[Analysis of clinical, echocardiographic, microbiological and cytopathological characteristics of pericardial effusions in a tertiary hospital care]. / Análisis de las características clínicas, ecocardiográficas, microbiológicas y citopatológicas de derrames pericárdicos en un hospital de tercer nivel de atención.

OBJECTIVE: To determine the prevalence, etiology, clinical, echocardiographic, microbiological and cytopathological characteristics of patients with pericardial effusion. METHODS: Observational, retrospective, cross-sectional analytical study. We reviewed medical records of patients undergoing pericardiocentesis for a 5 years period. We used descriptive statistics, measures of central tendency and dispersion for analysis. RESULTS: The prevalence of pericardial effusion was 1.1%. Predominant in women (60.4%) and there was a mean age of 49 years. The main causes were neoplastic 32.1%, idiopathic 27.4% and rheumatological 10.4%. A percentage of 27.1 had cardiac tamponade whereas dyspnea and muffled heart sounds were the most common clinical data. The right atrial and ventricular collapse occurred in 84.9 and 75.5%, respectively. The pericardial fluid cytology yielded better in neoplastic causes a sensitivity of 54%, specificity 95%, positive predictive value 85% and negative predictive value 81%. CONCLUSIONS: The prevalence of pericardial effusion in a tertiary care hospital was 1.1%, the main cause was neoplastic. In the evaluation of moderate or severe pericardial effusion we found that right atrial and ventricular collapses were the most common echocardiographic findings. The cytopathological study had a high specificity for the diagnosis of neoplasia.

[Wiskott Aldrich syndrome associated to vasculitis: a therapeutic challenge]. / Síndrome de Wiskott Aldrich asociado con vasculitis: un desafÌo terapéutico.

Wiskott Aldrich syndrome (WAS) is an X-linked primary immunodeficiency, associated with WASP gene mutation that causes severe immunological abnormalities and alterations in platelet function. A seven year old male patient with WASP, began with acute abdominal pain, fever and knee swelling. The diagnosis of septic arthritis was made, and he was treated with broad-spectrum antibiotics and human gammaglobulin. During treatment he presented digestive tract bleeding with hypovolemic shock; after 72 hours palpable purpura in upper and lower extremities appeared. Then Henoch-Schönlein purpura with abdominal vasculitis was suspected, and later confirmed by histopathology. Methylprednisolone pulses were initiated, showing improvement within 24 hours. The patient had a severe inflammatory reaction, caused by a serious infectious disease, but the clinical evolution suggested an autoimmune disease such as Henoch-Schönlein purpura. Up to 20% of patients with WAS have autoimmune manifestations of vasculitis. An early diagnosis of autoimmunity in WAS is important for a favorable clinical outcome.

Análisis de las características clínicas, ecocardiográficas, microbiológicas y citopatológicas de derrames pericárdicos en un hospital de tercer nivel de atención / Analysis of clinical, echocardiographic, microbiological and cytopathological characteristics of pericardial effusions in a tertiary hospital care

Objetivo: Conocer la prevalencia, las causas, los hallazgos clínicos, ecocardiográficos, microbiológicos y citopatológicos de pacientes con derrame pericárdico. Métodos: Estudio observacional, retrospectivo, transversal y analítico. Se analizaron expedientes clínicos de pacientes que reciben pericardiocentesis durante un periodo de 5 años. Se empleó estadística descriptiva, con medidas de tendencia central y de dispersión para el análisis. Resultados: La prevalencia de derrame pericárdico fue del 1.1%. Predominó en mujeres (60.4%) y se observó una media de edad de 49 años. La principal causa asociada fue neoplásica, con un 32.1%, seguida de la idiopática y reumatológica con un 27.4 y 10.4%, respectivamente. Se presentó taponamiento cardiaco en un 27.1%, siendo la disnea y la presencia de tonos cardiacos apagados los datos clínicos más comunes. El colapso auricular y ventricular derechos se presentaron en el 84.9 y 75.5%, respectivamente. El estudio citopatológico de líquido pericárdico presentó un mayor rendimiento diagnóstico en el estudio de derrames asociados a neoplasias, mostrando una sensibilidad del 54%, una especificidad del 95%, un valor predictivo positivo del 85% y un valor predictivo negativo del 81%. Conclusiones: La prevalencia de derrame pericárdico en un hospital de tercer nivel fue del 1.1%, predominando la etiología neoplásica. El colapso auricular y ventricular derechos son los hallazgos ecocardiográficos más comunes en la presencia de derrame pericárdico moderado o severo. El estudio citopatológico tiene una alta especificidad ante la sospecha de causa neoplásica.

Objective: To determine the prevalence, etiology, clinical, echocardiographic, microbiological and cytopathological characteristics of patients with pericardial effusion. Methods: Observational, retrospective, cross-sectional analytical study. We reviewed medical records of patients undergoing pericardiocentesis for a 5 years period. We used descriptive statistics, measures of central tendency and dispersion for analysis. Results: The prevalence of pericardial effusion was 1.1%. Predominant in women (60.4%) and there was a mean age of 49 years. The main causes were neoplastic 32.1%, idiopathic 27.4% and rheumatological 10.4%. A percentage of 27.1 had cardiac tamponade whereas dyspnea and muffled heart sounds were the most common clinical data. The right atrial and ventricular collapse occurred in 84.9 and 75.5%, respectively. The pericardial fluid cytology yielded better in neoplastic causes a sensitivity of 54%, specificity 95%, positive predictive value 85% and negative predictive value 81%. Conclusions: The prevalence of pericardial effusion in a tertiary care hospital was 1.1%, the main cause was neoplastic. In the evaluation of moderate or severe pericardial effusion we found that right atrial and ventricular collapses were the most common echocardiographic findings. The cytopathological study had a high specificity for the diagnosis of neoplasia.

[Griscelli syndrome in a Mexican girl]. / Síndrome de Griscelli en una niña mexicana.

Griscelli syndrome is an infrequent disease first described in 1978. It is inherited in autosomal recessive form, and is distinguished by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement and uncontrolled phases of macrophage and lymphocyte activation. We report the case of a female child who started with ataxic gait when she was 23 months old. At physical examination a phenotype with brown skin and silvery gray hair, eyebrows and eyelashes was observed. Neurological evolution was with remissions and exacerbations, with cerebellar and, finally, bulbar compromise.