title: [Fulminant Wilson's disease in Costa Rica. Clinico-pathological study of 7 cases].
abstract: In the last eighteen years, from 1972 to 1989, around 150 cases of Wilson's disease have been diagnosed in Costa Rica (6/100.000 inhabitants). In the San Juan de Dios Hospital, 120 cases have been studied during this period, seven of whom died with a picture of acute hepatic insufficiency, hemolytic anemia, encephalopathy, intestinal bleeding and renal insufficiency. In four of the cases, postmortem histopathologic studies were done with high resolution microscopy, which revealed extensive submassive necrosis of the liver, with severe cholestatic, lytic and acidophilic necrosis with nodular, irregular regeneration and specially microvacuolar steatosis, different from that observed in other forms of fulminant hepatitis. With the clinical, laboratory and histopathologic findings, we concluded that fulminant Wilson's disease is a well-defined pathological clinical entity of fatal evolution with no response to therapy, including early treatment with penicillamine and steroids.
authors: Unidad de Hígado, Laboratorio R. L. Peters, Hospital San Juan de Dios, San José, Costa Rica.
authors: S A
authors: SA
authors: Herra
authors: F J
authors: FJ
authors: Hevia
authors: M
authors: M
authors: Vargas
authors: K
authors: K
authors: Schosinsky
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D002648:Child; D003300:Copper / Q000378:metabolism; D003364:Costa Rica / Q000453:epidemiology; D005190:Family; D005260:Female; D006527:Hepatolenticular Degeneration / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D008297:Male; D012189:Retrospective Studies
chemical_list: D003300:Copper
vernacular_title: Enfermedad de Wilson fulminante en Costa Rica. Estudio clínico patologico de 7 casos.