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Neurodegenerative disorders such as Alzheimer's disease and macular degeneration represent major sources of human suffering, yet the factors influencing disease severity remain poorly understood. Sex has been implicated as one potential modifying factor. Here, we show that female sex is a risk factor for worsened outcomes in a model of retinal degeneration. Further, we show that this susceptibility is caused by the presence of female-specific circulating sex hormones. The adverse effect of female sex hormones was specific to diseased retinal neurons, and depletion of these hormones ameliorated this phenotypic effect. These findings provide novel insights into the pathogenesis of neurogenerative diseases and how sex hormones can impact the severity of disease. These findings have far-reaching implications for clinical trial design and the use of hormonal therapy in females with certain neurogenerative disorders.
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The aim of the research is to examine the relationships between the following variables (a) Theory of Planned Behavior (TPB), composed of Attitudes (ATT), Subjective Norms (SN), and Perceived Behavioral Control (PBC); and (b) Consciousness (EC) on the dependent variable Environmentally Responsible Purchase Intention (ERPI) from the perspective of the Latin American consumer in a pandemic. Currently, the literature on the relationships proposed in the explanatory model is still scarce at a theoretical and practical level, without empirical evidence in Latin America. The data collection is from 1624 voluntary responses from consumers in Chile (n = 400), Colombia (n = 421), Mexico (n = 401), and Peru (n = 402) collected through online surveys. Using structural equation modeling (SEM) and multi-group to test invariance analysis and the moderating effects, we can determine the relationship between the variables in the proposed model, generating evidence from Latin American countries. The empirical analysis verified that Attitude (ATT), Perceived Behavioral Control (PBC), and Environmental Consciousness (CE) have a positive and significant effect on Environmentally Responsible Purchase Intention (ERPI). The results also show that the generation variable presents invariance. Therefore, the groups are not different at the model level for the generation variable, becoming relevant to the difference at the path level. Therefore, the results of this study become a relevant contribution, indicating a moderating effect on the generation variable. This research provides insights for understanding Latin American consumers, and managerial implications are also provided for developing strategies to promote sustainable consumption.
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COVID-19 , Mudança Social , Humanos , América Latina , Teoria Psicológica , Atitude , Intenção , Inquéritos e Questionários , Comportamento do ConsumidorRESUMO
Las encefalitis autoinmunes son una condición emergente, caracterizada por la aparición repentina de síntomas psicóticos o depresivos "de novo", crisis convulsivas o estatus epiléptico refractario, o demencia rápidamente progresiva. Las encefalitis autoinmunes están asociadas a diversos fenómenos desencadenantes, como infecciones virales previas entre las más comunes, y se asocian con la presencia de anticuerpos antineuronales y/o onconeuronales, que deben estudiarse ante la sospecha de esta entidad. Es muy importante desarrollar un diagnóstico presuntivo y precoz, ya que la terapia con inmunosupresores como los corticoides -iniciados en el momento oportuno-, puede cambiar su evolución hacia la mejoría clínica. Presentamos un paciente con encefalitis autoinmunes y anticuerpos anti-Titina positivos (habitualmente presentes en timoma y miastenia gravis), no asociados a neoplasia conocida y con buena respuesta a esteroides.
Autoimmune Encephalitis, are an emerging condition, characterized by the sudden onset of psychotic or depressive symptoms "de novo", refractory seizures or epilepsy, or rapidly progressive dementias. The autoimmune encephalitis are associated to various triggered phenomena as a previous viral infections among others; it's related to the presence of antineuronal and/or onconeuronal antibodies, and there must be studied when autoimmune encephalitis is suspected. It is very important to develop a presumptive and early diagnosis, since steroid therapy -on opportunity time- can change its evolution towards clinical improvement. We present a patient with autoimmune encephalitis, and positive anti-Titin antibodies (usually presents in thymoma and myasthenia gravis) not associated with known neoplasia, and with a good response to steroids.
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El Síndrome de Ramsay-Hunt (SRH), es la segunda causa de parálisis facial periférica (PFP). Causado por el virus Varicella zoster (VVZ), ipsilateral a la PFP,presenta unaerupción herpetiforme y cefalea en distribución del nervio facial. Presentamos el caso de una mujer, 54 años, con SRH y cefalea persistente cuyo líquido cerebroespinal (LCE) fue compatible con meningitis. Se indicó aciclovir endovenoso (EV). La literatura no recomienda estudio de LCE en PFP; y en SRH se sugiere en inmunosuprimidos y complicaciones del SRH como queratopatía, accidentes-cerebrovasculares, y meningitis. Un LCE alterado en SRH, indica modificar la conducta terapéutica.
