Hipertensión pulmonar y leiomiomas uterinos, ¿existe relación causal? Serie de casos y revisión fisiopatológica / Pulmonary Hypertension and Uterine Leiomyomas, Is there a Causal Relationship? Case Series and Pathophysiological Review

Introducción: Los leiomiomas uterinos son un tipo de neoplasia benigna de frecuente aparición en mujeres de edad reproductiva, relacionados con enfermedad tromboem- bólica venosa. Este vínculo surge del efecto producido por la compresión de fibromas que genera estasis venosa en la región pelviana. Sin embargo, este pareciera no ser el único factor que lo relaciona con el desarrollo posterior de hipertensión pulmonar, sino que su presencia es gatillo de una serie de fenómenos que influyen sobre la vasculatu - ra pulmonar y también a nivel sistémico. Método: Revisión de una serie de casos (seis) atendidos en nuestra unidad, seguido de una revisión sobre la relación entre leiomio- mas y distintas formas de hipertensión pulmonar con una revisión desde la fisiopatología. Resultado y conclusiones: Encontramos sustento bibliográfico en los múltiples caminos fisiopatológicos que relacionan los mediadores vasculares comunes, que parecieran ser el punto clave en la relación entre estas dos patologías.

Introduction: Uterine leiomyomas are a type of benign neoplasm that frequently appears in women of reproductive age, related to venous thromboembolic disease. This link arises from the effect produced by the compression of fibroids, which generates venous stasis in the pelvic region. However, this seems not to be the only factor that re- lates it to the subsequent development of pulmonary hypertension, but rather its presence is a trigger for a series of phenomena that influence the pulmonary vasculature and also at a systemic level. Method: Review of a series of cases (six) cared for in our unit, followed by a review on the relationship between leiomyomas and different forms of pulmonary hypertension with a review from the pathophysiology. Result and conclusions: We found bibliographic support in the multiple pathophysiological paths that relate the common vascular mediators, which appear to be the key point in the relationship between these two pathologies.

Edema pulmonar por reexpansión. Reporte de Caso / Pulmonary edema due to re-expansion. Case Report

Introducción. El edema pulmonar por reexpansión es una complicación poco frecuente, secundaria a una rápida reexpansión pulmonar posterior al drenaje por toracentesis o toracostomía cerrada. Al día de hoy, se ha descrito una incidencia menor al 1 % tras toracostomía cerrada, con mayor prevalencia en la segunda y tercera década de la vida. Su mecanismo fisiopatológico exacto es desconocido; se ha planteado un proceso multifactorial de daño intersticial pulmonar asociado con un desequilibrio de las fuerzas hidrostáticas. Caso clínico. Presentamos el caso de un paciente que desarrolló edema pulmonar por reexpansión posterior a toracostomía cerrada. Se hizo una revisión de la literatura sobre esta complicación. Resultados. Aunque la clínica sugiere el diagnóstico, la secuencia de imágenes desempeña un papel fundamental. En la mayoría de los casos suele ser autolimitado, por lo que su manejo es principalmente de soporte; sin embargo, se han reportado tasas de mortalidad que alcanzan hasta el 20 %, por tanto, es importante conocer los factores de riesgo y las medidas preventivas. Conclusión. El edema pulmonar de reexpansión posterior a toracostomía es una complicación rara en los casos con neumotórax, aunque es una complicación que se puede presentar en la práctica diaria, por lo cual debe tenerse en mente para poder hacer el diagnóstico y un manejo adecuado.

Introduction. Re-expansion pulmonary edema is a rare complication secondary to rapid pulmonary re-expansion after drainage by thoracentesis and/or closed thoracostomy. As of today, an incidence of less than 1% has been described after closed thoracostomy, with a higher prevalence in the second and third decades of life. Its exact pathophysiological mechanism is unknown; a multifactorial process of lung interstitial damage associated with an imbalance of hydrostatic forces has been proposed. Clinical case. We present the case of a patient who developed pulmonary edema due to re-expansion after closed thoracostomy, conducting a review of the literature on this complication. Results. Although the clinic suggests the diagnosis, the sequence of images plays a fundamental role. In most cases, it tends to be a self-limited disease, so its management is mainly supportive. However, mortality rates of up to 20% have been recorded. Therefore, it is important to identify patients with major risk factors and initiate preventive measures in these patients. Conclusions. Re-expansion pulmonary edema after thoracostomy is a rare complication in cases with pneumothorax; however, it is a complication that can occur in daily practice. Therefore, it must be kept in mind to be able to make the diagnosis and an adequate management.

Síndrome de Eisenmenger secundario a comunicación interatrial tipo ostium secundum en mujer adulta / Eisenmenger Syndrome Associated with an ostium secundum Atrial Septal Defect in an Adult Woman

El síndrome de Eisenmenger es la forma más severa de presentación de hipertensión arterial pulmonar secundaria a defectos cardíacos congénitos no reparados, aunque su prevalencia es baja, continúa siendo un reto para los sistemas de salud de los países en vías de desarrollo por su complejidad en el manejo. Presentación del caso. Paciente femenina sin antecedentes médicos conocidos quien consulta por disnea relacionada a los esfuerzos y policitemia. Intervención terapéutica. Se realiza ecocardiograma transesofágico que arroja la presencia de defecto interatrial tipo ostium secundum e hipertensión arterial pulmonar severa, con cortocircuito de derecha a izquierda, se inicia oxigenoterapia y terapia farmacológica. Evolución clínica. Paciente permaneció ingresada presentando notable mejora a la disnea, se le dio de alta con referencia a la clínica de cardiopatías congénitas del adulto en Hospital Nacional Rosales.

Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension secondary to an unrepaired congenital heart disease. Despite the low prevalence, it remains a challenge for the public health service of developing countries due to the complexity of the treatment. Case presentation. A female patient without known medical history, who consults with dyspnea on exertion and polycythemia. Treatment. A transesophageal echocardiogram was performed, showing an ostium secundum atrial septal defect and severe pulmonary arterial hypertension with a right-left shunt. Supplemental oxygen was administrated and pharmacological treatment was started. Outcome. The patient presented remarkable clinical improvement to dyspnea, she was discharged with medical reference to the Adult Congenital Heart Disease clinic at Rosales National Hospital.

