title: [Cardiac tamponade as first manifestation in Mediterranean fever with autosomal dominant form].
abstract: 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.
authors.affiliation: Servicio de Pediatría, Hospital Universitario de San Juan, Alicante, España. Electronic address: pacosanchezferrer0@hotmail.com.
authors.forename: F
authors.initials: F
authors.lastname: Sánchez Ferrer
authors.affiliation: Servicio de Pediatría, Hospital General Universitario, Alicante, España.
authors.forename: M
authors.initials: M
authors.lastname: Martinez Villar
authors.affiliation: Servicio de Pediatría, Hospital General Universitario, Alicante, España.
authors.forename: A
authors.initials: A
authors.lastname: Fernández Bernal
authors.affiliation: Servicio de Pediatría, Hospital General Universitario, Alicante, España.
authors.forename: I
authors.initials: I
authors.lastname: Martín de Lara
authors.affiliation: Servicio de Pediatría, Hospital General Universitario, Alicante, España.
authors.forename: I
authors.initials: I
authors.lastname: Paya Elorza
mesh_terms: D000293:Adolescent; D002305:Cardiac Tamponade / Q000209:etiology*; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000175:diagnosis / Q000235:genetics*; D005799:Genes, Dominant; D006801:Humans; D008297:Male
keywords: Autosomal dominant; Autosómico dominante; Cardiac tamponade; Fiebre mediterránea familiar; Mediterranean fever; Taponamiento cardiaco