[Prolonged survival after local excision of a rectal leiomyosarcoma]. / Sobrevida prolongada luego de la resección local de un leiomiosarcoma de recto.

Leiomyosarcoma is a stromal tumor, originated from smooth muscle cells. The pathologic diagnosis represents a challenge in terms of differentiation from leiomyomas and gastrointestinal stromal tumors (GIST), defined by immunohistochemistry techniques. Its location in the rectum is extremely rare. So, management is not standardized. However, in the largest published series it was found that the abdominoperineal resection leads to better results in the prevention of local recurrence compared with local excision. We present here the case of a 44-year-old woman, whose first clinical manifestation of the disease was fever of prolonged course. A 5 cm leiomyosarcoma was diagnosed at 4 cm from the anal margin. A local transanal resection was performed. The patient is free of disease after 8 years.

[Tumor-induced osteomalacia: rhinosinusal hemangiopericytoma]. / Osteomalacia inducida por tumor: hemangiopericitoma rinosinusal.

Tumor-induced osteomalacia is a rare disease of bone metabolism. The characteristic of this disease is an increase in phosphate excretion followed by hypophosphatemia, due to phosphaturic agents produced by different types of tumors. Tumor resection results in complete resolution of clinical, biochemical and radiological abnormalities. We present the case of a 61 year old man with signs, symptoms and laboratory findings consistent with oncogenic osteomalacia due to a rhino-sinusal mesenchymal tumor. The histological diagnosis showed a vascular neoplasm: hemangiopericytoma.

[A case of melioidosis in Argentina]. / Un caso de melioidosis en la Argentina.

We describe a case of 17-year- old man native of Dominican Republic, with Hodgkin's lymphoma, who presented soft espontaneous draining nodules. In the clinical samples grew Burkholderia pseudomallei; the etiological agent of melioidosis. He received antimicrobial treatment with imipenem and amoxicillin/clavulanic with very good clinical evolution of the infectious process. Melioidosis diagnosis could be underestimated due to the low incidence of Burkholderia pseudomallei in our continent. The definitive diagnosis depends of the isolation and identification in the clinical sample.

[Pulmonary cryptococcoma with involvement of the chest wall in an immunocompetent patient]. / Criptococoma pulmonar con invasión torácica en un varón inmunocompetente.

The pulmonary cryptococcosis in immunocompetent patients is unusual, and its presentation as large masses with involvement of the chest wall and the neighboring soft tissues has not been found in our bibliographic research. The variety gattii (serotype B/C) is limited geographically to tropical and subtropical regions and appears to affect particularly immunocompetent individuals. We describe the case of a 51-year-old man from the Northeast of Argentina, with a history of smoking 10 pack/year. He presented an increased volume of the left shoulder and reported that for the last six months he had been unable to move it due to the pain. A percutaneous biopsy of the mass provided a diagnosis of Cryptococcus neoformans, variety gattii. The patient was treated with antifungal therapy showing a favourable outcome with a progressive decrease of the mass.

[Nutritional status, systemic inflammatory response syndrome and mortality in the elderly hospitalized patient]. / El estado nutricional, la respuesta inflamatoria sistémica y la mortalidad en el anciano internado.