Associação do Nível de ST2 Solúvel com Mortalidade em 6 Meses e/ou Hospitalização Recorrente Relacionada a Doenças Cardiovasculares em Embolia Pulmonar / Association of soluble ST2 Level with 6-month Mortality and/or Recurrent Cardiovascular-Related Hospitalization in Pulmonary Embolism

Resumo Fundamento: A associação de supressão solúvel da tumorigênese-2 (sST2) com prognóstico em embolia pulmonar (EP) é desconhecida. Objetivo: Este estudo teve como objetivo investigar a relação entre os níveis de sST2 em pacientes com EP aguda e mortalidade em 6 meses e hospitalizações recorrentes. Métodos: Este estudo prospectivo incluiu 100 pacientes com EP aguda. Os pacientes foram classificados em dois grupos de acordo com a mortalidade em 6 meses e a presença de hospitalizações recorrentes relacionadas a doenças cardiovasculares. Dois grupos foram comparados. Um valor de p de 0,05 foi considerado estatisticamente significativo. Resultados: Os níveis de ST2 solúvel foram significativamente maiores no grupo com mortalidade e internações recorrentes. (138,6 ng/mL (56,7-236,8) vs. 38 ng/mL (26,3-75,4); p<0,001) O melhor limite de corte para níveis de sST2 na previsão de um desfecho composto de mortalidade em 6 meses e/ou a hospitalização recorrente relacionada a doenças cardiovasculares foi >89,9, com especificidade de 90,6% e sensibilidade de 65,2%, de acordo com a curva Receiver Operating Characteristic (área sob a curva = 0,798; IC 95%, 0,705-0,891; p <0,0001). Após ajuste para fatores de confusão que foram estatisticamente significativos na análise univariada ou para as variáveis correlacionadas com os níveis de sST2, nível de sST2 (OR = 1,019, IC 95%: 1,009-1,028, p 0,001) e proteína C reativa (PCR). (OR = 1,010, IC 95%: 1,001-1,021, p = 0,046) continuaram a ser preditores significativos de mortalidade em 6 meses e/ou hospitalização recorrente relacionada a doenças cardiovasculares no modelo de regressão logística múltipla através do método backward stepwise. Conclusões: O nível de ST2 solúvel parece ser um biomarcador para prever mortalidade em 6 meses e/ou hospitalização recorrente relacionada a doenças cardiovasculares em pacientes com EP aguda.

Abstract Background: The association of soluble suppression of tumorigenesis-2 (sST2) levels with prognosis in pulmonary embolism (PE) is unknown. Objective: This study aimed to investigate the relationship between sST2 levels in patients with acute PE and 6-month mortality and recurrent hospitalizations. Methods: This prospective study included 100 patients with acute PE. Patients were classified into two groups according to 6-month mortality and the presence of recurrent Cardiovascular-Related hospitalizations. Two groups were compared. A p-value of 0.05 was considered statistically significant. Results: Soluble ST2 levels were significantly higher in the group with mortality and recurrent hospitalizations. (138.6 ng/mL (56.7-236.8) vs. 38 ng/mL (26.3-75.4); p<0.001) The best cut-off threshold for sST2 levels in the prediction of a composite outcome of 6-month mortality and/or recurrent Cardiovascular-Related hospitalization was found to be >89.9 with a specificity of 90.6% and a sensitivity of 65.2%, according to the receiver operating characteristic curve (area under the curve = 0.798; 95% CI, 0.705-0.891; p <0.0001). After adjusting for confounding factors that were either statistically significant in the univariate analysis or for the variables correlated with the sST2 levels, sST2 level (OR = 1.019, 95% CI: 1.009-1.028, p 0.001) and C-reactive protein (CRP ) (OR = 1.010, 95% CI: 1.001-1.021, p = 0.046) continued to be significant predictors of 6-month mortality and/or recurrent Cardiovascular-Related hospitalization in the multiple logistic regression model via backward stepwise method. Conclusion: Soluble ST2 level seems to be a biomarker to predict 6-month mortality and/or recurrent Cardiovascular-Related hospitalization in patients with acute PE.

Relação entre a Complacência Pulmonar Estática e a Falha de Extubação em Pacientes Pós-Operatório de Cirurgia Cardíaca / Relationship between Static Lung Compliance and Extubation Failure in Postoperative Cardiac Surgery Patients

Resumo Fundamento: Pouco explorada na decisão de extubação no pós-operatório de cirurgia cardíaca, a complacência pulmonar estática seriamente afetada no procedimento cirúrgico pode levar à insuficiência respiratória e à falha na extubação. Objetivo: Avaliar a complacência pulmonar estática no pós-operatório de cirurgia cardíaca e relacionar sua possível redução aos casos de falha na extubação dos pacientes submetidos ao método fast-track de extubação. Métodos: Foram incluídos pacientes que realizaram cirurgia cardíaca com uso de circulação extracorpórea (CEC) em um hospital universitário estadual admitidos na UTI sob sedação e bloqueio residual. Tiveram sua complacência pulmonar estática avaliada no ventilador mecânico por meio do software que utiliza o least squares fitting (LSF) para a medição. No período de 48 horas após a extubação os pacientes foram observados respeito à necessidade de reintubação por insuficiência respiratória. O nível de significância adotado para os testes estatísticos foi de 5%, ou seja, p<0,05. Resultados: Obtiveram sucesso na extubação 77 pacientes (75,49%) e falharam 25 (24,51%). Os pacientes que falharam na extubação tiveram a complacência pulmonar estática mais baixa quando comparados aos que tiveram sucesso (p<0,001). Identificamos o ponto de corte para complacência por meio da análise da curva Receiver Operating Characteristic Curve (ROC) sendo o ponto de corte o valor da complacência <41ml/cmH2O associado com maior probabilidade de falha na extubação (p<0,001). Na análise de regressão múltipla, verificou-se a influência da complacência pulmonar (dividida pelo ponto de corte da curva ROC) com risco de falha 9,1 vezes maior para pacientes com complacência <41ml/cmH2O (p< 0,003). Conclusões: A complacência pulmonar estática <41ml/cmH2O é um fator que compromete o sucesso da extubação no pós-operatório de cirurgia cardíaca.

Abstract Background: Static lung compliance, which is seriously affected during surgery, can lead to respiratory failure and extubation failure, which is little explored in the decision to extubate after cardiac surgery. Objective: To evaluate static lung compliance in the postoperative period of cardiac surgery and relate its possible reduction to cases of extubation failure in patients submitted to the fast-track method of extubation. Methods: Patients undergoing cardiac surgery using cardiopulmonary bypass (CPB) at a state university hospital admitted to the ICU under sedation and residual block were included. Their static lung compliance was assessed on the mechanical ventilator using software that uses least squares fitting (LSF) for measurement. Within 48 hours of extubation, the patients were observed for the need for reintubation due to respiratory failure. The level of significance adopted for the statistical tests was 5%, i.e., p<0.05. Results: 77 patients (75.49%) achieved successful extubation and 25 (24.51%) failed extubation. Patients who failed extubation had lower static lung compliance compared to those who succeeded (p<0.001). We identified the cut-off point for compliance through analysis of the Receiver Operating Characteristic Curve (ROC), with the cut-off point being compliance <41ml/cmH2O associated with a higher probability of extubation failure (p<0.001). In the multiple regression analysis, the influence of lung compliance (divided by the ROC curve cut-off point) was found to be 9.1 times greater for patients with compliance <41ml/cmH2O (p< 0.003). Conclusions: Static lung compliance <41ml/cmH2O is a factor that compromises the success of extubation in the postoperative period of cardiac surgery.

