[Fulminant Wilson's disease in Costa Rica. Clinico-pathological study of 7 cases]. / Enfermedad de Wilson fulminante en Costa Rica. Estudio clínico patologico de 7 casos.

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.

Prevalence of delta antigen/antibody in B-viral-associated hepatocellular carcinoma.

Sera from 39 patients with B-viral associated hepatocellular carcinoma were studied for the presence of delta antibody using solid-phase blocking radioimmunoassay. Only one patient (2.5%) had circulating delta antibody in the serum, and stainable delta antigen in the nuclei of the nontumor liver. A similar study of the sera of 57 patients with chronic B-viral disease without hepatocellular carcinoma revealed 18 patients (31.5%) with delta antibody. This significant difference (P less than 0.001) in the prevalence of delta antibody among these two groups is probably related to the epidemiologic background, reflected as a racial difference.