Ramsay-Hunt Syndrome (RHS) is the second leading cause of peripheral facial palsy (PFP). Caused by the Varicella zoster virus (VZV), ipsilateral to the PFP, it presents a herpetiform rash and headache in the facial nerve distribution. We present the case of a 54-year-old woman with RHS and persistent headache whose cerebrospinal fluid (CSF) was compatible with meningitis. Intravenous acyclovir was indicated. The literature does not recommend an CSF study in PFP; in RHS it is suggested in immunosuppressed patients and complications of RHS such as keratopathy, cerebrovascular accidents, and meningitis. An altered CSF in RHS indicates modifying the therapeutic conduct.
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BACKGROUND: Thymic tumors are unusual neoplasms, representing 0.2 to 1.5% of tumors in humans, but correspond to 20% of mediastinal tumors and 50% of those that occur in the anterior mediastinum. They tend to appear around the fourth and fifth decades of life without gender predilection. Up to 30% of patients are asymptomatic, therefore many are incidentally diagnosed. Radical thymectomy is the treatment of choice with high survival rates when detected in the early stages. METHODS: This was a retrospective descriptive study, including 18 adult patients' diagnosis of thymic neoplasm, who were managed with surgical resection from 2011 to 2019. Information about demographics, clinical characteristics, imaging findings, surgical and medical management, plus histological findings was obtained and reported. RESULTS: 18 patients with thymic tumors were included, of which specific histologic studies reveled thymomas, carcinomas, neuroendocrine tumors, thymolipoma and thymic cyst. Mean age was 52.7 years, with a predominance of male population. The main symptom was dyspnea, followed by cough and chest pain. Paraneoplastic syndromes such as myasthenia gravis, aplastic anemia and Cushing syndrome were reported. 89% of cases were treated by radical thymectomy alone, while only 2 cases required chemotherapy and radiotherapy. There were no surgical complications. Mean hospital stay length was 11. 9 days, with only 1 mortality during hospital admission. 5-year survival rate was 81%. CONCLUSIONS: The treatment of choice is radical thymectomy, which has been shown to positively impact patient mortality. Early detection is key to improve patient outcomes.
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Síndromes Paraneoplásicas/epidemiologia , Timectomia , Timo/patologia , Neoplasias do Timo/cirurgia , Idoso , Carcinoma/complicações , Carcinoma/diagnóstico , Carcinoma/mortalidade , Carcinoma/cirurgia , Colômbia/epidemiologia , Feminino , Humanos , Tempo de Internação/estatística & dados numéricos , Lipoma/complicações , Lipoma/diagnóstico , Lipoma/mortalidade , Lipoma/cirurgia , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/cirurgia , Síndromes Paraneoplásicas/etiologia , Estudos Retrospectivos , Taxa de Sobrevida , Timoma/complicações , Timoma/diagnóstico , Timoma/mortalidade , Timoma/cirurgia , Timo/diagnóstico por imagem , Timo/cirurgia , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/mortalidadeRESUMO
BACKGROUND: The sternum is considered an unusual tumor site, corresponding to 15% of all thoracic wall tumors. Primary sternal tumors are even rarer and most commonly malignant. We present the case of a young man who consulted with a painful sternal mass, which after its resection is confirmed to be a cavernous hemangioma. CASE PRESENTATION: A 39-year-old man, with unremarkable medical history besides a 2-year-long sternal pain, non-irradiated, which worsens over the last few months and is accompanied by the appearance of a sternal palpable mass. On physical exam, there was a bulging of the sternal manubrium, with no inflammatory changes. Thoracic CT scan shows an expansive and lytic lesion of the sternum, compromising the manubrium and extending to the third sternocostal joint, without intrathoracic compromise nor cleavage plane with mediastinal vascular structures. The patient is taken to resection of the mass and sternal reconstruction using prosthetic material and pectoral and fasciocutaneous muscular flaps. Histopathological findings: cavernous hemangioma with negative borders and no other malignant findings. CONCLUSIONS: Sternal hemangiomas can cause defects in the bone structure and show an expansive growth, challenging the differentiation between a benign or malignant lesion. Therefore, they should be considered malignant until shown otherwise. Management involves radical surgery with curative purposes and posterior reconstruction to improve quality of life, as shown with our patient.
