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La atención de una persona tras sufrir los primeros síntomas de Infarto Agudo de Miocardio. (IAM), debe procurarse en los primeros 90 minutos, por lo cual es importante actuar inmediatamente y contar con pruebas de laboratorio en la unidad de emergencia. Los presentes lineamientos incluyen los criterios de elegibilidad para el uso adecuado de los biomarcadores rápidos, así como la estandarización en la interpretación de los resultados para la identificación temprana del diagnóstico diferencial de disnea y dolor torácico en los diferentes niveles de atención del Ministerio de Salud

The care of a person after suffering the first symptoms of Acute Myocardial Infarction (AMI) should be sought within the first 90 minutes, which is why it is important to act immediately and to have laboratory tests available in the emergency unit. The present guidelines include the eligibility criteria for the appropriate use of rapid biomarkers, as well as the standardization of the interpretation of the results for the early identification of the differential diagnosis of dyspnea and chest pain at the different levels of care of the Ministry of Heal

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Introduction : L'objectif du travail était d'estimer la fréquence hospitalière des affections respiratoires prises en charge à l'Hôpital d'Instruction des Armées-Centre Hospitalier Universitaire (HIA-CHU) de Parakou. Méthodes : Etude transversale descriptive menée de février 2023 à juillet 2024. Ont été inclus, tous les patients âgés d'au moins 15 ans, reçus en consultation ou hospitalisés pour affections respiratoires à l'HIA-CHU de Parakou et ayant donné leur consentement pour participer à l'étude. Les informations sur les caractéristiques socio-démographiques, cliniques, paracliniques, diagnostiques et évolutives ont été recueillies. Résultats : Au total, 221 patients ont été inclus. L'âge moyen était de 43,75 ± 22 ans et le sex-ratio (H/F) égal à 1,4. Les fonctionnaires ou salariés du privé (23,6%), les élèves ou étudiants (16,7%) et les ménagères (16,7%) étaient les plus représentés. Ils ont été reçus dans 76% des cas en consultation pneumologique et dans 21,7% aux urgences. La dyspnée (67,4%), la toux (65,2%) et la douleur thoracique (48,4%) étaient les principaux motifs de consultation. Les antécédents médicaux étaient dominés par l'hypertension artérielle (15,8%), le tabagisme (9,5%) et l'alcoolisme (6,8%). Les principales affections diagnostiquées étaient la pneumonie aigue communautaire (32,1%), l'asthme (22,2%) et la bronchopneumopathie chronique obstructive (6,3%). L'hospitalisation a concerné 57 (25,8%) patients avec une durée moyenne d'hospitalisation de 11 ± 7,8 jours. Le taux de létalité globale pour l'ensemble de ces affections respiratoires était de 1,8% (4/221) sur la période d'étude. Conclusion : Les pneumonies aiguës communautaires et l'asthme sont les pathologies dominantes chez les adultes pris en charge à l'HIA-CHU. Elles doivent faire l'objet d'une attention particulière, tant en termes de traitement que de prévention.

Introduction: The objective of the study was to estimate the hospital frequency of respiratory conditions managed at the Military Teaching Hospital of Parakou. Methods: A descriptive cross-sectional study was conducted from February 2023 to July 2024. All patients aged at least 15 years, seen in consultation or hospitalized for respiratory conditions at the HIA-CHU of Parakou, and who consented to participate in the study, were included. Information on socio-demographic, clinical, paraclinical, diagnostic, and evolutionary characteristics was collected Results: A total of 221 patients were included. The average age was 43.75 ± 22 years, with a sex ratio (M/F) of 1.4. Civil servants or private-sector employees (23.6%), students (16.7%), and housewives (16.7%) were the most represented groups. They were seen in 76% of cases in pulmonology consultations and in 21.7% in the emergency department. Dyspnea (67.4%), cough (65.2%), and chest pain (48.4%) were the main reasons for consultation. The medical history was dominated by hypertension (15.8%), smoking (9.5%), and alcoholism (6.8%). The main diagnosed conditions were community-acquired acute pneumonia (32.1%), asthma (22.2%), and chronic obstructive pulmonary disease (COPD) (6.3%). Hospitalization concerned 57 (25.8%) patients, with an average hospital stay of 11 ± 7.8 days. The overall case fatality rate for all respiratory conditions was 1.8% (4/221) during the study period. Conclusion: Community-acquired acute pneumonia and asthma are the predominant conditions among adults managed at the HIA-CHU. They require special attention in terms of treatment and prevention.