In order to evaluate the relationship between systemic inflammatory response and mortality in the older hospitalized patient, we developed a prospective cohort study in which we evaluated a nutritional score (SGA), years of instruction, functional status, organic failure (Marshall), presence of sepsis, comorbidities (Charlson), cognitive state (MMSE), albumin, erythrocyte sedimentation rate and mortality. Fifty two patients were included, 19 men (36.5%) and 33 women (63.5%), mean age was 80 (Interquartile Range 12.5) years. 29 (55.8%) patients were well-nourished and 23 (44.2%) malnourished, 53.8% of patients developed sepsis at admission or during hospitalization. Total nosocomial mortality was 7.7 % (n = 4) and one-year mortality was 31.8% (n = 14). Comparative analyses showed older age (80 vs. 78; p = 0.012), less years of instruction (7 vs. 8; p = 0.027), lower MMST (14 vs. 27; p = 0.017), lower previous functional status (21 vs. 32; p < 0.0001), lower albumin (3 vs. 3.35; p = 0.014) and higher organic failure score at admission (3 vs. 1; p = 0.01) with more number of affected organs (2 vs. 1; p = 0.003) in malnourished patients compared to well nourished ones. Higher incidence of sepsis -at admission or during hospitalization- (73.9% vs. 37.9%; p = 0.01) and more severe stages of sepsis were also observed in malnourished patients. One-year mortality was significantly higher in malnourished (52.2% vs. 9.5%, log rank test = 0.002). In conclusion, malnourished patients presented greater systemic inflammatory response.

Hospital and 1-year outcomes of septic syndromes in older people: a cohort study.

PURPOSE: Our objective was to describe the relationship between sepsis syndrome mortality and cognitive and physical disability in elderly persons. METHODS: A 1-year consecutive cohort study in clinical beds of a university hospital was performed. Variables were severity of sepsis syndrome, organ failure, functional status, age, sex, and positive cultures. Outcomes were in-hospital and 1-year mortalities. RESULTS: The study included 137 patients (>70 years), both sexes. Data from 116 (84.5%) patients were obtainable at 1-year follow-up. Forty-eight (35%) patients presented with sepsis (11/137, 8%) or severe sepsis (37/137, 27%). In-hospital mortality was 15.3% (0% for sepsis and 21.8% if severe) and increased with organ failure (p <.0001). One-year mortality was 54.78% (63/116), mostly related to severe sepsis; predictors were severe organ failure (p <.0001), prior functional status (p =.0005), and Mini-Mental State Examination (p =.03). Prior functional status and organ failure were independent predictors. CONCLUSIONS: In-hospital and 1-year mortality increased with septic syndrome severity, prior functional status, and organ failure.

[Brown tumor as the initial manifestation of primary hyperparathyroidism]. / Tumor pardo como manifestación inicial de hiperparatiroidismo primario.

Brown tumor is a localized form of osteitis fibrosa cystica, being part of the hyperparathyroid bone disease. It rarely is the first manifestation of hyperparathyroidism, since nowadays, the diagnosis is made at an asymptomatic or minimally symptomatic stage. We present a case of a left superior maxillar brown tumor as the first manifestation of primary hyperparathyroidism due to a parathyroid adenoma. A parathyroidectomy was performed, and there was a regression of the bone lesion, without the need of performing other local surgical procedures.

[Young woman with hyperthyroidism associated with severe tricuspid regurgitation]. / Mujer joven con hipertiroidismo asociado a insuficiencia tricuspídea grave.

Cardiovascular manifestations of hyperthyroidism are frequent and sometimes are relevant in the clinical picture. Usually an hyperdynamic circulatory state hallmarks the disease with low peripheral resistance, increased intravascular volume and cardiac output. However, right chamber dilatation with tricuspid valve regurgitation and cardiac failure are unusual. We present the case of a young woman with Graves-Basedow disease without cardiovascular history who complained about palpitations, peripheral edemas, weight loss and fever. The clinical findings were tachycardia with irregular pulse, right heart failure and regurgitant tricuspid murmur that increased with inspiration. The chest radiograph and the echocardiogram showed right ventricular dilatation and severe tricuspid regurgitation without pulmonary hypertension. The treatment with propranolol, corticosteroids and diuretics was successful. The patient was asymptomatic with sinus rhythm. We discuss the effects of thyroidal hormones on the cardiovascular system and postulate pathophysiologic mechanisms of heart failure in hyperthyroidism.