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Hemangioma Cavernoso/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Esterno/cirurgia , Parede Torácica/cirurgia , Adulto , Hemangioma Cavernoso/diagnóstico por imagem , Humanos , Masculino , Qualidade de Vida , Esterno/diagnóstico por imagem , Parede Torácica/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal neoplasms that originate from mesenchymal growth in the pleura, tend to be single tumors, usually have an indolent course and show nonspecific symptoms. SFTP can be often diagnosed from an incidental finding of a single mass in the thorax and should be confirmed by biopsy and immunohistochemistry. A minority of cases may present Doege-Potter syndrome (DPS, episodes of refractory hypoglycemia) associated with production of insulin-like growth factor 2 (IGF-2). Both SFTP and DPS are rare occurrences with less than 2000 cases reported worldwide. The curative treatment is tumor resection. Two cases of patients with DPS caused by SFTP are presented below.
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BACKGROUND: Myospherulosis develops as the result of a reaction between exogenous lipids and red blood cells (RBC) of the patient, being the latter injured when perceived as a foreign body by the immune system, triggering an intense inflammatory response. CASE PRESENTATION: A 63-year-old man with a history of dyslipidemia, Barret's esophagus, and coronary disease, who was taken to thoracoscopy and left inferior lobectomy due to the presence of a pulmonary mass. A primary pulmonary adenocarcinoma was diagnosed. On follow up a PET-CT was performed, in which a metabolically active lesion was described adjacent to the prior lobectomy, suggesting a local relapse. EBUS-TBNA was then performed, obtaining a sample from which histopathological pattern compatible with myospherulosis was obtained. CONCLUSIONS: Although it is a rare entity, myospherulosis has a well-defined morphological pattern. By not recognizing myospherulosis as a benign lesion, a patient's risk of invasive cancer can be overestimated. It is a phenomenon found with increasing frequency and has been reported in different tissues, however, this is the first report in the literature of myospherulosis of the lung. Greater awareness is required regarding the existence of this phenomenon.
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Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico , Eritrócitos , Granuloma/patologia , Lipídeos , Pneumopatias/patologia , Brônquios , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Lipomas are the most common benign soft tissue tumors in the general population. These lesions can appear on any part of the body and usually develop in the subcutaneous superficial tissue. Lipomas that show ossifying changes are very rare, representing less than 1% of the reported lipomas. They usually manifest as hard nodular lesions in the head and neck, the extremities, the sternoclavicular region, and the subcutaneous tissue in general; they are rare in the costal arches. CASE PRESENTATION: We report the case of a patient with a history of multiple diseases and 2 tumor-like lesions with internal lytic areas detected in the fourth right costal arch and in the eighth left costal arc; we describe his clinical manifestations, radiological and laboratory findings as well as the pathological results and outcome. CONCLUSIONS: Ossifying lipomas are rare benign tumors with asymptomatic clinical presentation. It is important to perform an adequate radiological differentiation from other more aggressive lesions such as liposarcomas.
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INTRODUCTION: Paragangliomas (PGGL) are rare neuroendocrine tumors arising from non-epithelial extra-adrenal chromaffin cells. They have been described in different sites: abdomen, pelvis, head, neck and thorax. Incidence is very low, occurring in less than 2-8/million per year. PGGL's of the lung are extremely rare, they have a slow growth and present as painless lesions. Biopsy is the method of choice for diagnosis and prognosis. PRESENTATION OF CASE: This is a 70-year-old woman with chronic cough, with a CT-scan showing a 3.3-cm mass in the left lower lobe. After video-assisted thoracic surgery, histologic findings confirmed a non-functioning pulmonary paraganglioma. We present the clinical, radiological, pathological findings and clinical course. DISCUSSION: Primary pulmonary PGGL's are extremely rare neuroendocrine tumors with low-grade malignancy, difficult to distinguish from other pulmonary tumors relying only on imaging techniques. In this case, PGGL presented as an incidentaloma during the evaluation of chronic cough. After histological diagnosis, genetic testing are ideally performed to identify somatic or germline mutations that may condition a higher risk of malignancy and metastasis. CONCLUSION: PGGL's must be considered when other diagnoses are unlikely due to immunohistochemistry findings. Larger studies in this field are needed to determine the risk factors for its development and to determine which populations have the greatest potential for malignant transformation.
