatador e pós-broncodilatador. Resultados: Foram coletados dados de 72 pacientes. Destes, 55,5% apresentaram resultados espirométricos dentro dos limites normais. A alteração respiratória mais frequente foi o distúrbio ventilatório obstrutivo, presente em 29,2% dos pa-cientes. Conclusões: A presença de dispneia em pacientes com espirometria dentro da normalidade pode indicar uma avaliação adicional da função pulmonar, assim como outras etiologias para a dispneia (AU).

Disección de aorta secundario a trauma, manejo clínico en Hospital General / Aortic dissection secondary to trauma, clinical management in General Hospital

INTRODUCCIÓN. La disección de aorta es una patología poco frecuente, cuando se presenta, lo hace de una manera catastrófica manifestada por dolor torácico o lumbar intenso acompañado de compromiso hemodinámico agudo, un diagnóstico y tratamiento temprano suelen ser cruciales para evitar la muerte del paciente, así mismo, una disección de aorta puede ser secundario a un trauma torácico por desaceleración y se presenta en 1,5% a 2%, lo que induce sobre todo a un desgarro localizado en la región del istmo aórtico. CASO CLÍNICO. Paciente masculino de 35 años, con antecedente de accidente en vehículo motorizado que provocó un traumatismo cráneo encefálico moderado, hematoma epidural parietal derecho, con resolución quirúrgica hace 18 meses, se descartó lesiones a nivel de tórax con radiografía de tórax normal, además se realizó ecografía FAST en ventana perihepática, suprapúbica, periesplénica y subxi-foidea sin encontrar alteración. Acudió al servicio de emergencia por un cuadro de hemiparesia derecha más disartria de 4 horas de evolución, se realiza tomografía de cráneo sin encontrarse lesión, catalogándose como evento cerebrovascular isquémico con recuperación neurológica. Durante su estancia hospitalaria presenta un cuadro de disnea de grandes esfuerzos y tras realizar exámenes complementarios de control se llega a una conclusión de una cardiomegalia, por lo que es referido a consulta de cardiología donde se realiza ecocardiograma, en la cual se observa disección de aorta ascendente más aneurisma por lo que se decide su ingreso y se instaura tratamiento mediante medidas de soporte, con derivación a hospital de especialidades para cirugía cardiotorácica. CONCLUSIONES. La disección de aorta secundario a un traumatis-mo es poco frecuente, sin embargo, el diagnostico siempre debe estar presente en caso de trauma por desaceleración, ya que es vital para la supervivencia del paciente y su manejo oportuno.

INTRODUCTION. Aortic dissection is an infrequent pathology; when it occurs, it does so in a catastrophic manner manifested by intense thoracic or lumbar pain accompanied by acute he-modynamic compromise. Early diagnosis and treatment are usually crucial to avoid the patient's death; likewise, aortic dissection can be secondary to thoracic trauma due to deceleration and occurs in 1.5% to 2%, which mainly induces a localized tear in the region of the aortic isthmus. CLINICAL CASE. 35-year-old male patient, with a history of motor vehicle accident that caused a moderate head injury, right parietal epidural hematoma, with surgical resolution 18 months ago, chest injuries were ruled out with normal chest X-ray, and FAST ultrasound was performed in perihepatic, suprapubic, perisplenic and subxiphoid window without finding any alteration. She went to the emergency department for a picture of right hemiparesis and dysarthria of 4 hours of evolution, a cranial tomography was performed without finding any lesion, being clas-sified as an ischemic cerebrovascular event with neurological recovery. During his hospital stay he presented with dyspnea of great effort and after performing complementary control tests, a cardiomegaly was found, so he is referred to a cardiology clinic where an echocardiogram is per-formed, which showed dissection of the ascending aorta plus aneurysm, admission is decided and treatment is instituted through supportive measures, with referral to a specialty hospital for cardiothoracic surgery. CONCLUSIONS. Aortic dissection secondary to trauma is rare, however, the diagnosis should always be present in case of trauma due to deceleration, as it is vital for the survival of the patient and its timely treatment.