Osteomalacia inducida por tumor: hemangiopericitoma rinosinusal / Tumor-induced osteomalacia: rhinosinusal hemangiopericytoma

La osteomalacia inducida por tumor es una rara enfermedad del metabolismo óseo caracterizada por el aumento en la excreción de fosfato a nivel renal seguido de hipofosfatemia. Es causada por agentes fosfatúricos producidos por determinados tumores. La resección total del tumor resulta en la completa reversión de las anormalidades bioquímicas, la desaparición de las manifestaciones clínicas y los hallazgos en los estudios por imágenes. Presentamos el caso de un varón de 61 años con cuadro clínico y laboratorio compatibles con osteomalacia oncogénica inducida por tumor mesenquimático de localización rinosinusal. En nuestro caso el diagnóstico histológico correspondió a una neoplasia de tipo vascular: hemangiopericitoma.

Tumor-induced osteomalacia is a rare disease of bone metabolism. The characteristic of this disease is an increase in phosphate excretion followed by hypophosphatemia, due to phosphaturic agents produced by different types of tumors. Tumor resection results in complete resolution of clinical, biochemical and radiological abnormalities. We present the case of a 61 year old man with signs, symptoms and laboratory findings consistent with oncogenic osteomalacia due to a rhino-sinusal mesenchymal tumor. The histological diagnosis showed a vascular neoplasm: hemangiopericytoma.

Un caso de melioidosis en la Argentina / A case of melioidosis in Argentina

Se describe el caso de un varón de 17 años oriundo de República Dominicana, con antecedente de linfoma de Hodgkin, que presenta tumoraciones blandas con supuración espontánea. En sus cultivos desarrolló Burkholderia pseudomallei, agente etiológico de la melioidosis. El paciente recibió tratamiento antibiótico con imipenem y luego con amoxicilina-ácido clavulánico con muy buena evolución clínica del proceso infeccioso. En razón de la baja incidencia de Burkholderia pseudomallei en nuestro continente el diagnóstico de melioidosis pudo haber sido subestimado. Su diagnóstico definitivo depende del aislamiento e identificación del agente causal en la muestra clínica.

We describe a case of 17-year- old man native of Dominican Republic, with Hodgkin´s lymphoma, who presented soft espontaneous draining nodules. In the clinical samples grew Burkholderia pseudomallei; the etiological agent of melioidosis. He received antimicrobial treatment with imipenem and amoxicillin/clavulanic with very good clinical evolution of the infectious process. Melioidosis diagnosis could be underestimated due to the low incidence of Burkholderia pseudomallei in our continent. The definitive diagnosis depends of the isolation and identification in the clinical sample.

Criptococoma pulmonar con invasión torácica en un varón inmunocompetente / Pulmonary cryptococcoma with involvement of the chest wall in an immunocompetent patient

La criptococosis pulmonar en pacientes inmunocompetentes es una entidad poco habitual y su presentación como masas de gran tamaño, con compromiso de la pared torácica y de los tejidos blandos vecinos, no la encontramos descriptas en nuestra revisión bibliográfica. La variedad gattii (serotipoB/C) está acotada geográficamente a regiones tropicales y subtropicales y parece afectar preferentemente a individuos inmunocompetentes. Presentamos el caso de un hombre de 51 años proveniente del noreste de la Argentina, fumador de 10 atados/año que consulta por aumento del volumen del hombro izquierdo e impotencia funcional por intenso dolor de seis meses de evolución. Mediante una biopsia percutánea de la masa, se diagnostica Cryptococcus neoformans, variedad gattii. El paciente recibe terapéutica antifúngica, mostrando una evolución favorable con disminución progresiva de la masa.