A mudança de paradigma no tratamento do tromboembolismo venoso grave / The paradigm shift in treatment of severe venous thromboembolism

Resumo A embolia pulmonar (EP) é a terceira maior causa de morte cardiovascular e a principal de morte evitável intra-hospitalar no mundo. O conceito PERT® (do inglês, pulmonary embolism response team) envolve seu diagnóstico e tratamento precoce e multidisciplinar. A trombose venosa profunda (TVP) é a sua causa inicial na maioria dos casos e é responsável por complicações como a recidiva tromboembólica, a síndrome pós-trombótica e a hipertensão pulmonar tromboembólica crônica. Uma abordagem inicial semelhante ao PERT nos casos de TVP ilíaco-femoral grave pode reduzir não apenas o risco imediato de EP e morte, mas também suas sequelas tardias. Novas técnicas percutâneas e aparatos de trombectomia mecânica para o tromboembolismo venoso (TEV) vêm demonstrando resultados clínicos encorajadores. Propomos o desenvolvimento de um conceito ampliado de resposta rápida ao TEV, que envolve não apenas a EP (PERT®) mas também os casos graves de TVP: o time de resposta rápida para o TEV (TRETEV®), ou do inglês Venous Thromboembolism Response Team (VTERT®).

Abstract Pulmonary embolism (PE) is the third leading cause of cardiovascular death and the main cause of preventable in-hospital death in the world. The PERT® (Pulmonary Embolism Response Team) concept involves multidisciplinary diagnosis and immediate treatment. Deep venous thrombosis (DVT) is the initial cause of most cases of PE and is responsible for complications such as chronic thromboembolic recurrence, postthrombotic syndrome, and chronic thromboembolic pulmonary hypertension. An aggressive approach to severe cases of iliofemoral DVT similar to the PERT® system can not only reduce the immediate risk of PE and death but can also reduce later sequelae. New percutaneous techniques and mechanical thrombectomy devices for venous thromboembolism (VTE) have shown encouraging clinical results. We propose the development of an expanded concept of rapid response to VTE, which involves not only PE (PERT®) but also severe cases of DVT: the Venous Thromboembolism Response Team (VTERT®).

Tromboembolismo pulmonar agudo de riesgo intermedio: ¿está indicada la trombólisis sistémica? / Acute intermediate-risk pulmonary embolism: is systemic thrombolytic therapy indicated?

Introducción: el tromboembolismo pulmonar (TEP) se ha clasificado de acuerdo con las características del paciente y los hallazgos paraclínicos en riesgo bajo, intermedio y alto, siendo la mayor dificultad para el clínico el manejo del TEP de tipo intermedio, antes conocido como submasivo, donde existe controversia sobre las recomendaciones para trombólisis sistémica. Algunos autores y publicaciones señalan su uso, pero la Sociedad Europea de Cardiología (ESC) y la Asociación Americana de Hematología (ASH) no lo recomiendan. Materiales y métodos: revisión sistemática de la literatura con un total de 28 artículos extraídos de la búsqueda en las bases de datos; de estos, 7 cumplían con los criterios de inclusión (6 revisiones sistemáticas/metaanálisis y una prueba de análisis secuencial sobre ensayos aleatorizados), para un compendio de 39.879 pacientes con TEP. Resultados: dentro de los desenlaces evaluados, el sangrado mayor ocurrió en 8.1 a 9.24%, la mortalidad en quienes se les realizó trombólisis sistémica se reportó entre un 2.1 y 2.2%, encontrando disminución en el riesgo de deterioro hemodinámico al comparar la trombólisis sistémica con la anticoagulación, informando 4.1% versus 14.1% respectivamente. Discusión y conclusiones: en los estudios analizados, la trombólisis sistémica en los pacientes con TEP de riesgo intermedio no demostró impacto en cuanto a mortalidad ni desarrollo de hipertensión pulmonar secundaria, sin embargo, existe evidencia contradictoria respecto a su posible valor en la recurrencia del evento, que en la mayoría de los casos sobrepasa el riesgo de sangrado mayor (cercano a 10%).

Introduction: pulmonary embolism (PE) can be classified into low, intermediate, and high-risk, based on the patient characteristics and symptoms. Intermediate-risk PE, formerly known as submassive PE, poses the greatest challenge to clinicians, as indication for systemic thrombolytic therapy, remains controversial. Some authors and publications recommend its use, but the European Society of Cardiology (ESC) and the American Society of Hematology (ASH) do not. Materials and methods: a systematic literature review of 28 articles retrieved from search databases; of which, 7 met the inclusion criteria (6 systematic reviews/meta-analyses and a sequential analysis test on randomized trials) analyzing 39.879 PE patients. Results: among the assessed outcomes, major bleeding occurred in 8.1 to 9.24%, mortality in those who underwent systemic thrombolytic therapy was reported to be 2.1 to 2.2%; when comparing systemic thrombolytic therapy with anticoagulation therapy for preventing hemodynamic deterioration, the results were 4.1% and 14.1% respectively. Discussion and conclusions: the analyzed studies evidenced no impact on mortality or development of secondary pulmonary hypertension, in intermediate-risk PE patients receiving systemic thrombolytic therapy. However, results on its possible prevention of PE recurrence, while exceeding the risk of major bleeding (in nearly 10%), in most cases, are contradictory.

Riesgo cardiovascular en pacientes con esclerosis sistémica / Cardiovascular Risk in Patients with Systemic Sclerosis

Introducción: El riesgo cardiovascular es importante en la evaluación de los pacientes con esclerosis sistémica. Objetivo: Determinar el riesgo cardiovascular en pacientes con esclerosis sistémica. Métodos: Se realizó un estudio transversal y descriptivo en pacientes protocolizados del Servicio de Reumatología, en el período de enero 2020 a enero 2022. Se recogieron variables demográficas, clínicas, y se aplicó la calculadora de riesgo cardiovascular Framingham. Resultados: Se incluyeron 105 pacientes con edad media de 48,6 ± 15,3 años, el grupo más frecuente de 50 a 59 años (36,2 por ciento), predominó el sexo femenino 92,2 por ciento el color de piel blanca (74,3 por ciento), el tiempo de evolución fue mayor a 5 años (66,7 por ciento) con una media de 10,5 ± 9,3. El valor promedio de la escala de gravedad modificada de Medsger fue 5,1 ± 2,7 y el 72,4 por ciento con afectación leve. El fenómeno de Raynaud y la fibrosis pulmonar fueron más frecuentes con un 89,5 por ciento y 55,2 por ciento. El índice de Rodnan en promedio fue de 13,1 ± 8,0 y los reactantes de fase aguda normales en la mayoría. Los factores de riesgo cardiovascular más frecuentes fueron la HTA (30,2 por ciento) y dislipidemia (19,9 por ciento). El índice de masa corporal que predominó fue de peso adecuado (54,3 por ciento). Predominó el riesgo cardiovascular bajo según score de Framingham (86 por ciento). Existieron diferencias significativas entre las medias del tiempo de evolución y el riesgo cardiovascular (10 ± 6,9 frente a 9,6 ± 8,8 frente a 16,9 ± 10,8; p = 0,032). Conclusiones: El riesgo cardiovascular en los pacientes con esclerosis sistémica fue bajo(AU)

