[Fabry's disease. Rare etiology of a long-term inflammatory syndrome. Apropos of a case].
A man aged 54 years presented multiple symptoms (acroparesthesia, familial deafness, cardiomyopathy, diarrhea, adenopathy with infiltration of frothy macrophages, pancytopenia with a dense marrow, chronic meningitis, renal failure) associated with intermittent fever, with feverish attacks and a temperature of 40 degrees C, and with a severe biologic febrile syndrome. Fabry's disease was diagnosed only after 3 years of fruitless explorations. The reasons for this delay are analysed and it is suggested than Fabry's disease be added to the list of conditions responsible for fever or for a persistent inflammatory syndrome.
D003967:Diarrhea / Q000209:etiology; D000795:Fabry Disease / Q000150:complications*; D005335:Fever of Unknown Origin / Q000209:etiology*; D006801:Humans; D007249:Inflammation / Q000209:etiology*; D008297:Male; D008581:Meningitis / Q000209:etiology; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use
D011241:Prednisone
La maladie de Fabry. Etiologie rare de syndrome inflammatoire au long cours. A propos d'une observation.