COVID-19 e o ciclo gravídico-puerperal: uma revisão narrativa / COVID-19 and the pregnancy cycle: a narrative review

Até este momento da pandemia de COVID-19, embora as gestantes não tenham maior risco de se infectar do que a população geral, elas têm maiores riscos de desenvolver formas graves e demandar cuidados de UTI e ventilação invasiva, so- bretudo aquelas que apresentam comorbidades. No Brasil, a mortalidade materna por COVID-19 está entre as mais elevadas do mundo. A transmissão vertical do SARS-CoV-2 parece ser um evento raro, e até o momento não se observou aumento da ocorrência de abortos e malformações. Entretanto, a COVID-19 está associada a elevadas taxas de prematuridade, baixo peso ao nascer e admissão em UTI neona- tal. Em adaptação a esse novo cenário, são indicados cuidados especiais durante o ciclo gravídico-puerperal, sendo útil destacar: o espaço crescente da telemedicina no pré-natal; a não obrigatoriedade da realização de cesariana em caso de gestan- te infectada no momento do parto e a liberação da amamentação pelas puérperas com COVID-19.

Eficacia y tolerabilidad Resultados del tratamiento para la fibrosis pulmonar idiopática. Experiencia de la vida real / Efficacy and Tolerability Outcomes of Treatment for Idiopathic Pulmonary Fibrosis. Real-life Experience

Introducción: la fibrosis pulmonar idiopática (FPI) es una enfermedad progresiva y cró-nica con muy mal pronóstico. Actualmente, existen dos fármacos para esta patología. El propósito de nuestro estudio es evaluar los efectos del tratamiento en los pacientes de una consulta en vida real.

Introduction: idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease with a very poor prognosis. Two drugs are currently available for this disease. The purpo-se of our study is to evaluate the effects of treatment in patients in a real-life practice.

O uso de métodos contraceptivos hormonais em pacientes com asma: repercussões clínicas / The use of hormonal contraceptives methods in patients with asthma: clinical repercussions

Objetivo: Analisar o uso dos contraceptivos hormonais em mulheres com asma e a escolha desses métodos contraceptivos para essa população, com avaliação de eventuais repercussões sobre novos episódios de asma e sibilos. Métodos: Foram selecionados estudos longitudinais, ensaios clínicos, revisões sistemáticas e metanálises. As plataformas consultadas foram PubMed, Embase, Cochrane e SciELO, com a utilização dos descritores: "contracepção", "contracepção hormonal", "sistema intrauterino liberador de levonorgestrel" e "asma". Resultados: Dois grandes estudos demonstraram que o uso de contraceptivos hormonais esteve associado à redução do risco de novos episódios de asma. Uma revisão sistemática concluiu que os resultados para o uso de contraceptivos hormonais para mulheres com asma foram mistos, com aumento ou redução dos seguintes riscos: novo episódio de asma e aumento da frequência das crises e dos sibilos. O uso da contracepção hormonal em pacientes obesas portadoras de asma é controverso. Conclusão: Os resultados para o uso de contraceptivos hormonais em mulheres com asma são inconsistentes, com relatos de aumento ou de redução do risco de novos episódios. O uso do método contraceptivo deve ser discutido individualmente, levando-se em consideração outros fatores de risco associados e o desejo da mulher. A paciente deverá ser orientada se houver piora dos sintomas clínicos de asma na vigência do uso de qualquer método contraceptivo hormonal.