Introduction: Cardiovascular risk is important in the evaluation of patients with systemic sclerosis. Objective: To determine the cardiovascular risk in patients with systemic sclerosis. Methods: A cross-sectional and descriptive study was carried out in protocolized patients of Rheumatology Service, from January 2020 to January 2022. Demographic and clinical variables were collected, and Framingham cardiovascular risk calculator was used. Results: One hundred five patients were included with a mean age of 48.6 ± 15.3 years, the most frequent group was 50 to 59 years (36.2percent), female sex (92.2percent) predominated, as well as white skin color (74.3percent). The evolution time was greater than 5 years (66.7percent) with a mean of 10.5 ± 9.3. The average value of modified Medsger severity scale was 5.1 ± 2.7 and 72.4percent had mild involvement. Raynaud's phenomenon and pulmonary fibrosis were more common at 89.5percent and 55.2percent. Rodnan index on average was 13.1 ± 8.0 and the acute phase reactants were normal in the majority. The most frequent cardiovascular risk factors were HBP (30.2percent) and dyslipidemia (19.9percent). The predominant body mass index was adequate weight (54.3percent). Low cardiovascular risk according to Framingham score prevailed (86percent). There were significant differences between the mean duration of evolution and cardiovascular risk (10 ± 6.9 vs. 9.6 ± 8.8 vs. 16.9 ± 10.8; p = 0.032). Conclusions: The cardiovascular risk in patients with systemic sclerosis was low(AU)

Real-time changes in rib cage expansion and use of abdominal mechanical stimulation in newborns: a quasi-experimental study / Mudanças na expansibilidade torácica com o uso de estímulo mecânico na região abdominal em recém-nascidos: um estudo quase-experimental

ABSTRACT Objective: To assess the rib cage expansion and respiratory rate in newborns using an abdominal stabilization band. Methods: The study included 32 newborns of both genders, with gestational age between 35 and 41 weeks. The abdominal stabilization band was used for 15 minutes between the xiphoid process and the anterosuperior iliac crest, with an abdominal contention 0.5cm smaller than the abdominal circumference. The rib cage expansion was evaluated by a breathing transducer (Pneumotrace II™) three minutes before using the band, during the use (15 minutes), and ten minutes after removing the band. The Shapiro-Wilk test verified data normality, and the Wilcoxon test compared the variables considering rib cage expansion and respiratory rate. Significance was set to p<0.05. Results: There was an increase in respiratory rate when comparing before and ten minutes after removing (p=0.008) the abdominal stabilization band, as well as when comparing during its use and ten minutes after its removal (p=0.001). There was also an increase in rib cage expansion when comparing before and during the use of the abdominal stabilization band (p=0.005). Conclusions: The use of the abdominal stabilization band promoted an increase in the rib cage expansion and respiratory rate in the assessed newborns and may be a viable option to improve the respiratory kinematics of this population.

RESUMO Objetivo: Avaliar a expansibilidade torácica e a frequência respiratória em recém-nascidos que fizeram uso de uma faixa de estabilização abdominal. Métodos: O estudo incluiu 32 recém-nascidos de ambos os sexos, com idade gestacional entre 35 e 41 semanas. A faixa de estabilização abdominal foi mantida por 15 minutos entre o processo xifoide e a espinha ilíaca anterossuperior, com contenção abdominal 0,5 cm menor do que a circunferência abdominal. A expansibilidade torácica foi avaliada por um transdutor piezoelétrico (Pneumotrace II™) 3 minutos antes do uso da faixa, durante 15 minutos, e 10 minutos após sua retirada. A normalidade das variáveis foi testada pelo teste de Shapiro-Wilk e a análise comparativa da expansibilidade torácica e da frequência respiratória foi realizada por meio do teste t pareado, considerando-se p<0,05. Resultados: Houve aumento da frequência respiratória quando comparados os tempos antes do uso da faixa e 10 minutos após a retirada (p=0,008), bem como quando comparados os tempos durante o uso e 10 minutos após a retirada da faixa (p=0,001). Houve aumento da expansibilidade torácica quando comparados os tempos antes e durante o uso da faixa (p=0,005). Conclusões: O uso da faixa de estabilização abdominal conferiu aumento da expansibilidade torácica e da frequência respiratória nos recém-nascidos estudados, podendo ser uma opção viável para a melhora da cinemática respiratória dessa população.

Respiratory system parameters in children with low severity cystic fibrosis: is there early involvement in relation to healthy peers? / Parâmetros do sistema respiratório em crianças com fibrose cística de baixa gravidade: há acometimento precoce em relação a pares saudáveis?

ABSTRACT Objective: To compare and analyze pulmonary function and respiratory mechanics parameters between healthy children and children with cystic fibrosis. Methods: This cross-sectional analytical study included healthy children (HSG) and children with cystic fibrosis (CFG), aged 6-13 years, from teaching institutions and a reference center for cystic fibrosis in Florianópolis/SC, Brazil. The patients were paired by age and sex. Initially, an anthropometric evaluation was undertaken to pair the sample characteristics in both groups; the medical records of CFG were consulted for bacterial colonization, genotype, and disease severity (Schwachman-Doershuk Score — SDS) data. Spirometry and impulse oscillometry were used to assess pulmonary function. Results: In total, 110 children were included, 55 in each group. In the CFG group, 58.2% were classified as excellent by SDS, 49.1% showed the ΔF508 heterozygotic genotype, and 67.3% were colonized by some pathogens. Statistical analysis revealed significant differences between both groups (p<0.05) in most pulmonary function parameters and respiratory mechanics. Conclusions: Children with cystic fibrosis showed obstructive ventilatory disorders and compromised peripheral airways compared with healthy children. These findings reinforce the early changes in pulmonary function and mechanics associated with this disease.

