Taponamiento cardiaco como forma de inicio de fiebre mediterránea familiar con herencia autosómica dominante / Cardiac tamponade as first manifestation in mediterranean fever with autosomal dominant form

La fiebre mediterránea familiar (FMF) es una enfermedad hereditaria que se caracteriza por episodios breves y recurrentes de fiebre y dolor por inflamación de una o varias serosas (peritoneo, pleura, pericardio, sinovial o túnica vaginal del testículo). La amiloidosis es su complicación más importante y suele ser la principal causa de muerte en los casos en que se presenta. El diagnóstico se basa en la clínica y se confirma mediante pruebas genéticas. Para el tratamiento, se utiliza colchicina a 0,02-0,03 mg/kg/día, que permite tanto evitar la crisis como el desarrollo de la insuficiencia la renal. Presentamos el caso de un niño de 13 años en el que se diagnosticó FMF tras varios episodios coincidentes con fiebre de pericarditis con taponamiento cardiaco. La confirmación genética mostró un patrón de herencia poco frecuente autosómico dominante

Familial Mediterranean fever (FMF) is a hereditary disease characterized by brief, recurring and self-limited episodes of fever and pain with inflammation, of one or several serous (peritoneum, pleura, pericardium, synovial or vaginal tunic of the testicle). Amyloidosis is its more important complication and the principal reason of death in the cases in which it appears. Diagnosis is based on the clinic and is confirmed by genetic tests. The treatment with Colchicine (0,02-0,03 mg/kg/day) prevents the recurrence of FMF attacks and the development of secondary (AA) amyloidosis. We report a case of a 13-year-old child in which FMF was diagnosed after several coincidental episodes with fever, pericarditis and cardiac tamponade. The genetic confirmation showed an autosomal dominant inheritance that is less frecuent than the recesive form, in this disease

[Cardiac tamponade as first manifestation in Mediterranean fever with autosomal dominant form]. / Taponamiento cardiaco como forma de inicio de fiebre mediterránea familiar con herencia autosómica dominante.

Familial Mediterranean fever (FMF) is a hereditary disease characterized by brief, recurring and self-limited episodes of fever and pain with inflammation, of one or several serous (peritoneum, pleura, pericardium, synovial or vaginal tunic of the testicle). Amyloidosis is its more important complication and the principal reason of death in the cases in which it appears. Diagnosis is based on the clinic and is confirmed by genetic tests. The treatment with Colchicine (0,02-0,03 mg/kg/day) prevents the recurrence of FMF attacks and the development of secondary (AA) amyloidosis. We report a case of a 13-year-old child in which FMF was diagnosed after several coincidental episodes with fever, pericarditis and cardiac tamponade. The genetic confirmation showed an autosomal dominant inheritance that is less frecuent than the recesive form, in this disease.

[Francisella tularensis infection transmitted by prairie dog]. / Infección por Francisella tularensis transmitida por un perro de las praderas.

Tularemia is a zoonosis caused by Francisella tularensis; it mainly affects wild animals and only occasionally affects human beings. Uncontrolled trade involving "exotic" animals could become a new route of acquisition. We report the case of a 3-year-old boy who contracted the disease through a bite from a prairie dog. Outbreaks of tularemia have recently been described in some of these animals captured in Texas (USA) and subsequently sold to other countries. Infection due to Yersinia pestis has also been described in humans through contact with prairie dogs with the disease. Streptomycin and gentamicin are currently the drugs of choice against tularemia infection.

Infección por Francisella tularensis transmitida por un perro de las praderas / Francisella tularensis infection transmitted by prairie dog

La tularemia es una zoonosis causada por Francisella tularensis, que afecta principalmente a animales silvestres, y de forma casual al ser humano. La comercialización incontrolada de animales de origen "exótico" puede suponer una nueva vía de adquisición. Presentamos el caso de un varón de 3 años que presentó la enfermedad tras la mordedura de un perro de la pradera. Recientemente se han descrito brotes de tularemia en estos animales capturados en Texas (EE.UU.), y distribuidos comercialmente en varios países. También se ha descrito la infección en humanos por Yersinia pestis tras el contacto con perros de la pradera enfermos. La estreptomicina y la gentamicina son actualmente los fármacos de elección frente a las infecciones por tularemia (AU)