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BACKGROUND: The term goiter is used to describe any abnormal growth of the thyroid gland, which can be diffuse or nodular, and can be associated with normal, diminished, or increased thyroid function. Multinodular goiter is a common disease whose prevalence increases at age 50. Clinical manifestations can be due to thyroid function impairment or related to size and location of the gland with compressive symptoms. Intrathoracic location is less frequent, can be mistaken with pulmonary lesions and usually implies a difficult surgical approach. CASE PRESENTATION: A 66-year-old woman with a history of subtotal thyroidectomy presented with 7-month dyspnea, dry cough. There was no evidence of neck masses, or jugular engorgement. Physical examination was normal. Chest x-ray showed an 11 cm mass in the upper right hemithorax. Computed tomography (CT)-scan, showed calcifications, and compression of the superior vena cava without infiltration, the right subclavian vein and left displacement of the trachea. Distinction between intrapulmonary or mediastinal location was not clear. Biopsy showed thyroid origin, and bilateral thoracotomy was performed with confirmation of a giant multinodular goiter. CONCLUSIONS: Intrathoracic goiter should undergo surgical or ablative management if compressive symptoms of the airway and cervical or thoracic vessels are present. The large size of the tumor along with the presentation after thyroidectomy and the seeming location in the right upper lobe made this particular case striking. Specially in the elderly, multidisciplinary perioperative management is key for a successful recovery.
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Pulmonary complications are prevalent among patients with hematologic malignancies, who are at high risk of developing acute respiratory distress syndrome (ARDS). Although diffuse alveolar damage is considered the diagnostic hallmark of ARDS, there are plenty of other non-diffuse alveolar damage etiologies that can mimic ARDS and benefit from a specific therapy, therefore correcting the underlying cause. When the etiology remains unclarified despite noninvasive procedures, a surgical lung biopsy (either open via thoracotomy or video-assisted thoracoscopic surgery [VATS]) may be warranted. However, the role of surgical lung biopsy has not been extensively studied in patients with hematologic malignancy and ARDS and so doubt exists about the risk-benefit relationship of such procedures. In this article, we report a series of 8 critically ill patients with hematologic malignancies and ARDS, who underwent VATS lung biopsy, in a specialized institution in Cali, Colombia, from 2015 to 2019, with special emphasis on its diagnostic yield, modifications in treatment protocol, and safety. VATS lung biopsy is a minimally invasive procedure that appears to be a relatively safe with few postoperative complications and minimal perioperative mortality. It has a high diagnostic yield, resulting in a modification of treatment in a nondepreciable percentage of patients. However, this subset of patients was critically ill, with a high risk of mortality, and the lung biopsy did not appear to affect in this aspect. Future randomized controlled trials are needed to further clarify this topic.
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Neoplasias Hematológicas/diagnóstico , Pulmão/patologia , Síndrome do Desconforto Respiratório/diagnóstico , Cirurgia Torácica Vídeoassistida/efeitos adversos , Adolescente , Adulto , Idoso , Biópsia/métodos , Estado Terminal , Feminino , Neoplasias Hematológicas/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Síndrome do Desconforto Respiratório/patologia , Estudos Retrospectivos , Toracotomia/métodos , Adulto JovemRESUMO
Introducción. La miastenia grave es una enfermedad autoinmunitaria con una prevalencia mundial de 150 a 250 casos por 1 Ì000.000 de habitantes. El tratamiento recomendado para la miastenia grave sin timoma es la timectomía total, la cual es la única alternativa de curación. Métodos. Se llevó a cabo un estudio descriptivo y retrospectivo de una serie de casos de pacientes adultos con miastenia grave sin timoma sometidos a timectomía, durante el periodo de 2010 a 2017. En el análisis estadístico descriptivo, se utilizaron frecuencias absolutas y porcentajes para las variables cualitativas y, para las variables cuantitativas, se utilizaron la mediana y el rango intercuartílico. Resultados. Veintiocho pacientes con miastenia grave sin timoma se sometieron a timectomía desde el año 2010 hasta el 2017. Se categorizaron según la clasificación del estado posterior a la intervención de la Myasthenia Gravis Foundation of America y se evidenció que 4 (14,3 %) pacientes presentaban remisión completa y el grado 3 de manifestaciones clínicas mínimas fue el más frecuente en 19 (67,9 %); 26 (92,9 %) tuvieron mejoría con respecto al cambio del estado clínico, en 2 (7,1 %) no se documentaron cambios y en ningún paciente hubo empeoramiento, exacerbación o muerte secundaria a la enfermedad. Conclusiones. A lo largo de siete años se practicó timectomía a 28 pacientes con diagnóstico de miastenia grave sin timoma, aproximadamente, en el 15 % de los pacientes hubo remisión completa, el grado 3 de manifestaciones mínimas fue el más frecuente y el 93 % presentó mejoría de su estatus clínico.
