Tratamiento con sirolimus y prednisona en enfermedad de Erdheim-Chester con compromiso cardiaco: caso clínico / Sirolimus and prednisone for the treatment of Erdheim-Chester disease with cardiac involvement: report of one case

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder. We report a 76-years-old man who suffered a cardiac tamponade secondary to ECD. A pericardial window was made and during the operation the surgeons observed that the myocardium was diffusely infiltrated. Twenty-eight months before, ECD was clinically diagnosed and prednisone and methotrexate were prescribed. Due to disease progression which culminated in the cardiac tamponade, methotrexate was changed to sirolimus aiming to obtain plasma levels between 5-8 ng/ml. This treatment stabilized his cardiac function allowing a survival of 52 months after its initiation, with fewer side effects.

Calcificaciones pulmonares metastásicas en un trasplantado renal con un injerto con función renal adecuada: Caso clínico / Metastatic pulmonary calcification in a patient with a functioning kidney allograft: Report of one case

In renal transplants patients, metastatic pulmonary calcifications have been reported occasionally when the grafts are dysfunctional and rarely when they are functioning normally. We report a male who received a renal allograft in 1994 at the age of 61 years. Nineteen years later a routine chest X ray showed diffuse infiltrates and a CT scan showed diffuse calcifications in both lungs. These were interpreted as metastatic pulmonary calcifications. The last available laboratory determinations were a serum creatinine of 1.4 mg/dl and urinary protein excretion of 255 mg/24 hours. No further studies were done since the patient experienced a sudden death due to an acute myocardial infarction.

Fibrosis retroperitoneal asociada a uso crónico de ergotamina. Tratamiento con colchicina y esteroides: Caso clínico / Retroperitoneal fibrosis associated with chronic use of ergotamine: Report of one case

Retroperitoneal fibrosis (RPF) associated with chronic use of ergotamine is a very rare disorder. We report a 45-year-old woman who presented with a RPf after using, almost daily for 23 years, ergotamine tartrate for migraine relief. FRP presented as a chronicinflammatory state, anemia, abdominal and lumbosacral pain and a hypogastric mass. A CT-Scan showed a periaortic mass and left hydronephrosis. A percutaneous biopsy was obtained and the patient was subjected to a surgical ureterolysis and tissue resection. The biopsy confirmed the presence of RPf. Due to persistent symptoms and increase in the volume of periaortic tissue, treatment with colchicine 1 mg/day and defazacort 30 mg/day was started, resulting in a rapid di-sappearance of symptoms, disappearance ofinflammation and a significant reduction in the volume of the periaortic tissue. The patient remains in complete remission after 29 months of follow up.

Pronóstico de pacientes ingresados a Unidad de Cuidados Intensivos por falla respiratoria asociada a presencia de infiltrados pulmonares / Prognosis of patients with acute respiratory failure and pulmonary infiltrates admitted to intensive care unit

En la unidad de cuidados intensivos (UCI), la insuficiencia respiratoria aguda (IRA) asociada a infiltrados pulmonares de causa desconocida se relaciona con alta morbilidad y mortalidad. El objetivo principal de este trabajo fue conocer el pronóstico de pacientes con IRA, infiltrados pulmonares y necesidad de ventilación mecánica (VM) ingresados a UCI. Como objetivo secundario se planteó evaluar la utilidad de medios diagnósticos de uso habitual en este grupo de pacientes en relación al pronóstico y la conducta terapéutica. Se realizó un estudio observacional retrospectivo que incluyó todos los pacientes ingresados a la UCI del Hospital Naval Almirante Nef por IRA de causa desconocida asociada a infiltrados pulmonares en radiografía de tórax y que tengan necesidad de VM entre los años 2006 y 2010. Se incluyeron 90 pacientes de los cuales 45,6 por ciento fueron de sexo masculino. La edad promedio fue 66 años (DS 17,1). El puntaje promedio de score APACHE II fue 20,6 (DS 7,7). La mortalidad fue 52,2 por ciento y el tiempo promedio de estadía en UCI fueron 14 días (DS 14,4). La principal causa de IRA fue síndrome de distress respiratorio agudo (32 por ciento).Otras causas fueron neumonía adquirida en la comunidad (28,2 por ciento), neumonía aspirativa (12,2 por ciento), neumonía nosocomial (7,8 por ciento) y neumonía criptogénica organizada (6,7 por ciento). La realización de tomografía computarizada de tórax, lavado broncoalveolar y biopsia pulmonar determinó cambio de conducta terapéutica en 31 por ciento, 58 por ciento y 88 por ciento de los casos, respectivamente. En conclusión, los pacientes con IRA tienen alta mortalidad. La causa más frecuente fue el síndrome de distress respiratorio agudo. El uso de procedimientos diagnósticos pueden cambiar las medidas terapéuticas empleadas, en especial la biopsia pulmonar.

In the intensive care unit (ICU), acute respiratory failure (ARF) associated with unexplained pulmonary infiltrates is associated with high morbidity and mortality. The main objective of this study was to determine the prognosis of patients with ARF and pulmonary infiltrates admitted to ICU. The secondary objective was proposed to evaluate the usefulness of diagnostic methods frequently used in this patient group and correlated them with prognosis and therapeutic management. We performed a retrospective observational study that included all patients admitted to ICU of Almirante Nef Naval Hospital with ARF of unknown cause associated with pulmonary infiltrates on chest radiography during the years 2006-2010. We included 90 patients of which 45 percent were male. The average age was 66 years. The average APACHE II score was 20.6. Mortality was 52.2 percent and the average length of stay in the ICU was 14 days (SD 14.4). The main cause of ARF was acute respiratory distress syndrome (32 percent). Other causes were community-acquired pneumonia (28.2 percent), aspiration pneumonia (12.2 percent), nosocomial pneumonia (7.8 percent) and cryptogenic organized pneumonia (6.7 percent. The performance of chest computed tomography, bronchoalveolar lavage and lung biopsy determined therapeutic behavior change in 31 percent 58 percent and 88 percent of cases, respectively. In conclusion, patients with ARF have high mortality. The most common cause was acute respiratory distress syndrome. The use of diagnostic procedures may change the therapeutic measures used, particularly lung biopsy.