The pulmonary cryptococcosis in immunocompetent patients is unusual, and its presentation as large masses with involvement of the chest wall and the neighboring soft tissues has not been found in our bibliographic research. The variety gattii (serotype B/C) is limited geographically to tropical and subtropical regions and appears to affect particularly immunocompetent individuals. We describe the case of a 51-year-old man from the Northeast of Argentina, with a history of smoking 10 pack/year. He presented an increased volume of the left shoulder and reported that for the last six months he had been unable to move it due to the pain. A percutaneous biopsy of the mass provided a diagnosis of Cryptococcus neoformans, variety gattii. The patient was treated with antifungal therapy showing a favourable outcome with a progressive decrease of the mass.

El estado nutricional, la respuesta inflamatoria sistémica y la mortalidad en el anciano internado / Nutritional status, systemic inflammatory response and mortality in the elderly hospitalized patient

La desnutrición en el anciano involucra un estado inflamatorio. Con el objetivo de evaluar en el paciente internado la relación con la respuesta inflamatoria sistémica y la mortalidad desarrollamos un estudio de cohortes prospectivo en el que evaluamos un score nutricional (SGA), años de instrucción, capacidad funcional, falla orgánica (Marshall), presencia de sepsis, comorbilidades (Charlson), estado cognitivo (MMSE), albúmina, eritrosedimentación y mortalidad. Se incluyeron 52 pacientes, 19 hombres (36.5%) y 33 mujeres (63.5%) con una mediana de edad de 80 (RI 12.5) años. Los pacientes normonutridos fueron 29 (55.8%) y los desnutridos 23 (44.2%).El 53.8% de los pacientes desarrollaron sepsis al ingreso o en la internación. La mortalidad intrahospitalaria en toda la muestra fue 7.7% (n = 4) y al año fue del 31.8% (n = 14). En el análisis comparativo se evidenció mayor edad (80 vs. 78; p = 0.012), menos años de instrucción (7 vs. 8; p = 0.027), un MMST menor (14 vs. 27; p = 0.017), menor capacidad funcional previa (21 vs. 32; p < 0.0001), menor valor de albumina (3 vs. 3.35; p = 0.014) y mayor score de falla orgánica de ingreso (3 vs. 1; p = 0.01) con mayor número de órganos afectados (2 vs. 1; p = 0.003) en los desnutridos con respecto a los normonutridos. También se observó mayor incidencia de sepsis -al ingreso o durante la internación- (73.9% vs. 37.9%; p = 0.01) y niveles de sepsis más graves en desnutridos. La mortalidad al año fue significativamente mayor en los desnutridos (52.2% vs. 9.5%, log rank test = 0.002). En conclusión, los pacientes desnutridos presentaron mayor respuesta inflamatoria sistémica.

In order to evaluate the relationship between systemic inflammatory response and mortality in the older hospitalized patient, we developed a prospective cohort study in which we evaluated a nutritional score (SGA), years of instruction, functional status, organic failure (Marshall), presence of sepsis, comorbidities (Charlson), cognitive state (MMSE), albumin, erythrocyte sedimentation rate and mortality. Fifty two patients were included, 19 men (36.5%) and 33 women (63.5%), mean age was 80 (Interquartile Range 12.5) years. 29 (55.8%) patients were well-nourished and 23 (44.2%) malnourished, 53.8% of patients developed sepsis at admission or during hospitalization. Total nosocomial mortality was 7.7 % (n = 4) and one-year mortality was 31.8% (n = 14). Comparative analyses showed older age (80 vs. 78; p = 0.012), less years of instruction (7 vs. 8; p = 0.027), lower MMST (14 vs. 27; p = 0.017), lower previous functional status (21 vs. 32; p < 0.0001), lower albumin (3 vs. 3.35; p = 0.014) and higher organic failure score at admission (3 vs. 1; p = 0.01) with more number of affected organs (2 vs. 1; p = 0.003) in malnourished patients compared to well nourished ones. Higher incidence of sepsis -at admission or during hospitalization- (73.9% vs. 37.9%; p = 0.01) and more severe stages of sepsis were also observed in malnourished patients. One-year mortality was significantly higher in malnourished (52.2% vs. 9.5%, log rank test = 0.002). In conclusion, malnourished patients presented greater systemic inflammatory response.

