ABSTRACT Lymphangiomas are rare and correspond to 0.7% to 4.0% of mediastinal tumors, and isolated mediastinal location occurs in 1% of cases. They are benign tumors that originate from a congenital malformation of the lymphatic vessels and are diagnosed more frequently in children less than 2 years of age. Chylous ascites is a clinical manifestation of thoracic duct lymphangioma and is composed of lymph accumulation caused by dilation of this lymphatic channel. It appears milky in the peritoneal cavity, containing triglyceride levels higher than 200 mg/dl. We report the case of a young patient with chylous ascites and lymphangioma of the thoracic duct, who was conservatively treated with octreotide and a low-fat diet with medium-chain triglycerides.

Abstract: Bronchogenic cysts are uncommon mediastinal masses that usually evolve unsuspected and are incidentally found. They have origin from the primitive tracheobronchial tube before 40 days of gestation. Their clinical features are often related to size, location, and complications. Progressive pain and regional compressive symptoms may occur. Surgical resection is the first option for definitive treatment, which propitiates the discard of differential hypotheses with several benign and malignant conditions to establish the correct definitive diagnosis. The purpose of the case study herein reported is to enhance the suspicion index of non-specialists about this scarcely described entity.

Resumen: Los quistes broncogénicos son masas mediastínicas poco comunes que generalmente evolucionan de manera insospechada y se encuentran incidentalmente. Tienen su origen en el tubo traqueobronquial primitivo antes de los 40 días de gestación. Sus características clínicas a menudo están relacionadas con el tamaño, la ubicación y las complicaciones. Puede haber dolor progresivo y síntomas compresivos regionales. La resección quirúrgica es la primera opción para el tratamiento definitivo, que propicia el descarte de hipótesis diferenciales con diversas afecciones benignas y malignas para establecer el diagnóstico definitivo correcto. El propósito del estudio de caso aquí presentado es mejorar el índice de sospecha de no especialistas sobre esta entidad pocas veces descrita.

Abstract: A previously healthy 18-year-old man was admitted with asthenia, fever, shivering, oppressive chest pain, and orthopnea of three-day duration. He had an infected finger wound and lymphangitis on his forearm, self-medicated with topical ointments unsuccessfully. He was febrile (39 oC), hypotensive, with low SpO2 and oliguria, without peripheral edema, hepatojugular reflux or pericardial friction rub. He suddenly had jugular distention, muffled heart sounds and paradoxical pulse, indicating cardiac tamponade, further confirmed. Erythrocyte sedimentation rate, neutrophil-lymphocyte count ratio, C-reactive protein, and procalcitonin) were elevated. Despite of intensive care he had irreversible cardiac arrest. Autopsy revealed hemopericardium causing death by cardiac tamponade and pulmonary edema, in addition to fibrinous pericarditis, hepatic abscess, and acute tubular necrosis. Methicillin-resistant Staphylococcus aureus (MRSA) was found in tissue and blood samples. Eventual tuberculosis coinfection and pericardial involvement by malignancy were ruled out. The role of autopsy to better understand mechanisms of cardiac tamponade is commented.

Resumen: Un hombre previamente sano de 18 años ingresó al hospital con astenia, fiebre, temblores, dolor torácico opresivo y ortopnea, con duración de tres días. Presentaba una herida infectada en un dedo de la mano y linfangitis en el antebrazo, automedicadas con ungüentos tópicos sin mejora. Estaba febril (39 oC), con hipotensión, baja SpO2 y oliguria, sin edema periférico, reflujo hepatoyugular o frote pericárdico. De súbito tuvo distensión yugular, sonidos amortiguados del corazón y pulso paradójico, indicando un taponamiento cardíaco, que fue confirmado. La velocidad de sedimentación de eritrocitos, la proporción de recuento de neutrófilos y linfocitos, la proteína C reactiva y la procalcitonina fueron elevados. A pesar de los cuidados intensivos tuvo paro cardíaco irreversible. La autopsia reveló hemopericardio causando muerte por taponamiento cardíaco y edema pulmonar, además de pericarditis fibrinosa, absceso hepático y necrosis tubular aguda. Staphylococcus aureus resistente a la meticilina (MRSA) se encontró en muestras de tejido y sangre. Eventual coinfección por tuberculosis o invasión pericárdica por malignidad fueron descartadas. Se comenta el papel de la autopsia para comprender mejor los mecanismos del taponamiento cardíaco.