RESUMO Objetivo: Comparar e analisar parâmetros de função pulmonar e de mecânica respiratória entre escolares saudáveis e com fibrose cística (FC). Métodos: Estudo transversal que incluiu escolares saudáveis (GES) e com FC (GFC), com idades entre seis e 13 anos, provenientes de instituições de ensino e de um centro de referência da FC em Florianópolis/SC, Brasil, pareados por idade e sexo, respectivamente. Inicialmente, conduziu-se avaliação antropométrica para pareamento e caracterização de ambos os grupos e, no GFC, consultou-se prontuário médico para registro dos dados de colonização bacteriana, genótipo e gravidade da doença (Escore de Schwachman-Doershuk — ESD). Para a avaliação da função pulmonar, realizou-se espirometria e a avaliação da mecânica respiratória foi conduzida por meio do sistema de oscilometria de impulso. Resultados: Participaram 110 escolares, 55 em cada grupo. No GFC, 58,2% foram classificados pelo ESD como excelentes, 49,1% apresentaram genótipo ∆F508 heterozigoto e 67,3% eram colonizados por alguma patógeno. Houve diferença significativa (p<0,05) na maioria dos parâmetros de função pulmonar e de mecânica respiratória entre os grupos. Conclusões: Escolares com FC apresentaram distúrbio ventilatório obstrutivo e com comprometimento de vias aéreas periféricas, em comparação aos escolares hígidos. Esse evento reforça o início precoce da alteração de função pulmonar e de mecânica respiratória nessa enfermidade, evidenciados pelos achados desta investigação.

Relative incidence of interstitial lung diseases in Brazil / Incidência relativa de doenças pulmonares intersticiais no Brasil

ABSTRACT Objective: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. Methods: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. Results: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (c2 = 312.4; p < 0.001). Conclusions: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.

RESUMO Objetivo: Avaliar a frequência relativa de casos incidentes de doenças pulmonares intersticiais (DPI) no Brasil. Métodos: Levantamento retrospectivo de casos novos de DPI em seis centros de referência entre janeiro de 2013 e janeiro de 2020. O diagnóstico de DPI seguiu os critérios sugeridos por órgãos internacionais ou foi feito por meio de discussão multidisciplinar (DMD). A condição foi caracterizada como DPI não classificável quando não houve um diagnóstico final específico após a DMD ou houve discordância entre dados clínicos, radiológicos ou histológicos. Resultados: A amostra foi composta por 1.406 pacientes (média de idade = 61 ± 14 anos), sendo 764 (54%) do sexo feminino. Dos 747 casos expostos a antígenos para pneumonite de hipersensibilidade (PH), 327 (44%) tiveram diagnóstico final de PH. Houve relato de história familiar de DPI em 8% dos casos. Os achados de TCAR foram indicativos de fibrose em 74% dos casos, incluindo faveolamento, em 21%. Autoanticorpos relevantes foram detectados em 33% dos casos. Biópsia transbrônquica foi realizada em 23% dos pacientes, e biópsia pulmonar cirúrgica, em 17%. Os diagnósticos finais foram: DPI associada à doença do tecido conjuntivo (em 27%), PH (em 23%), fibrose pulmonar idiopática (em 14%), DPI não classificável (em 10%) e sarcoidose (em 6%). Os diagnósticos variaram significativamente entre os centros (c2 = 312,4; p < 0,001). Conclusões: Nossos achados mostram que DPI associada à doença do tecido conjuntivo é a DPI mais comum no Brasil, seguida pela PH. Esses resultados destacam a necessidade de uma estreita colaboração entre pneumologistas e reumatologistas, a importância de fazer perguntas detalhadas aos pacientes a respeito da potencial exposição a antígenos e a necessidade de campanhas de saúde pública destinadas a enfatizar a importância de evitar essa exposição.

Equilíbrio, quedas e risco de quedas na DPOC: revisão sistemática dos instrumentos de avaliação, propriedades de medida e utilidade clínica / Balance, falls, and risk of falls in COPD: systematic review of assessment instruments, measurement properties, and clinical utility

Resumo Objetivos Identificar os instrumentos de avaliação de equilíbrio, quedas e risco de quedas utilizados na DPOC, avaliar suas propriedades de medida, qualidade da evidência e utilidade clínica. Método A revisão foi produzida seguindo as orientações PRISMA e COSMIN, registrada no PROSPERO: CRD42021235118. As pesquisas foram realizadas de novembro de 2021 a setembro de 2022 nas bases de dados PubMed, LILACS, CINAHL, Embase, Web of Science e PEDro. Estudos observacionais transversais e coorte foram incluídos, sem restrição de idioma ou ano de publicação, desde que descreveram instrumentos clínicos de avaliação do equilíbrio, quedas e risco de quedas reportando no mínimo, uma das propriedades de medida: validade, confiabilidade e responsividade. Dois revisores independentemente aplicaram os critérios de elegibilidade, risco de viés pela COSMIN, qualidade da evidência pela abordagem GRADE e a avaliação da utilidade clínica pela Escala de Tyson e Connell. Resultados 9.102 estudos foram selecionados e 21 incluídos na revisão, nove estudos demonstraram propriedades de medida adequadas e suficientes e 12 instrumentos foram identificados, dos quais seis, foram avaliados quanto a qualidade de evidência. Conclusão Revisões sistemáticas de propriedades de medida requerem revisores especializados e habilidade em análise qualitativa. Com grau de recomendação "A", a Berg Balance Scale (BBS) e o teste Timed Up and Go (TUG) foram os instrumentos mais indicados na DPOC. Ao acrescentar a avaliação da utilidade clínica ao resultado, o TUG demonstra superioridade ao BBS, demostrando ser uma ótima ferramenta para triar indivíduos que necessitem de uma avaliação minuciosa do equilíbrio, quedas e risco de quedas.

Abstract Objectives to identify balance assessment instruments, issues and risks of issues used in COPD, evaluate their measurement properties, quality of evidence and clinical utility. Method A review was produced following the PRISMA and COSMIN guidelines, registered in PROSPERO: CRD42021235118. Searches were carried out from November 2021 to September 2022 in the PubMed, LILACS, CINAHL, Embase, Web of Science and PEDro databases. Cross-sectional and cohort observational studies were included, without restrictions on language or year of publication, as long as they described clinical instruments for assessing balance, falls and risk of falls reporting at least one of the measurement properties: validity, reliability and responsiveness. Two reviewers will independently apply the eligibility criteria, travel risk by COSMIN, quality of evidence by the GRADE approach and assessment of clinical utility by the Tyson and Connell Scale. Results 9,102 studies were selected and 21 included in the review, nine studies demonstrated adequate and sufficient measurement properties and 12 instruments were identified, of which six were evaluated for the quality of evidence. Conclusion Systematic reviews of measurement properties require specialized reviewers and skills in qualitative analysis. With a recommendation GRADE of "A", the Berg Balance Scale (BBS) and the Timed Up and Go (TUG) test were the most recommended instruments for COPD. By requiring the evaluation of the clinical utility of the result, the TUG demonstrates superiority to the BBS, proving to be a great tool for judging individuals who need a thorough assessment of balance, falls and risk of falls.