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La proteinosis alveolar pulmonar (PAP) es una enfermedad que entra en el gran número de patologías pulmonares intersticiales, con una baja incidencia y prevalencia a nivel mundial. Consiste en un acúmulo de material proteináceo derivado del surfactante que se genera por diferentes causas (primaria, secundaria, congénita y no clasificada), dentro de las cuales la causa autoinmune o primaria es la más frecuente. Su tratamiento de elección es el lavado pulmonar total, con mejoría según el grado de afectación y avance de la enfermedad. En la actualidad, hay nuevas terapias y otras que están en estudio, pero en países en vías de desarrollo muchas veces no se encuentran disponibles. Presentamos el caso de una paciente femenina de 32 años de edad que se presentó en insuficiencia respiratoria tipo 1 y debutando con PAP.

Pulmonary Alveolar Proteinosis (PAP) is a disease that belongs to the large number of interstitial lung pathologies, with a low incidence and prevalence worldwide. It consists of an accumulation of proteinaceous material derived from surfactant, which is generated by different causes (primary, secondary, congenital and unclassified), within which the autoimmune or primary cause is the most frequent. Its treatment of choice is total lung lavage, with improvement depending on the degree of involvement and progression of the disease. Currently, there are new therapies and others that are being studied, but in developing countries they are often not available. We present the case of a 32-year-old female patient who presented with type 1 respiratory failure and debuting with PAP.

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La infestación por Paragonimus spp. clásicamente se da por la ingestión de crustáceos de agua dulce, crudos, poco cocidos o en forma de ceviches. Se presenta el caso de un paciente adulto que conscientemente ingería cangrejos crudos y desarrolló sintomatología pulmonar con tos y disnea, lo cual lo llevó a efectuarse estudios broncoscópicos cuyo resultado citológico del cepillado bronquial fue de adenocarcinoma pulmonar. Se le realizó biopsia pulmonar y la patología fue concluyente de la presencia de huevecillos y formas larvarias de Paragonimus spp., que es una entidad poco frecuente en Guatemala. El paciente recibió tratamiento con prazicuantel y mostró buena res-puesta clínica.

Infestation by Paragonimus spp. classically occurs through the ingestion of freshwater crustaceans, raw, undercooked or in the form of "ceviches". We present the case of an adult patient who consciously ingested raw crabs and developed pulmonary symptoms with cough and dyspnea, which leaded him to undergo bronchoscopic studies, whose cytological result of bronchial brushing was pulmonary adenocarcinoma. A lung biopsy was performed and the pathology was conclusive of the presence of eggs and larval forms of Paragonimus spp., which is a rare entity in Guatemala. Patient received treatment with praziquantel and showed a good clinical response.

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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.

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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.

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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.

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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.

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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.

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La leiomiomatosis metastatizante benigna (LMB) es una condición rara. Fue descrita por primera vez por Forkel en 1910 y hasta el momento se han reportado al menos 150 casos en la literatura. Ocurre por la metástasis y proliferación de tejido muscular liso, a partir de un mioma uterino. A nivel imagenológico se manifiesta con múltiples nódulos localizados con mayor frecuencia en pulmón, ganglios linfáticos, peritoneo, hueso, corazón y piel. Los casos sintomáticos suelen ser llevados a resección quirúrgica, ooforectomía bilateral y/o inhibición hormonal. Presentamos el caso de una mujer de 51 años, quien acude a consulta por cuadro clínico de dos años de evolución caracterizada por disnea progresiva, asociada a tos seca y dolor torácico. Se documentó antecedente de histerectomía por hemorragia uterina disfuncional hace 14 años cuyo reporte de patología concluía diagnóstico de leiomiomatosis uterina. La tomografía de tórax reveló múltiples nódulos pulmonares aleatorios sólidos no calcificados. Las muestras obtenidas del parénquima pulmonar fueron por toracoscopia derecha realizándose lobectomía segmentaria lateral del lóbulo medio, con hallazgos histopatológicos de proliferación mesenquimal, epitelial y marcadores de inmunohistoquímica compatibles con leiomiomatosis metastásica.