Tumor pardo como manifestación inicial de hiperparatiroidismo primario / Brown tumor as the initial manifestation of primary hyperparathyroidism

El tumor pardo es una forma localizada de osteítis fibrosa quística, parte del compromiso óseo por hiperparatiroidismo. Como primera expresión de hiperparatiroidismo es infrecuente, debido a que actualmente éste se diagnostica en estadios asintomáticos o mínimamente sintomáticos. Presentamos el caso de una paciente con un tumor pardo localizado en el maxilar superior izquierdo, como primera manifestación de hiperparatiroidismo primario causado por un adenoma paratiroideo. Posterior a la realización de una paratiroidectomía el tumor evolucionó con franca regresión, sin necesidad de ningún otro procedimiento quirúrgico local.

Brown tumor is a localized form of osteitis fibrosa cystica, being part of the hyperparathyroid bone disease. It rarely is the first manifestation of hyperparathyroidism, since nowadays, the diagnosis is made at an asymptomatic or minimally symptomatic stage. We present a case of a left superior maxillar brown tumor as the first manifestation of primary hyperparathyroidism due to a parathyroid adenoma. A parathyroidectomy was performed, and there was a regression of the bone lesion, without the need of performing other local surgical procedures.

Mujer joven con hipertiroidismo asociado a insuficiencia tricuspídea grave / Young woman with hyperthyroidism associated with severe tricuspid regurgitation

Las manifestaciones cardiovasculares del hipertiroidismo son frecuentes, y en ocasiones dominan el cuadro clínico. Con frecuencia, la enfermedad se manifiesta por un estado circulatorio hiperdinámico, con disminución de la resistencia vascular periférica, aumento de la volemia y del volumen minuto cardíaco. La dilatación de las cavidades cardíacas con insuficiencia tricuspídea grave e insuficiencia cardíaca derecha sin hipertensión pulmonar constituye una forma inusual de presentación. Se presenta el caso de una joven paciente, portadora de enfermedad de Graves Basedow, sin antecedentes cardiovasculares, que evoluciona en el transcurso de un mes con edema de miembros inferiores, palpitaciones, diarrea, pérdida de peso y fiebre. Al examen clínico se evidencian taquicardia con pulso irregular, signos de insuficiencia cardíaca derecha acompañado de un intenso soplo regurgitante que aumentaba con la inspiración. Por radiografía de tórax y ecocardiograma se constata cardiomegalia e insuficiencia tricuspídea grave sin hipertensión pulmonar. El cuadro se resuelve en forma rápida luego del tratamiento con propranolol, corticoides y diuréticos, con reversión de la arritmia y franca mejoría de los signos y síntomas. Se analizan en forma breve los efectos de las hormonas tiroideas en el aparato cardiovascular y se postulan probables mecanismos fisiopatológicos de la insuficiencia cardíaca en el hipertiroidismo.

Cardiovascular manifestations of hyperthyroidism are frequent and sometimes are relevant in the clinical picture. Usually an hyperdynamic circulatory state hallmarks the disease with low peripheral resistance, increased intravascular volume and cardiac output. However, right chamber dilatation with tricuspid valve regurgitation and cardiac failure are unusual. We present the case of a young woman with Graves-Basedow disease without cardiovascular history who complained about palpitations, peripheral edemas, weight loss and fever. The clinical findings were tachycardia with irregular pulse, right heart failure and regurgitant tricuspid murmur that increased with inspiration. The chest radiograph and the echocardiogram showed right ventricular dilatation and severe tricuspid regurgitation without pulmonary hypertension. The treatment with propranolol, corticosteroids and diuretics was successful. The patient was asymptomatic with sinus rhythm. We discuss the effects of thyroidal hormones on the cardiovascular system and postulate pathophysiologic mechanisms of heart failure in hyperthyroidism.