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Humanos , Miastenia Gravis , Cirurgia Torácica , Timectomia , Resultado do TratamentoRESUMO
Objective: Cardiac sympathetic denervation (CSD) using video-assisted thoracoscopy is a therapeutic alternative for cardiac arrhythmias refractory to conventional treatment in patients with ventricular structural heart disease, mainly due to ischemia, and in patients with hereditary conditions associated with sudden death such as long QT syndrome. In general, it is performed in cases with recurrent episodes of ventricular tachycardia or electrical storm, in spite of conventional treatment. The objective of this study is to show the experience of this institution with DSCI in refractory patients to conventional management and the results derived from its application. Methods: This was an observational retrospective study. The records of patients with a history of ventricular arrhythmias treated in our center with pharmacological treatment, catheter ablation, or implantation of an implantable cardioverter-defibrillator (ICD), who underwent video-assisted CSD were analyzed and described. Results: A total of six patients were included in the study. Patients with structural heart disease were the most frequent, median age was 56 ± 16 years; 67% were male. The procedure evolved without complications in any of the patients and an overall significant improvement was observed. A 24-month follow-up was conducted; two patients had recurrence episodes presenting as slow ventricular tachycardia without severe symptoms and a third patient presented an episode of ventricular fibrillation aborted by the ICD. Conclusion: Video-assisted CSD should be considered as a treatment option for patients with potentially dangerous arrhythmias that do not respond to conventional treatment, especially in recurrent ventricular tachycardia.
Objetivo: La denervación simpática cardiaca izquierda (DSCI) por toracoscopia se ha convertido en una alternativa terapéutica para el manejo de arritmias cardíacas refractarias al tratamiento convencional en pacientes con cardiopatía estructural, principalmente isquémicos, y enfermedades hereditarias asociadas con muerte súbita como el síndrome de QT largo. Generalmente se realiza en quienes manifiestan episodios recurrentes de arritmias ventriculares o incluso tormenta eléctrica a pesar del tratamiento convencional. El objetivo de este estudio es mostrar la experiencia de esta institución con la DSCI en pacientes refractarios al manejo convencional y los resultados derivados de su aplicación. Métodos: Se revisaron los registros de 6 pacientes con antecedente de arritmias ventriculares tratados previamente con medicamentos y en algunos casos con ablación con catéter y la mayoría con desfibrilador automático implantable, que fueron llevados DSCI por toracoscopia video-asistida (VATS). Resultados: La principal enfermedad de base fue la cardiopatía estructural, la indicación más prevalente fue tormenta arrítmica incontrolable, la edad promedio fue de 56 ± 16 años, el 67% de los individuos fueron hombres. Este procedimiento no mostró complicaciones en ninguno de los pacientes y se encontró mejoría sintomática en todos los casos. Se realizó seguimiento por 24 meses; dos pacientes tuvieron recurrencias por taquicardia ventricular lenta sin síntomas severos y uno por fibrilación ventricular. Conclusion: La DSCI por VATS debe considerarse como opción terapéutica para pacientes con arritmias de difícil manejo.
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Introduction: Human and porcine anatomy are comparable. In consequence, the porcine biomodel has the potential to be implemented in the training of surgical professionals in areas such as solid organ transplantation. Objectives: We described the procedures and findings obtained in the experiments of translational respiratory medicine with the porcine biomodel, within an experimentation animal laboratory, and we present a comparative review between human and porcine lung. Materials and methods: The experiment was done in nine pigs of hybrid race within a laboratory of experimental surgery. The anatomy and histology of the respiratory tract were studied with fibrobronchoscopy, bronchial biopsy and bronchoalveolar lavage. The bronchoalveolar lavage was studied with liquid-based cytology and assessed with Papanicolau and hematoxylin-eosin staining. Molecular pathology techniques such as immunohistochemistry, flow cytometry, and electronic microscopy were implemented. The pigs were subjected to left pneumonectomy with posterior implantation of the graft into another experimental pig. Results: Histopathologic and molecular studies evidenced predominance of alveolar macrophages (98%) and T-lymphocytes (2%) in the porcine bronchoalveolar lavage. Studies on the porcine lung parenchyma revealed hyperplasic lymphoid tissue associated with the bronchial walls. Electronic microscopy evidenced the presence of T-lymphocytes within the epithelium and the cilia diameter was similar to the human. Conclusions: The porcine biomodel is a viable tool in translational research applied to the understanding of the respiratory system anatomy and the training in lung transplantation. The implementation of this experimental model has the potential to strength the groups who plan to implement an institutional program of lung transplantation in humans.