Objective: To analyze the use of hormonal contraceptives in women with asthma and the choice of this contraceptive method for this population, evaluating possible repercussions on new episodes of asthma and wheezing. Methods: Longitudinal studies, clinical trials, systematic reviews and meta-analyses were selected. Platforms consulted: PubMed, Embase, Cochrane, SciELO, using the descriptors: "contraception", "hormonal contraception", "levonorgestrel-releasing intrauterine system" and "asthma". Results: Two large studies demonstrated that the use of hormonal contraceptives was associated with a reduced risk of new episodes of asthma. A systematic review concluded that the results for the use of hormonal contraceptives for women with asthma were mixed, with increased or decrease in the following risks: new asthma episodes, increased frequency and wheezing. The use of hormonal contraception in obese patients with asthma is controversial. Conclusion: The results for the use of hormonal contraceptives in women with asthma are inconsistent, with reports of increased or reduced risk of new episodes. The use of the contraceptive method should be discussed individually, taking into account other associated risk factors and the woman's desire. The patient will be advised if there is a worsening of the clinical symptoms of asthma while using any hormonal contraceptive method.

Control de síntomas al final de la vida con Dexmedetomidina: Estudio observacional de centro único / End-of-life symptom control with dexmedetomidine: A single-center observational study

Introducción: El manejo de los síntomas refractarios con dexmetomidina (DXM) es una alternativa en pacientes oncológicos en la atención al final de la vida (AFV) lo que puede convertirse en un gran desafío, ante la necesidad de iniciar sedación paliativa. El objetivo del presente estudio es determinar el efecto de la DXM comparado con el midazolam (MDZ) en el control de síntomas refractarios en pacientes con cáncer avanzado AFV, como parte de una estrategia asistencial de sedación paliativa. Metodología: Estudio observacional, longitudinal, se revisaron los informes médicos en las historias clínicas de pacientes hospitalizados con diagnóstico de cáncer avanzado terminal y síntomas refractarios (dolor, disnea y delirio), que recibieron sedación paliativa con DXM o MDZ. Se utilizaron los registros de Escala de Evaluación de Síntomas de Edmonton - revisada (ESAS-r) para intensidad de síntomas y la Escala de Sedación/Agitación de Richmond (RASS) para respuesta a la sedación. Resultados: Un total de 35 pacientes recibieron DXM y 18 MDZ. El dolor (32 %) y delirio (30 %) fueron los síntomas refractarios más identificados. Dolor intenso en 89.3 % antes del inicio de DXM. A las 72 horas, el grupo DXM presentó disminución de la intensidad del delirio a leve y moderado, mientras que la mayoría de los pacientes del grupo MDZ fallecieron antes de las 72 horas. Conclusiones: El uso clínico de la DXM permite un enfoque multimodal, ampliando su utilidad en la atención al final de la vida para el manejo del dolor y delirio, ya que permite un estado de respuesta para la interacción con los miembros de la familia, función relevante en cuidados paliativos.

Introduction: Managing refractory symptoms with dexmedetomidine (DXM) is an alternative for cancer patients in end-of-life care (EOLC), which can become a great challenge, given the need to start palliative sedation. This study aims to determine the effect of DXM compared to midazolam (MDZ) in controlling refractory symptoms in patients with advanced EOLC cancer as part of a palliative sedation care strategy. Methodology: In this observational, longitudinal study, the medical reports in the medical records of hospitalized patients diagnosed with terminal advanced cancer and refractory symptoms (pain, dyspnea, and delirium) who received palliative sedation with DXM or MDZ were reviewed. The Edmonton Symptom Assessment Scale-revised (ESAS-r) scores for symptom intensity and the Richmond Sedation/Agitation Scale (RASS) for response to sedation were used. Results: A total of 35 patients received DXM, and 18 received MDZ. Pain (32%) and delirium (30%) were the most identified refractory symptoms. Intense pain in 89.3% before DXM started; at 72 hours in 64.3%, the pain was reported as mild. Delirium was severe in 77.7%, DXM was used in 59.3%, and MDZ was used in 40.7%. At 72 hours, the DXM group presented a decrease in delirium intensity to mild and moderate, while most of the patients in the MDZ group died before 72 hours. Conclusions: The clinical use of DXM allows a multimodal approach, expanding its usefulness in end-of-life care for managing pain and delirium since it provides a state of response for interaction with family members, a function relevant in palliative care.