Abstract A 76-year-old woman with antecedent of bronchiectasis and productive cough during four months was admitted because of worsening of cough six hours ago. She had been under prolonged broad-spectrum antibiotic therapy without improvement. During admission the chest images and laboratory evaluations established the diagnosis of lung aspergillosis. Chronic pulmonary aspergillosis manifests as cavity, fibrosis, aspergilloma or nodules. The diagnosis depends on typical pulmonary images and microscopy and culture evidence of the fungus or positivity of the specific serologic tests for Aspergillus. It is mandatory to rule out other hypothesis, in special tuberculosis. The role of galactomannan test is also commented.

Resumen Una mujer de 76 años de edad con antecedente de bronquiectasias y tos productiva durante cuatro meses fue admitida debido al empeoramiento de la tos hace seis horas. Había estado bajo terapia antibiótica de largo espectro prolongada sin mejoría. Durante el ingreso, las imágenes torácicas y las evaluaciones de laboratorio establecieron el diagnóstico de aspergilosis pulmonar. La aspergilosis pulmonar crónica se manifiesta como cavidad, fibrosis, aspergiloma o nódulos. El diagnóstico depende de imágenes pulmonares típicas y evidencia del hongo en la microscopia y cultivo, o la positividad de las pruebas serológicas específicas para Aspergillus. Es obligatorio descartar otras hipótesis, la tuberculosis en especial. También se comenta el papel de la prueba de galactomanano.

Abstract: Serous ovarian carcinoma is among the main causes of cancer-related death, and the levels of pretreatment CA 125 can be associated with survival in the high grade tumors. A postmenopausal woman with ovarian cancer and elevated levels of CA 125, presented with hydrothorax and massive ascites, which are features of pseudo-Meigs’ syndrome. Data of immunohistochemistry study were consistent with high grade serous carcinoma. Diagnostic challenges of the syndrome and the mechanisms of effusions are also commented.

Resumen: El carcinoma seroso del ovario está entre las causas principales de muerte relacionada con el cáncer, y los niveles de CA 125 del pretratamiento se pueden asociar con supervivencia en los tumores de alto grado. Una mujer posmenopáusica con cáncer de ovario y niveles elevados de CA 125, presentó hidrotórax y ascitis masiva, que son características del síndrome de pseudo-Meigs. Los datos del estudio de inmunohistoquímica fueron consistentes con el carcinoma seroso de alto grado. También se comentan los retos diagnósticos del síndrome y los mecanismos de las efusiones.

A 62-year-old Brazilian man who lived in endemic areas of tropical diseases had an episode of hematemesis associated with portal hypertension. He used to swim in natural ponds during childhood and developed the hepatosplenic form of schistossomiasis with moderate ascites, in addition to the characteristic features of abdominal Caput Medusae. The aim of the report is highlight the role of chronic liver disease and schistossomiasis

Un hombre natural de Brasil de 62 años de edad que vivía en zonas endémicas de enfermedades tropicales presentó un episodio de hematemesis asociada con hipertensión portal. Frecuentemente se bañaba en los estanques naturales durante la infancia y desarrolló la forma hepatosplénica de la esquistosomiasis con ascitis moderada, además de los rasgos abdominales característicos de la Cabeza de Medusa. El objetivo del informe es poner de relieve el papel de la enfermedad hepática crónica y de la esquistosomiasis