Introducción. La anatomía humana y porcina son comparables. En consecuencia, el biomodelo porcino tiene el potencial de ser implementado para entrenar al profesional quirúrgico en áreas como el trasplante de órganos sólidos. Objetivo. Describir los procedimientos y hallazgos obtenidos mediante experimentos de medicina respiratoria traslacional con biomodelos porcinos realizados en un laboratorio de experimentación animal, y hacer una revisión comparativa entre el pulmón humano y el porcino. Materiales y métodos. El experimento se llevó a cabo en nueve cerdos de raza híbrida en un laboratorio de cirugía experimental. Se estudiaron la anatomía y la histología de las vías respiratorias mediante fibrobroncoscopia, biopsia bronquial y lavado broncoalveolar. El lavado broncoalveolar se estudió con citología en base líquida y se evaluó con las coloraciones de Papanicolau y hematoxilina y eosina. Se utilizaron técnicas de patología molecular, como inmunohistoquímica, citometría de flujo y microscopía electrónica. Los cerdos se sometieron a neumonectomía izquierda con posterior implante del injerto en otro cerdo experimental. Resultados. Los estudios histopatológicos y moleculares evidenciaron un predominio de macrófagos alveolares (98 %) y linfocitos T (2 %) en el lavado broncoalveolar porcino. En los estudios del parénquima pulmonar porcino se encontró tejido linfoide hiperplásico asociado a las paredes bronquiales. La microscopía electrónica evidenció linfocitos T dentro del epitelio y el diámetro de las cilias porcinas fue similar al de las humanas. Conclusiones. El biomodelo porcino es viable en la investigación traslacional para el entendimiento de la anatomía del sistema respiratorio y el entrenamiento en trasplante pulmonar. La implementación de este modelo experimental podría fortalecer los grupos que planean implementar un programa institucional de trasplante pulmonar en humanos.
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Transplante de Pulmão , Modelos Animais , Suínos , Pesquisa Translacional Biomédica/métodos , Animais , Biópsia , Medula Óssea/ultraestrutura , Líquido da Lavagem Broncoalveolar/citologia , Broncoscopia , Humanos , Pulmão/irrigação sanguínea , Pulmão/ultraestrutura , Transplante de Pulmão/métodos , Pneumonectomia/métodos , Especificidade da Espécie , Coleta de Tecidos e Órgãos/métodosRESUMO
BACKGROUND: Primary effusion lymphoma is a rare, high-grade non-Hodgkin's lymphoma that usually occurs in immunosuppressed or human immunodeficiency virus-positive individuals in advanced stages of the disease. However, primary effusion lymphoma occasionally affects immunocompetent patients who are infected with human herpes virus type 8 or Epstein-Barr virus. This disease manifests with liquid collections in cavities, producing constitutional symptoms; fever; weight loss; and symptoms related to extrinsic compression, such as dyspnea or abdominal discomfort. Diagnosis is confirmed with cytological or tissue evaluation showing large, multinucleated lymphoid cells with positive specific markers for the disease, such as CD45 and markers related to viral infections, when present. There is no standard treatment for primary effusion lymphoma, but several chemotherapy protocols are recommended, usually with poor results. CASE PRESENTATION: We present a case of an adult human immunodeficiency virus-negative Hispanic origin woman with primary effusion lymphoma with pleuritic, pericardial, and peritoneal compromise who also had unusual complications during a diagnostic procedure: the accidental rupture of the left ventricle and the development of a secondary left ventricular pseudoaneurysm. We describe the clinical, radiological, and laboratory characteristics as well as the outcome of this case. CONCLUSIONS: Primary effusion lymphoma is a very rare entity that represents 4% of non-Hodgkin's lymphoma cases associated with human immunodeficiency virus and 0.1% to 1% of all lymphomas in patients with another type of immunodeficiency in regions where human herpes virus type 8 is not endemic. This reported case is an unusual presentation of primary effusion lymphoma because it occurred in an immunocompetent human immunodeficiency virus-negative adult woman without the presence of Kaposi's sarcoma or Castleman's disease and for whom the clinical course after chemotherapy was successful. However, the rupture of the free wall of the left ventricle is a very rare catastrophic event that usually occurs after myocardial infarction. Left ventricle free wall rupture rarely goes unnoticed, but when it occurs, it leads to the development of a ventricular pseudoaneurysm in which the rupture is contained by the pericardium with an organized thrombus and an adjacent hematoma.