Características clínicas, diagnóstico e tratamento de mulher com síndrome de Meigs: relato de caso e revisão narrativa / Clinical characteristics, diagnosis and treatment of a woman with Meigs syndrome: case report and narrative review

O objetivo deste estudo é descrever o caso de mulher com síndrome de Meigs e apresentar a revisão narrativa sobre o tema. Paciente do sexo feminino, 30 anos, nulípara, encaminhada ao hospital por massa anexial e história prévia de drenagem de derrame pleural. Evoluiu com instabilidade hemodinâmica por derrame pleural hipertensivo à direita, sendo submetida a drenagem torácica, com citologia do líquido negativa. Após, foi submetida a laparotomia: realizada salpingo-ooforectomia esquerda. A congelação e a análise histopatológica diagnosticaram fibroma ovariano. A citologia ascítica foi negativa. CA-125 elevado, presença de derrames cavitários e exame de imagem suspeito podem mimetizar um cenário de neoplasia maligna de ovário em estágio avançado. Entretanto, na síndrome de Meigs clássica, o tratamento é cirúrgico, sendo o diagnóstico obtido por meio da análise histopatológica do tumor ovariano. O manejo da síndrome de Meigs clássica é cirúrgico e, após a remoção do tumor, o derrame pleural e a ascite desaparecem.

To describe a case of Meigs syndrome and present a narrative review of the condition. Female patient, 30 years old, nulliparous, referred to the hospital due to an adnexal mass and a previous drainage of pleural effusion. She developed hemodynamic instability due to a hypertensive right pleural effusion being submitted to chest drainage, with negative cytology of the fluid. She underwent laparotomy: Left salpingo-oophorectomy was performed and frozen section and histopathological analysis diagnosed an ovarian fibroma. Ascites cytology was negative. Elevated CA-125, presence of cavitary effusions, suspicious imaging exam can mimic a scenario of ovarian cancer at an advanced stage. However, in classical Meigs syndrome, treatment is surgical, and the diagnosis is obtained through histopathological analysis of the ovarian tumor. Classical Meigs syndrome' management is surgical. After tumor removal, pleural effusion and ascites resolve.

Enfermedad de Pompe y compromiso respiratorio / Pompe Disease and Respiratory Impairment

Introducción: El compromiso respiratorio en la enfermedad de Pompe es una de sus manifestaciones más frecuentes, tiene un impacto negativo en la calidad de vida y facilita la aparición de múltiples complicaciones. Se puede presentar como parte evolutiva de la enfermedad o incluso ser el síntoma inicial de la patología. Contenidos: La presentación clínica del compromiso respiratorio en la enfermedad de Pompe se caracteriza por disnea, ortopnea, cefalea y tos, y sus cambios paraclínicos incluyen hipercapnia, policitemia, elevación del índice de apnea/hipopnea, así como disminución de la capacidad vital y de las presiones inspiratoria y espiratorias máximas. El compromiso respiratorio es causado principalmente por disfunción muscular (especialmente el diafragma) y alteración de la regulación del sistema nervioso central. Conclusiones: Es fundamental la valoración acuciosa inicial de los patrones respiratorios y por supuesto su seguimiento, el cual dependerá del tipo de progresión de la disfunción respiratoria (rápida o lenta) o del requerimiento específico para cada paciente (ventilación no invasiva o invasiva).

Introduction: Respiratory compromise in Pompe disease is one of the most frequent manifestations, which has a negative impact on quality of life and leads to multiple complications. This can occur as an evolutionary part of the disease, or even be the initial symptom of the pathology. Contents: Its clinical presentation is characterized by dyspnea, orthopnea, headache, and cough, and its paraclinical changes include hypercapnia, polycythemia, elevated apnea/hypopnea index, decreased vital capacity, and decreased maximum inspiratory and expiratory pressures. Respiratory compromise is caused mainly by muscular dysfunction (especially the diaphragm) and alteration of regulation of the central nervous system. Conclusions: An initial careful assessment of respiratory patterns is essential, and of course their follow-up, that will depend of the type of progression of respiratory dysfunction (rapid or slow) or the specific requirement for each patient (non-invasive or invasive ventilation).