Impact of COVID-19 social distancing recommendations on pulmonary function, nutritional status, and morbidity in patients with cystic fibrosis / Impacto das recomendações de distanciamento social por COVID-19 sobre a função pulmonar, estado nutricional e morbidade em pacientes com fibrose cística

ABSTRACT Objective: To evaluate the impact of COVID-19 social distancing recommendations on nutritional status, pulmonary function, and morbidity in patients with cystic fibrosis (CF). Methods: A retrospective cohort study including patients older than six years with a diagnosis of CF was performed. Demographic and clinical data, anthropometric measurements, pulmonary function, days of antibiotic use, and length of hospital stay were recorded. Variables were recorded at three time points relative to the baseline for implementation of social distancing measures: T-1 (14 months before implementation), T0 (baseline), and T1 (14 months after implementation). Delta (Δ) was calculated for each period: Δ1 (pre-pandemic T0-T-1) and Δ2 (pandemic T1-T0). Results: The study included 25 patients, with a mean age of 11.7±4.3 years. The mean forced expiratory volume in the first second (FEV1) was 85.6±23.6%, and body mass index (BMI) was 17.5±3.0 kg/m2. When comparing the two periods (Δ1 and Δ2), there was a significant increase in the FEV1/forced vital capacity (FVC) ratio (p=0.013) and in the forced expiratory flow between 25 and 75% of vital capacity (FEF25-75%) (p=0.037) in the pandemic period. There was also a significant reduction (p=0.005) in the use of antibiotics in the pandemic period compared with the pre-pandemic period. The Δ1 and Δ2 values did not differ significantly for BMI, FEV1, or length of hospital stay. Conclusions: COVID-19 social distancing recommendations had a positive impact (decrease) on morbidity (use of antibiotics) and small airway obstruction (FEF25-75%) in patients with CF.

RESUMO Objetivo: Avaliar o impacto das recomendações de medidas de distanciamento social por COVID-19 sobre estado nutricional, função pulmonar e morbidade em pacientes com fibrose cística (FC). Métodos: Estudo de coorte, retrospectivo, que incluiu pacientes com diagnóstico de FC e idade superior a seis anos. Foram registrados os dados demográficos, antropométricos, clínicos, de função pulmonar e o total de dias de uso de antibiótico e de hospitalizações. As variáveis foram registradas em três momentos relativos ao início das recomendações de distanciamento social: T-1 (14 meses antes), T0 (início das recomendações) e T1 (14 meses depois). Foram calculados deltas (Δ) para cada um dos períodos: Δ1 (pré-pandemia T0-T-1) e Δ2 (pandemia T1-T0). Resultados: Vinte e cinco pacientes, com média de idade de 11,7±4,3 anos, sendo 76% homozigotos para Δf508 e 28% colonizados por Pseudomonas aeruginosa, foram incluídos. A média do volume expiratório forçado no primeiro segundo (VEF1) foi de 85,6±23,6 (% do previsto) e o índice de massa corporal (IMC) foi de 17,5±3,0 kg/m2. Ao compararmos os períodos (Δ1 e Δ2), houve aumento significativo do VEF1/CVF (p=0,013) e do FEF25-75% (p=0,037) no período das recomendações de distanciamento. Também se observou redução significativa (p=0,005) do uso de antibióticos no período da pandemia em comparação ao período anterior a ela. Não houve diferenças significativas nos deltas para o IMC, VEF1 e dias de hospitalização. Conclusões: As recomendações de distanciamento social por COVID-19 tiveram impacto positivo (redução) sobre a morbidade (uso de antibióticos) e a obstrução de vias aéreas de menor calibre (FEF25-75%) em pacientes com FC.

Secuelas respiratorias clínicas, funcionales y tomográficas de pacientes hospitalizados por Covid-19 al año del diagnóstico: ESTUDIO SECUELAR-19 / Clinical, functional and tomographic respiratory sequels of patients hospitalized for COVID-19 at one year of diag nosis: SECUELAR-19 TRIAL

Resumen Introducción : Hay información escasa sobre evolución a largo plazo de pacientes hospitalizados por neumo nía COVID-19 moderada (NM) y grave (NG). El objetivo del estudio fue determinar impacto clínico, funcional respiratorio y tomográfico (TACAR) luego del alta a 12 meses del diagnóstico. Métodos : análisis según grupos NM y NG, desatura dores (PD) en prueba de caminata 6 min (PC6M) y patrón tomográfico símil fibrótico (SF). Comparamos resultados a 3 y 12 meses de seguimiento. Resultados : 194 pacientes enrolados, evaluados al año: 103 (53% ): masculinos (62.4%), edad 57.7 ± 10.9 años, comorbilidades (hipertensión arterial 38.8%, dia betes 29.6%, antecedentes respiratorios-AR- 18.4%). Com paramos variables a los 3 y 12 meses (media/DE): CVF 84%(19) a 88(19) (p = 0.01); 27% tuvo CVF<80% al año. En PC6M: 426 metros (108) a 447(92) (p = < 0.01). El 12.8% fue PD al año. NG tuvo mayor SF (40% vs. 27.9% p = 0.021). SF se relacionó con CVF <80% (p = 0.004) en toda la co horte, y NG (p < 0.001). Al año en análisis multivariado se asoció a CVF <80%, AR (OR 4.32, 1.15-16.25), diabetes (OR 2.96, 1.02-8.57) y patrón SF (OR 3.51, 1.25-9.88). PD se asoció a AR (OR 12.2, 2.41-61.85). Discusión : Se observó mejoría en todas las variables al año. Pero al año persisten alteraciones funcionales y tomográficas en <50% de los pacientes. El subgrupo de PD se relacionó a AR. Es importante el seguimiento protocolizado de los pacientes hospitalizados, especial mente los grupos NG, PD y SF.