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Traumatismos Cardíacos/etiologia , Ventrículos do Coração/lesões , Doença Iatrogênica , Linfoma de Efusão Primária/diagnóstico , Toracoscopia/efeitos adversos , Falso Aneurisma/diagnóstico por imagem , Falso Aneurisma/etiologia , Feminino , Humanos , Imunocompetência , Pessoa de Meia-Idade , Derrame Pericárdico/diagnóstico por imagem , Derrame Pleural/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Abstract Objective: Cardiac sympathetic denervation (CSD) using video-assisted thoracoscopy is a therapeutic alternative for cardiac arrhythmias refractory to conventional treatment in patients with ventricular structural heart disease, mainly due to ischemia, and in patients with hereditary conditions associated with sudden death such as long QT syndrome. In general, it is performed in cases with recurrent episodes of ventricular tachycardia or electrical storm, in spite of conventional treatment. The objective of this study is to show the experience of this institution with DSCI in refractory patients to conventional management and the results derived from its application. Methods: This was an observational retrospective study. The records of patients with a history of ventricular arrhythmias treated in our center with pharmacological treatment, catheter ablation, or implantation of an implantable cardioverter-defibrillator (ICD), who underwent video-assisted CSD were analyzed and described. Results: A total of six patients were included in the study. Patients with structural heart disease were the most frequent, median age was 56 � 16 years; 67% were male. The procedure evolved without complications in any of the patients and an overall significant improvement was observed. A 24-month follow-up was conducted; two patients had recurrence episodes presenting as slow ventricular tachycardia without severe symptoms and a third patient presented an episode of ventricular fibrillation aborted by the ICD. Conclusion: Video-assisted CSD should be considered as a treatment option for patients with potentially dangerous arrhythmias that do not respond to conventional treatment, especially in recurrent ventricular tachycardia.
Resumen Objetivo: La denervación simpática cardiaca izquierda (DSCI) por toracoscopia se ha convertido en una alternativa terapéutica para el manejo de arritmias cardíacas refractarias al tratamiento convencional en pacientes con cardiopatía estructural, principalmente isquémicos, y enfermedades hereditarias asociadas con muerte súbita como el síndrome de QT largo. Generalmente se realiza en quienes manifiestan episodios recurrentes de arritmias ventriculares o incluso tormenta eléctrica a pesar del tratamiento convencional. El objetivo de este estudio es mostrar la experiencia de esta institución con la DSCI en pacientes refractarios al manejo convencional y los resultados derivados de su aplicación. Métodos: Se revisaron los registros de 6 pacientes con antecedente de arritmias ventriculares tratados previamente con medicamentos y en algunos casos con ablación con catéter y la mayoría con desfibrilador automático implantable, que fueron llevados DSCI por toracoscopia video-asistida (VATS). Resultados: La principal enfermedad de base fue la cardiopatía estructural, la indicación más prevalente fue tormenta arrítmica incontrolable, la edad promedio fue de 56 +- 16 años, el 67% de los individuos fueron hombres. Este procedimiento no mostró complicaciones en ninguno de los pacientes y se encontró mejoría sintomática en todos los casos. Se realizó seguimiento por 24 meses; dos pacientes tuvieron recurrencias por taquicardia ventricular lenta sin síntomas severos y uno por fibrilación ventricular. Conclusion: La DSCI por VATS debe considerarse como opción terapéutica para pacientes con arritmias de difícil manejo.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Arritmias Cardíacas/cirurgia , Simpatectomia/métodos , Cirurgia Torácica Vídeoassistida/métodos , Arritmias Cardíacas/fisiopatologia , Recidiva , Estudos Retrospectivos , Seguimentos , Resultado do TratamentoRESUMO
Adenoid cystic carcinoma is a rare tumor of low frequency, low grade malignancy and slow growth originating from the major and minor salivary glands. Its primary pulmonary presentation is very rare, it can manifest with central airway obstruction and extensive lung involvement. Although it is slow growing, it has a tendency to local recurrence and late hematogenous metastases. The cornerstone of management consists in the complete resection of the tumor. Radiotherapy and adjuvant chemotherapy have been used in a limited number of patients with unsatisfactory results. Therefore, clinical judgment is of vital importance for early diagnosis and adequate selection of patients for surgical management. We present two cases of patients with primary pulmonary adenoid cystic tumor with invasion of the carina treated with sleeve pneumonectomy and carinal resection with adequate response to the intervention.
