[Acute hemolytic crisis followed by fulminant hepatic failure with fatal outcome, as a first clinical manifestation of Wilson's disease]. / Crise hemolítica aguda seguida por insuficiência hepática fulminante com evolução fatal, como primeira manifestação clínica da doença de Wilson.

We describe in this work a clinical case of a female patient aged 21 years, bearer of Wilson's disease, a first clinical manifestation of the disease occurred as an acute hemolytic crisis followed by fulminant hepatic failure evolving to death after 26 days' internment. The definitive diagnosis was obtained only as a quantitative measurement of hepatic copper from the necropsy material. The search for Kayser-Fleischer ring was negative and the serum ceruloplasmin level was 9 mg/dl (15 to 60). No involvement of the central nervous system was noted from the pathologic analysis. The patient presented two Coombs negative hemolytic crises during the internment; the first on being admitted to hospital and the second after a transjugular hepatic biopsy carried out on the 16th day after internment. The last hemolytic crisis was accompanied by an increase of serum and urinary copper levels. On this occasion the patient evolved to a progressive hepatic failure with severe jaundice and hepatic encephalopathy. We are presenting the clinical-biochemical evolution of the patient and we shall discuss the existent hypotheses to the pathophysiology of this rare form for manifestation of the Wilson's disease as well the diagnostic difficulties.

[Two cases of severe cutaneous vasculitis with thrombocytopenia associated with hepatic cirrhosis: autoimmune and infective-inflammatory component with endothelial lesion and restrictive pulmonary pattern in one case]. / Dois casos de vasculite cutânea grave com plaquetopenia associada à cirrose hepática: componente autoimune e infecto-inflamatório com lesão endotelial e padrão restritivo pulmonar em um caso.

Two clinical cases of female patients with hepatic cirrhosis and autoimmune multisystemic involvement with infectious intercurrent are reported. Case 1 presented infective endocarditis and erysipelas on the left thigh. In the course of the clinical picture a cutaneous vasculitis developed in the same place together with autoimmune thrombocytopenia, leukopenia and pulmonary restrictive picture with inflammatory pattern. There are also elevate immune complexes and complement consumption. Case 2 presented erysipelas on the left thigh cutaneous vasculitis and complement consumption. In Case 1 the infective endocarditis was treated with antibiotic therapy during 4 weeks followed by 1 mg/kg corticoid (Prednisone) with thrombocytopenia and leukopenia reversion. Case 2 presented an improvement with antibiotic only. The relation between chronic liver diseases and systemic autoimmune phenomena is commented, special attention being paid to the cutaneous, hematological and pulmonary affection.

Massive infestation by Ascaris lumbricoides of the biliary tract: report of a successfully treated case.

This is a report of a 25 years old black woman from the city of São Paulo, Brazil, who developed acute obstructive cholangitis of Ascaris lumbricoides with septicemia and multiple hepatic abscesses. The patient had sickle cell trait and normal delivery 3 months ago. Massive infestation of the biliary tract by Ascaris lumbricoides was diagnosed by abdominal ultrasonography and endoscopic retrograde cholangiography. Sixty worms were removed from the common bile duct and hepatic abscesses were drained by surgery. The infectious process was polymicrobial. The patient's recovery was complete after a long evolution with a wide spectrum antibiotic therapy. New surgeries were needed to remove residual worms in the biliary tract. The diagnostic methods, clinical-biochemical features and also the clinical and surgical management are presented. The biliary ascariasis pathophysiology is commented.

[Clinical, biochemical and histopathological development of post-transfusional non-A, non-B hepatitis from the acute picture to chronicity during 13.3 years]. / Evolução clínico-bioquímica e histopatologica de hepatite não-A, não-B pós-transfusional, do quadro agudo até a cronicidade, com duração de 13.3 anos.

This case was of a 45 year old female patient with a post-transfusion non-A non-B hepatitis which was accompanied since an acute phase to hepatic cirrhosis during a period of 159.7 months or 13.3 years. Four hepatic biopsies were carried out and they divided the follow-up into 5 evolutive periods. The biopsies revealed a progressive histologic from chronic persistent hepatitis to an active chronic hepatitis and cirrhosis. The aminotransferases followed a floating course in the whole period, with ALT greater than AST starting from the 3rd period. The 3rd period (from 5th to 8th year) was of least activity of the aminotransferases, and the 4th and 5th periods (from 8th to 13th year) showed the highest activity of ALT. The 2nd period (from 3rd to 5th year) showed the least portion of gamma globulin and the highest of albumin in comparison with the others. There was no connection between the levels of aminotransferases and the values of gamma globulin and albumin in the follow up process. The treatment employed in the 5th evolutive period (prednisone and colchicine) did not present any biochemical improvement.