Relación entre la distancia recorrida en la prueba de caminata de 6 minutos y el sexo, la edad, comorbilidades y la disnea en pacientes con EPOC / Relationship between the Distance Traveled in the Test of 6- Minute Walk and Sex, Age, Comorbidities and Dyspnea in Patients with CORP

Introducción: la enfermedad pulmonar obstructiva crónica (EPOC) es un trastorno res-piratorio caracterizado por síntomas clínicos y compromiso funcional que afecta la ca-pacidad aeróbica limitando las actividades cotidianas y la calidad de vida. La prueba de caminata de 6 minutos (C6M) es una prueba sencilla y de bajo costo que evalúa la capa-cidad de los pacientes para realizar sus actividades cotidianas. Objetivo: evaluar la re-lación entre la capacidad aeróbica medida por la distancia recorrida en la C6M y el se-xo, edad, disnea y comorbilidades cardiometabólicas en pacientes con EPOC. Material y métodos: estudio de corte transversal, descriptivo, basado en pruebas de caminatas de 6 minutos (C6M) realizadas en pacientes con EPOC. Resultados: se evaluaron 101 pacientes, hombres (63,4%), con una edad promedio de 74,1±8,7 años. Al correlacionar C6M con otras variables se encontraron diferencias estadísticamente significativas. La distancia media recorrida fue mayor en hombres que en mujeres (DM: 58,3 metros, IC 95%; 16 - 100,6, p=0,007). Los pacientes < 75 años, sin comorbilidades y disnea < 2 tu-vieron mejor desempeño en la C6M que los > 75 años (DM; 62,012 metros IC 95% 21,5 - 102,4, p=0,003), con comorbilidades (DM: 42,2 metros, IC 95%, 0.003 - 84,4; p=0,050) y disnea ≥ 2 (DM: 65,8 IC 95% 23,9 - 107,6, p=0,002). Conclusiones: el sexo femenino, la presencia de comorbilidad cardiovascular y metabólica, y la edad se asocian con dis-minución en la capacidad física aeróbica y funcional en los pacientes con EPOC. (AU)

Introduction: chronic obstructive pulmonary disease (COPD) is a respiratory disorder characterized by clinical symptoms and functional impairment that affects aerobic capacity, limiting daily activities and quality of life. The 6-minute walk test (C6M) is a simple, low-cost test that assesses a patient's ability to perform their daily activities. Objective: to evaluate the relationship between aerobic capacity measured by the distance covered in the C6M and gender, age, dyspnea and cardiometabolic comorbidities in patients with COPD. Materials and methods: cross-sectional, descriptive study, based on 6-minute walk tests (C6M) performed in patients with COPD. Results: 101 male patients (63.4%), with a mean age of 74.1 ± 8.7 years, were evaluated. When correlating C6M with other variables, statistically significant differences were found. The mean distance traveled was greater in men than in women (MD: 58.3 meters, 95% CI: 16 - 100.6, p=0.007). Patients <75 years old, without comorbidities and dyspnea <2 had better performance in the C6M than those >75 years old (MD; 62 meters CI 95% 21.5 - 102.4, p=0.003), with comorbidities (MD: 42.2 meters, 95% CI, 0.003 - 84.4; p=0.050) and dyspnea ≥ 2 (MD: 65.8, 95% CI 23.9 - 107.6, p=0.002). Conclusions: female sex, the presence of cardiovascular and metabolic comorbidity, and age are associated with decreased aerobic and functional physical capacity in patients with COPD. (AU)