Benign metastasizing leiomyomatosis (BML) is a rare condition, first described by Forkel in 1910, and at least 150 cases have been reported in the literature so far (1). It is caused by metastasis and proliferation of smooth muscle tissue from a uterine myoma. At the imaging level, it manifests with multiple nodules located most frequently in the lung, lymph nodes, peritoneum, bone, heart and skin. In symptomatic cases, they are usually taken to surgical resection, bilateral oophorectomy and/or hormonal inhibition, some cases being refractory. We present the case of a 51-year-old woman, who came for consultation due to a clinical picture of 2 years of evolution characterized by progressive dyspnea, associated with dry cough and chest pain. A history of hysterectomy for dysfunctional uterine bleeding 14 years ago was documented, and pathology report concluded a diagnosis of uterine leiomyomatosis. Chest CT scan revealed multiple non-calcified solid random pulmonary nodules. Samples obtained from the lung parenchyma were obtained by right thoracoscopy performing lateral segmental lobectomy of the middle lobe, with histopathological findings of mesenchymal proliferation, epithelial, and immunohistochemical markers compatible with metastatic leiomyomatosis.

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INTRODUCTION: The gestational period brings anatomical and physiological changes to women in several systems, especially the respiratory system. OBJECTIVE: To determine whether there is an association between gestational age (GA), Diabetes, uterine fundal height (UFH), Body Mass Index (BMI), level of dyspnea, and physical activity with maximum respiratory and nasal pressures. METHODS: Cross-sectional study that included 55 high-risk pregnant women in the 2nd and 3rd trimester of pregnancy at the Obstetrics Outpatient Clinic of the Hospital das Clínicas in Recife ­ PE, Brazil, personal, sociodemographic, anthropometric, clinical and Maximal Inspiratory Pressure (MIP) data were described and nasal inspiratory pressure (NIP), that, using regression and multivariate analysis, analyzed the influence of risk factors for high-risk pregnancy with NIP considering a p<0.005. RESULTS: Among the pregnant women, according to the clinical variables, it was observed that 56.4% (n=31) had a gestational age above 28 weeks, 27.3% (n=15) diabetes, 25.5% (n=14) asthma, 43.6% (n=24) Gestational Hypertension, 56.4% (n=31) obesity, 85.5% (n=47) complaints of dyspnea 38.1% (n=21) mild to severe dyspnea and 65.5% (n=36) vigorous physical activity. Low MIP (76.76 cmH2O) and NIP (68.62 cmH2O) values were found for age. An association was observed between an increase in UFH and a decrease of 0.8 cmH2O in NIP, regardless of gestational age. CONCLUSION: High-risk pregnant women in the second and third trimester of pregnancy have decreased NIP and MIP with a negative association of UFH with NIP regardless of gestational age.

INTRODUÇÃO: O período gestacional traz para a mulher alterações anatômicas e fisiológicas, em diversos sistemas, principalmente no sistema respiratório. OBJETIVO: Determinar se há associação da idade gestacional (IG), Diabetes, altura de fundo de útero (AFU), Índice de Massa Corpórea (IMC), nível de dispneia e atividade física com as pressões respiratória máxima e nasal. MÉTODO: Estudo transversal que incluiu 55 gestantes de alto risco no 2º e 3º trimestre de gestação do Ambulatório de Obstetrícia do Hospital das Clínicas de Recife ­ PE, foi descrito os dados pessoais, sociodemográficos, antropométricos, clínicos e das Pressão Inspiratória Máxima (PImáx) e pressão inspiratória nasal (PIN), que, por meio da regressão e da análise multivariada analisou a influência dos fatores de risco para gestação de alto risco com a PIN considerando um p<0,005. RESULTADOS: Dentre as gestantes, segundo as variáveis clínicas, observou-se que 56,4% (n=31) apresentaram idade gestacional acima de 28 semanas, 27,3% (n=15) diabetes, 25,5% (n=14) asma, 43,6% (n=24) Hipertensão Arterial Sistêmica Gestacional (HASG), 56,4% (n=31) obesidade, 85,5% (n=47) queixas de dispneia 38,1% (n=21) dispneia de um pouco forte a forte e 65,5% (n=36) atividade física vigorosa. Foram encontrados valores baixos de PImáx (76,76 cmH2O) e PIN (68,62 cmH2O) para a idade. Foi observada uma associação do aumento da AFU com a diminuição de 0,8 cmH2O da PIN, independentemente da idade gestacional. CONCLUSÃO: As gestantes de alto risco no segundo e terceiro trimestre gestacional apresentam PIN e PImáx diminuídas com uma associação negativa da AFU com a PIN independentemente da idade gestacional.

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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.

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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.

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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.

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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.