Introdução: Doenças cardiovasculares (DCVs) são as principais causas de mortalidade em pacientes portadores de falência renal crônica (FRC). Diversos fatores de risco estão envolvidos na patogênese e são classificados em tradicionais - que afetam a população em geral; e não tradicionais - que são peculiares aos pacientes renais crônicos. Hiperparatireoidismo secundário, um fator não tradicional e comum na FRC, causa aumento da taxa de reabsorção óssea e mobilização do cálcio e do fósforo. À medida que o produto cálcio x fósforo aumenta, a solubilidade desse par iônico pode ser excedida e ocorrer deposição de fosfato de cálcio nos tecidos cardiovasculares (denominada calcificação metastática). Objetivo: Verificar possível relação entre a espessura da artéria carótida primitiva e os níveis de PTH em pacientes com FRC. Métodos: Foram realizados exames ultrassonográficos com Doppler para medir a espessura da artéria carótida e avaliar possíveis correlações entre diferentes elevações nos níveis séricos do PTH, distúrbios minerais e fatores de risco tradicionais e as alterações encontradas na carótida de portadores de FRC dialítica e hiperparatireoidismo secundário. Resultados: Foi observada diferença no nível de colesterol e na idade dos pacientes que apresentavam sinais de calcificação arterial. Também foi detectada relação significativa entre os níveis de PTH e a espessura da parede da carótida (r = 0,31; p = 0,03). Conclusão: Dados desse estudo mostram a possível concomitância de fatores tradicionais e os relacionados com a FRC na gênese das DCVs na uremia.

Introduction: Cardiovascular diseases (CVDs) are the leading cause of death in terminal patients with chronic kidney failure (CKF). Diverse risk factors are involved in the pathogenesis, and are classified as traditional, which affect the general population; and non-traditional, which are peculiar to patients with CKF. Secondary hyperparathyroidism, a non-traditional and common factor in CKF, can cause an increased rate of bone absorption with mobilization of calcium and phosphorus. If the product of calcium x phosphorus is increased, the solubility of this ionic pair may be exceeded and deposition of calcium phosphate in cardiac and vascular tissues occur (called metastatic calcification). Objective: To verify eventual relationship between the thickness of the common carotid artery and the levels of PTH in patients with CKF. Methods: Evaluations by Doppler ultrasonography were performed to measure the width of the carotid artery wall and to search for possible correlations between different values of PTH serum levels, mineral disturbances and traditional risk factors in the carotid changes found in individuals with dialytic CKF and secondary hyperparathyroidism. Results: Differences in the cholesterol level and age were observed in patients with signals of arterial calcification. A significant relationship was also observed between the PTH serum levels and the carotid artery wall thickness (r = 0.31, p = 0.03). Conclusion: Data from this study show the possible concomitance of traditional factors and factors related to CKF in the genesis of CVDs in uremia.

Prader- Willi syndrome is an uncommon multisystem genetic disorder caused by defects of chromosome 15 (15qll-ql3), often due to deletions or uniparental disomy The syndrome is characterized by neonatal hypotonia, dysmorphic facial features, short stature, motor and mental disabilities, behavioral changes, hyperphagia, precocious obesity and hypogonadotropic hypogonadism. We present a 17year-old woman, with a previous genetic diagnosis of Prader-Willi syndrome and BMI of 74 Kg/m², that was admitted in anasarca, with marked cyanosis, dyspnea and oliguria. She presented high levels ofblood urea, creatinine and aminotransferases, in addition to hyperkalemia and hyperuricemia. She had been in regular use of fluoxetine during the last six months, and evolved with severe high bloodpressure and respiratory failure, which needed intensive care support. Moreover, sequéis and clear signs of recent selfinjuries were observed in her trunk, forearms and hands. The findings of morbid obesity, anasarca, self-injury, hyperuricemia and hypoxemia in Prader- Willi syndrome are emphasized.

El síndrome de Prader-Willi es un desorden multisistémico infrecuente causado por defectos genéticos del cromosoma 15 (15qll-ql3), debido a deleciones o disomía uniparental. Se caracteriza por hipotonía neonatal, dismorfias faciales, baja estatura, incapacidades motoras y mentales, problemas conductuales, hiperfagia, obesidad precoz e hipogonadismo hipogonadotrófico. Presentamos una mujer de 17 años, con IMC de 74 Kg/m² con diagnóstico genético previo del síndrome que ingresó con anasarca, intensa cianosis, disnea y oliguria. Presentaba elevación plasmática de urea, creatinina y aminotransferasas, asociadas con hiperkalemia e hiperuricemia. Había utilizado regularmente ñuoxetina durante los seis meses precedentes y evolucionó con hipertensión arterial severa e insuficiencia respiratoria, que requirieron de cuidados intensivos. Además, se constataron cicatrices y claras señales de automutilación reciente en su tronco, antebrazos y manos. Se destacan los hallazgos de obesidad mórbida, anasarca, automutilación, hiperuricemia e hipoxemia en el síndrome de Prader-Willi.