RESUMO
Objetivo: La denervación simpática cardiaca izquierda (DSCI) por toracoscopia se ha convertido en una alternativa terapéutica para el manejo de arritmias cardíacas refractarias al tratamiento convencional en pacientes con cardiopatía estructural, principalmente isquémicos, y enfermedades hereditarias asociadas con muerte súbita como el síndrome de QT largo. Generalmente se realiza en quienes manifiestan episodios recurrentes de arritmias ventriculares o incluso tormenta eléctrica a pesar del tratamiento convencional. El objetivo de este estudio es mostrar la experiencia de esta institución con la DSCI en pacientes refractarios al manejo convencional y los resultados derivados de su aplicación. Métodos: Se revisaron los registros de 6 pacientes con antecedente de arritmias ventriculares tratados previamente con medicamentos y en algunos casos con ablación con catéter y la mayoría con desfibrilador automático implantable, que fueron llevados DSCI por toracoscopia video-asistida (VATS). Resultados: La principal enfermedad de base fue la cardiopatía estructural, la indicación más prevalente fue tormenta arrítmica incontrolable, la edad promedio fue de 56 ± 16 años, el 67% de los individuos fueron hombres. Este procedimiento no mostró complicaciones en ninguno de los pacientes y se encontró mejoría sintomática en todos los casos. Se realizó seguimiento por 24 meses; dos pacientes tuvieron recurrencias por taquicardia ventricular lenta sin síntomas severos y uno por fibrilación ventricular. Conclusion: La DSCI por VATS debe considerarse como opción terapéutica para pacientes con arritmias de difícil manejo.
Objective: Cardiac sympathetic denervation (CSD) using video-assisted thoracoscopy is a therapeutic alternative for cardiac arrhythmias refractory to conventional treatment in patients with ventricular structural heart disease, mainly due to ischemia, and in patients with hereditary conditions associated with sudden death such as long QT syndrome. In general, it is performed in cases with recurrent episodes of ventricular tachycardia or electrical storm, in spite of conventional treatment. The objective of this study is to show the experience of this institution with DSCI in refractory patients to conventional management and the results derived from its application. Methods: This was an observational retrospective study. The records of patients with a history of ventricular arrhythmias treated in our center with pharmacological treatment, catheter ablation, or implantation of an implantable cardioverter-defibrillator (ICD), who underwent video-assisted CSD were analyzed and described. Results: A total of six patients were included in the study. Patients with structural heart disease were the most frequent, median age was 56 ± 16 years; 67% were male. The procedure evolved without complications in any of the patients and an overall significant improvement was observed. A 24-month follow-up was conducted; two patients had recurrence episodes presenting as slow ventricular tachycardia without severe symptoms and a third patient presented an episode of ventricular fibrillation aborted by the ICD. Conclusion: Video-assisted CSD should be considered as a treatment option for patients with potentially dangerous arrhythmias that do not respond to conventional treatment, especially in recurrent ventricular tachycardia.
Assuntos
Arritmias Cardíacas/cirurgia , Simpatectomia/métodos , Cirurgia Torácica Vídeoassistida/métodos , Adulto , Idoso , Arritmias Cardíacas/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Resultado do TratamentoRESUMO
The risk of sudden unexpected death in patients with epilepsy (SUDEP), is 20 to 25 times greater than in the general population. This increased risk is seen specially in refractory epilepsy, with an incidence of 9:1,000 patients-years. Risk factors have been established based on retrospective studies, finding that the frequency of seizures, specially generalized tonic clonic seizures, is the most important one. The physiopathological mechanism of SUDEP is not yet fully understood. Autonomic system abnormalities, as well as cardiac and respiratory changes have been found. The finding of new molecular biomarkers to identify patients with increased risk should be a priority. Treatment is based in the management of risk factors, although clear recommendations are hard to establish given the low level of evidence.