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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)

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El linfoma no Hodgkin compromete en un 50 % de los casos estructuras intratorácicas; la afectación bronquial es infrecuente. Presentamos el caso de una paciente con sospecha de enfermedad linfoproliferativa e infiltrados pulmonares. En la broncoscopia se observaron imágenes nodulares difusas prominentes de todo el árbol bronquial. La anatomía patológica de la biopsia bronquial fue compatible con linfoma no Hodgkin marginal y la evolución con quimioterapia fue favorable(AU)

Thoracic involvement occurs in 50 % of cases of Non-Hodgkin lymphoma. Bronchial involvement is rare. We describe a case presenting with probable lymphoproliferative disease and pulmonary infiltrates. Bronchoscopy revealed prominent diffuse nodular images throughout the bronchial tree. Bronchial biopsy yielded a diagnosis of marginal Non-Hodgkin Lymphoma. The patient had a good response to chemotherapy(AU)

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El sarcoma primario de arteria pulmonar es un tumor poco frecuente y con muy mal pronóstico. Su incidencia es difícil de estimar. Presenta una sintomatología inespecífica que dificulta y retrasa su diagnóstico. Presentamos un paciente de 62 años diagnostica do inicialmente de tromboembolismo pulmonar con una clínica discordante. Se decide continuar el estudio, que confirma posteriormente el diagnóstico de sarcoma primario de arteria pulmonar mediante biopsia(AU)

Primary pulmonary artery sarcoma is a rare tumor with a very poor prognosis. Its inci dence is hard to estimate. It presents with nonspecific symptoms, delaying the diagnosis and making it challenging. We present a 62-year-old patient initially diagnosed with pulmonary thromboembolism, exhibiting discordant symptoms. Further investigation was pursued, ultimately confirming the diagnosis of primary pulmonary artery sarcoma through biopsy(AU)

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El concepto de "dificultad para respirar" enfatiza la naturaleza subjetiva multidimensional de la disnea con componentes físicos, psicológicos, sociales, espirituales y existencia les. La "disnea total" aboga por un enfoque integral y centrado en el paciente más allá de la enfermedad. La noción de "disnea refractaria" debería ser evitada; implica cierto nihilismo terapéutico. Por tal motivo, se acuñó el concepto de "síndrome de disnea crónica" para reconocer las posibilidades de tratamiento y concientizar a los pacientes, los médicos, el equipo de salud y los investigadores. Las personas que viven con enfermedades respiratorias avanzadas y disnea crónica grave (y sus allegados) tienen una mala calidad de vida. El modelo clínico-conceptual "respirando-pensando-funcionando" incluye las tres reacciones cognitivas y conductuales predominantes que, al provocar círculos viciosos, empeoran y mantienen el síntoma. Para la evaluación exhaustiva de la disnea se dispone de diversos instrumentos que consideran la experiencia sensorial-perceptiva, la angustia afectiva (distress) y el impacto o carga de los síntomas. La dificultad para respirar durante las últimas semanas o días de vida puede denominarse "disnea terminal". Es un síntoma frecuente y uno de los más angustiantes en la última fase de la vida de pacientes con cáncer. En estas circunstancias el autorreporte puede subestimar la dificultad respiratoria. La Escala de Observación de Dificultad Respiratoria es el primer y único instrumento de evaluación de la disnea destinado a evaluar su presencia e intensidad en pacientes que no pueden comunicarse(AU)

The concept of shortness of breath emphasizes the multidimensional subjective nature of dyspnea with physical, psychological, social, spiritual, and existential components. "Total dyspnea" advocates a comprehensive, patient-centred approach beyond the disease. The term "refractory dyspnea" should be avoided; it implies a certain therapeutic nihil ism. For this reason, the term "chronic dyspnea syndrome" was coined to recognize the possibilities of treatment and raise awareness among patients, physicians, the health team, and researchers. People with advanced respiratory disease and severe chronic breathlessness (and those close to them) have a poor quality of life. The Breathing- Thinking-Functioning clinical-conceptual model includes the three predominant cognitive and behavioral reactions that, by causing vicious circles, worsen and maintain the symp tom. Various instruments are available for the comprehensive assessment of dyspnea. That considers the sensory-perceptual experience, the affective anguish (distress) and the impact or burden of the symptoms. Difficulty breathing during the last weeks or days of life may be called "terminal dyspnea." It is a frequent symptom and one of the most distressing in the latest phase of the life of cancer patients. In these circumstances, self-report may underestimate respiratory distress. The Respiratory Distress Observa tion Scale is the first and only dyspnea assessment instrument to assess its presence and intensity in patients unable to communicate(AU)

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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.