Abstract Introduction : There is scarce information on longterm evolution of hospitalized patients with moderate (MP) and severe (SP) COVID-19 pneumonia. Objective: to de termine clinical, respiratory function, and tomographic (HRCT) impact after being discharged 12 months after diagnosis. Methods : Analysis according to MP and SP, desatura tor patients (DP) in 6-minute walking test (6MWT) and HRCT fibrotic-like pattern (FLP). Results compared at 3 and 12 months of follow-up. Results : 194 patients enrolled and one year later 103 (53%) were evaluated: gender male (62.4%), age 57.7 ± 10.9 years, comorbidities (arterial hypertension 38.8%, diabetes 29.6%, and respiratory diseases-RD-18.4%). Variables compared 3 months to 12 months (mean/SD): FVC: 84%( 19) to 88%( 19) (p= 0.01). A 27% of patients had FVC<80% at one year. In 6MWT:426 (108) to 447 (92) (p = <0.01). 12.8% are DP in one year. SP had a greater FLP than MP (40% vs. 27.9%, p = 0.021). The FLP group was related to FVC < 80% (p = 0.004) in all patients but only in SP (p < 0.001). After one year, in multivariate analysis, FVC < 80% was associated with RD (OR 4.32, 1.15-16.25), diabetes (OR 2.96, 1.02-8.57) and FLP (OR 3.51, 1.25-9.88). DP were associated with RD (OR 12.2, 2.41-61.85). Discussion : Improvement was observed in all vari ables when comparing 3 to 12 months. However, after one year, functional and tomographic alterations persist in less than 50% of patients. DP subgroup was related to RD. Protocolled follow-up of hospitalized patients is important, especially in SP, DP, and FLP groups.

Uso de inhaladores para la enfermedad pulmonar obstructiva crónica en un grupo de pacientes colombianos

Introducción: En Colombia son escasos los datos sobre el uso de los inhaladores en pacientes con EPOC. Objetivo: Describir la técnica de uso de inhaladores de dosis medida y polvo seco en pacientes de un hospital colombiano. Materiales y métodos: Estudio descriptivo en pacientes mayores de 40 años con EPOC atendidos en un hospital en La Virginia, Risaralda, Colombia, entre el 1 de septiembre de 2019 al 31 de enero de 2020. La unidad de análisis fueron los pacientes. Se incluyeron variables sociodemográficas, clínicas y lista de chequeo para uso de inhaladores. Se aplicaron frecuencias y proporciones para variables discretas, estadísticas de tendencia central y dispersión para variables continuas. Resultados: Se incluyeron 104 pacientes con edad media de 73,6 ± 10,1 años; 57 eran mujeres (54,8 %). Además, 48 pacientes estaban clasificados como GOLD-D (46,2 %). Igualmente, 89 pacientes manifestaron haber recibido educación sobre el uso de broncodilatadores (85,6 %). Los más frecuentes fueron los inhaladores de dosis medida (DM) en 95 casos (91,3 %), seguido de los de polvo seco unidosis (7,7 %). Así mismo, 37 pacientes que usaron DM sin inhalocámara (35,6 %) no cumplieron los pasos de la lista de chequeo. En el sistema multidosis, el más realizado fue cerrar de manera adecuada el inhalador y el menos ejecutado, expulsar el aire lentamente evitando hacerlo cerca del inhalador (n = 6; 5,7 %). Discusión: Se lograron describir las características de la técnica de uso de los inhaladores en pacientes con EPOC. A pesar de que ningún paciente logró utilizar el inhalador de forma "perfecta", la mayoría han recibido educación por parte de los profesionales de la salud. Conclusión: Un alto porcentaje de pacientes usa inadecuadamente los dispositivos para suministrar los broncodilatadores. Esto puede impactar negativamente en el control de la enfermedad.

Introduction: In Colombia, there is limited data on the use of inhalers in patients with COPD. Objective: The objective was to describe the technique of using metered-dose inhalers and dry powder in patients in a Colombian hospital. Methods: Observational, descriptive study of patients over 40 years of age with COPD, treated in a hospital in La Virginia, Risaralda, Colombia, between September 1st, 2019 and January 31st, 2020. The unit of analysis were patients in consultation. Sociodemographic and clinical variables, and a checklist for use of inhalers were included. Frequencies and proportions were applied for discrete variables, statistics of central tendency and dispersion for continuous variables. Results: A total of 104 patients with an average age of 73.6 ± 10.1 years were included; 57 were women (54.8%). In addition, 48 patients were classified as GOLD-D (46.2%). Similarly, 89 patients reported having received education on the use of bronchodilators (85.6%). The most common were metered-dose (MD) inhalers in 95 cases (91.3%), followed by single-dose dry powder inhalers in eight patients (7.7%). Likewise, 37 patients who used DM without inhalochamber (35.6%) did not comply with the steps of the checklist. In the multidose system, the most performed was to properly close the inhaler and the least performed was to expel the air slowly, avoiding doing so near the inhaler (n=6; 5.7%). Discussion: The characteristics of the technique of using inhalers in patients with COPD were described. Although no patient was able to use the inhaler "perfectly", most have received education from health professionals. Conclusion: A high percentage of patients misuse the devices to deliver bronchodilators. This can negatively impact the control of the disease.

Comparación de dos estrategias educativas para pacientes con EPOC durante la rehabilitación pulmonar

Introducción: La Enfermedad Pulmonar Obstructiva Crónica (EPOC) ocasiona en quienes la padecen limitación física, emocional, social y deterioro de la calidad de vida relacionada con la salud (CVRS). El componente educativo es indispensable en todo programa de Rehabilitación Pulmonar (RP), ya que permite mantener resultados de intervención a largo plazo. Objetivo: Comparar el efecto de dos estrategias educativas para pacientes con EPOC desarrolladas en dos programas de RP en Cali, Colombia. Métodos: Estudio prospectivo longitudinal. Durante un periodo de 24 meses se seleccionaron por conveniencia pacientes con EPOC que aceptaron firmar el consentimiento informado. Los pacientes pertenecían a dos programas de RP, uno que realizó RP con educación individual (RPEI) y otro realizó RP con educación individual más grupal (RPEGI). Resultados: Ingresaron 48 pacientes, la disnea medida con la escala mMRC mostró mejoría estadísticamente significativa al inicio y al final del programa para ambos grupos, al igual que la distancia recorrida en el TC6M, con una mejoría en metros de 85,9 ± 12 en el grupo RPEGI y de 47,2 ± 19 en el grupo RPEI. Respecto a la ansiedad y depresión, se presentaron disminuciones significativas en la puntuación del HADS solo para el grupo RPEGI (p = 0,0120 y p = 0,001 respectivamente). En el cuestionario SGRQ todos los dominios presentaron cambios significativos para ambos grupos, excepto para el dominio actividad, que solo tuvo mejoría para el grupo RPEGI (p = 0,000). Finalmente, en el cuestionario LINQ los dos grupos mostraron cambios significativos. Conclusión: Todo programa de RP debe desarrollar un componente de educación. En este estudio, los pacientes con EPOC que recibieron educación individual más grupal presentaron mejores resultados al final de la intervención, especialmente en la ansiedad, depresión y el dominio actividad de la CVRS.

