Fulminant or subfulminant liver failure, complicated by encephalopathy and in many cases by death is seen to be a syndrome that may result from numerous causes. Although viral hepatitis, drug-induced hepatitis, and hepatitis due to various types of poisonings, in decreasing frequency, account for 90% of all cases, a variety of miscellaneous conditions account for the remainder. Consideration of the possibility of these less common etiologies by the clinician is of considerable importance, since some, including massive malignant involvement (such as leukemia) or acute fulminant Wilson's disease, may respond to specific treatment measures. Thus, unless hepatic transplantation proves to be applicable in FHF of many etiologic diagnosis may continue to have important therapeutic indications in at least some cases with this syndrome.

Sixty-five patients with histologically proven chronic active hepatitis of unknown cause but associated with the antiliver/kidney microsome antibody type 1, confirmed by immunofluorescence and immunoprecipitation, were selected as forming a special entity. This disease was found to be rare with a prevalence of 5/1,000,000. The female to male ratio was 8:1. The condition occurred at all ages but was most common between the ages of 2 and 14 years. In 22 of the 65 cases, the hepatitis was associated with an autoimmune disease, most commonly insulin-dependent diabetes, autoimmune thyroid disease and vitiligo. The same autoimmune diseases were present in first-degree relatives from seven families. In 36 cases, the onset of disease resembled acute viral hepatitis. Serum biochemical tests showed marked elevation in aminotransaminases and hypergammaglobulinemia. Paradoxically, serum and salivary IgA levels were often normal or low. Histologic findings were multifocal hepatic necrosis with bridging in the acute stage, and aggressive hepatitis with mononuclear cell infiltration or macronodular cirrhosis in the late stages. Serologically, apart from the presence of antiliver/kidney microsome antibody type 1, the disease was characterized by the absence of antiactin, antimitochondria and antinucleus antibodies; however, organ-specific autoantibodies were often present. The clinical course was usually severe: six patients in the acute stage presented with fulminant hepatitis, and all, except two, other patients progressed to cirrhosis. Prolonged treatment with corticosteroids and immunosuppressants was usually effective in rendering the cirrhosis inactive. The cumulative survival rate was 51% at 14 years. We propose to call this entity "anti-LKM1 chronic active hepatitis" or "autoimmune hepatitis type II" to differentiate it from classical "lupoid hepatitis" or autoimmune hepatitis type I.

Portopulmonary hypertension represents a major risk factor for transplantation; therefore, preoperative detection is crucial. The aims of this study were to determine (1) whether Doppler echocardiography performed at evaluation is a reliable tool for detecting portopulmonary hypertension and (2) the incidence of acquired portopulmonary hypertension profile after evaluation. One hundred sixty-five patients had Doppler echocardiography and right heart catheterization at evaluation over a 9-year period. All patients had a prospective follow-up, and the results of catheterization at evaluation were compared with those obtained at the time of transplantation. Seventeen of 165 patients met the criteria for portopulmonary hypertension on Doppler echocardiography. Portopulmonary hypertension was confirmed by catheterization in 10 patients and ruled out in 7. There were no false negatives for echocardiography. Mean pulmonary artery pressure was significantly higher during the initial phase of transplantation than at evaluation (17.8 ؎ 4.3 vs. 20.3 ؎ 5.5 mm Hg, respectively, P < .0001), and there was no significant correlation between values obtained at these 2 time points. Three patients showed to have acquired portopulmonary hypertension profile while waiting for a graft within time intervals ranging from 2.5 to 5 months. In conclusion, Doppler echocardiography is a highly sensitive tool for detecting portopulmonary hypertension. However, because this technique has a poor positive predictive value, right heart catheterization is recommended for confirming portopulmonary hypertension. In addition, the absence of portopulmonary hypertension at evaluation does not exclude the occasional occurrence of acquired portopulmonary hypertension profile after listing. P ulmonary hypertension associated with portal hypertension, the so-called portopulmonary hypertension, is a rare complication of cirrhosis. When severe, this condition is a major risk factor for transplantation because, in most cases, patients are at best partially responsive to medical therapies. 1 If it is impossible to lower mean pulmonary artery pressure below 40 to 50 mm Hg during transplantation procedure, any significant hemodynamic changes, such as those observed at the time of caval clamping and reperfusion of the graft, may result in irreversible cardiac arrest, especially if right ventricular function is impaired. 2 As a consequence, many authors consider that severe portopulmonary hypertension (i.e., mean pulmonary artery pressure [MPAP] above 40 mm Hg) represents a contraindication for liver transplantation because it would carry an unacceptable mortality rate. 3 Because portopulmonary hypertension is frequently asymptomatic until mean pulmonary pressure exceeds 40 mm Hg, most authors recommend systematic screening at evaluation. Several studies have suggested that Doppler echocardiography, when performed during pretransplantation evaluation, is a useful noninvasive tool to document or exclude portopulmonary hypertension, 4-6 even though this technique ...

We assessed prognostic factors in 115 patients with serologically defined fulminant hepatitis B. The diagnosis in each case was based on the finding of IgM antibody to the hepatitis B core antigen in serum. Multivariate analysis showed that factor V level (p less than 0.001), patient's age (p = 0.001), absence of detectable HBsAg by radioimmunoassay (p = 0.06) and serum alpha-fetoprotein concentration (p = 0.07) were independent predictors of survival. The survival rate in the 21 patients in whom HBsAg was not detected was 47%, which was significantly higher than the survival rate of 17% observed in the 94 HBsAg-positive patients (p = 0.006). In patients with fulminant hepatitis B, the absence of HBsAg in serum as detected by radioimmunoassay has an independent, favorable prognostic value.