Introduction: Chronic Obstructive Pulmonary Disease (COPD) causes physical, emotional and social limitation and deterioration in health-related quality of life (HRQL) in those who suffer from it. The educational component is essential in any Pulmonary Rehabilitation (RP) program, since it allows maintaining long-term intervention results. The objective of this study was to compare the effect of two educational strategies for COPD patients developed in two PR programs in Cali-Colombia. Methods: Longitudinal prospective study. Patients with COPD who agreed to sign the informed consent were selected, if convenient, during a period of 24 months. The patients belonged to two PR programs, one that carried out PR with individual education (PRIE) and another performed PR with individual education plus group (PRIG). Results: A total of 48 patients were admitted, dyspnea measured with the mMRC scale showed a statistically significant improvement at the beginning and at the end of the program for both groups, as well as the distance traveled in the 6MWT, with an improvement in meters of 85.9±12 in the PRIG group and 47.2±19 in the PRIE group. Regarding anxiety and depression, there were significant decreases in the HADS score only for the PRIG group (p=0.0120 and p=0.001, respectively). In the SGRQ questionnaire, all the domains showed significant changes for both groups, except for the activity domain, which only showed improvement for the PRIG group (p=0.000). Finally, in the LINQ questionnaire, both groups showed significant changes. Conclusion: Every PR program must develop an education component. In this study, COPD patients who received individual plus group education presented better results at the end of the intervention, especially in anxiety, depression and in the HRQoL activity domain.

Lesión pulmonar aguda producida por transfusión (TRALI): Reporte de caso

Introducción: La lesión pulmonar aguda (TRALI) y la sobrecarga circulatoria (TACO) son las principales causas de morbilidad y mortalidad relacionadas con la transfusión. La TRALI se presenta durante o después de las transfusiones de plasma y sus derivados, o por inmunoglobulinas en alta concentración intravenosa; se asocia a procesos sépticos, cirugías y transfusiones masivas. La TACO es la exacerbación de manifestaciones respiratorias en las primeras 6 horas postransfusión. Reporte caso: Paciente de sexo masculino de 38 días de vida, ingresó al servicio de urgencias con un cuadro clínico de 8 días de evolución, caracterizado por dificultad respiratoria dado por retracciones subcostales y aleteo nasal sin otro síntoma asociado, con antecedentes de importancia de prematuridad y bajo peso al nacer. El reporte de hemograma arrojó cifras compatibles con anemia severa, por lo que requirió transfusión de glóbulos rojos empaquetados desleucocitados. El paciente presentó un cuadro respiratorio alterado en un periodo menor a 6 horas, por lo que se descartaron causas infecciosas y finalmente se consideró cuadro compatible con TRALI. Conclusiones: Se debe considerar una lesión pulmonar aguda relacionada con una transfusión de sangre si se produce una insuficiencia respiratoria aguda durante o inmediatamente después de la infusión de hemoderivados que contienen plasma.

Introduction: Acute lung injury (TRALI) and circulatory overload (TACO) are the main causes of transfusion-related morbidity and mortality. TRALI occurs during or after transfusions of plasma or its derivatives, or by immunoglobulins in high intravenous concentration; it is associated with septic processes, surgeries, and massive transfusions. TACO is the exacerbation of respiratory manifestations in the first 6 hours post transfusion. Case report: A 38-day-old male was admitted to the emergency department with clinical symptoms experienced over the course of 8 days and characterized by respiratory distress due to subcostal retractions and nasal flaring with no other associated symptoms. Important antecedents included prematurity and low birth weight. The hemogram report showed figures compatible with anemia, which benefited from transfusion of packed red blood cells without leukocytes. In a period of less than 6 hours, the patient presented altered respiratory symptoms, practitioners ruled out infectious causes and finally considered clinical signs compatible with TRALI. Conclusion: Acute lung injury related to blood transfusion should be considered if acute respiratory failure occurs during or immediately after infusion of plasma-containing blood products.

Hernia pulmonar espontánea: reporte de caso

Introducción: La hernia pulmonar espontánea es una entidad muy poco frecuente. Corresponde a la protrusión del parénquima pulmonar y pleura a través de un defecto de la pared torácica. Está causado por el aumento de la presión intratorácica asociado a la debilidad de la pared. Caso Clínico: Presentamos el caso de un paciente asmático con diagnóstico de hernia pulmonar espontánea de la pared torácica y su resolución quirúrgica. El tratamiento varía según el caso y no existe consenso respecto al manejo conservador vs quirúrgico ni respecto a la técnica quirúrgica a utilizar.

Introduction: Spontaneous pulmonary hernia is a very rare entity. It corresponds to the protrusion of the lung parenchyma and pleura through a chest wall defect. It is caused by increased intrathoracic pressure associated with wall weakness. Case Report: We present the case of an asthmatic patient diagnosed with spontaneous pulmonary hernia of the chest wall and its surgical resolution. Treatment varies according to the case and there is no consensus regarding conservative vs. surgical management or the surgical technique to be used.

Hipertensión arterial pulmonar en pacientes con dermatomiositis juvenil y esclerosis sistémica en un centro pediátrico de tercer nivel

INTRODUCCIÓN: La hipertensión arterial pulmonar puede estar asociada secundariamente a enfermedades del tejido conectivo. Entre estas enfermedades, predominan la esclerosis sistémica y la dermatomiositis juvenil. MATERIALES Y MÉTODOS: Se realizó un estudio retrospectivo, descriptivo y transversal. Se incluyeron todos los pacientes con diagnóstico de dermatomiositis juvenil y esclerosis sistémica que acudieron a nuestro hospital. Posteriormente se verificaron los niveles de presión arterial pulmonar mediante ecocardiografía. RESULTADOS: Se incluyeron 58 pacientes, de los cuales sólo 17 pacientes tuvieron ecocardiografía diagnóstica. Entre ellos, dos pacientes presentaron hipertensión arterial pulmonar. CONCLUSIÓN: La detección oportuna de la hipertensión arterial pulmonar en las enfermedades del tejido conectivo es esencial. Generalmente es asintomático. Es necesario adherirse al protocolo internacional que sugiere realizar ecocardiografía en todos los pacientes con dermatomiositis juvenil y esclerosis sistémica.

INTRODUCTION: Pulmonary arterial hypertension may be secondary associated with connective tissue diseases. Among these diseases, systemic sclerosis and juvenile dermatomyositis predominate. MATERIALS AND METHODS: A retrospective, descriptive and cross-sectional study was carried out. All patients with a diagnosis of juvenile dermatomyositis and systemic sclerosis who attended our hospital were included. Pulmonary arterial pressure levels were subsequently verified by echocardiography. RESULTS: 58 patients were included, of which only 17 patients had a diagnostic echocardiography. Among them, two patients presented pulmonary arterial hypertension. CONCLUSION: Timely detection of pulmonary arterial hypertension in connective tissue diseases is essential. It is generally asymptomatic. It is necessary to adhere to the international protocol that suggests performing echocardiography in all patients with juvenile dermatomyositis and systemic sclerosis.