Search

Background: Recently, sporadic cases of acute hepatitis and fulminant hepatitis caused by hepatitis E virus (HEV) have been reported in Japan. However, few reports have addressed the issue of HEV infection during childhood., Methods: This study included 5 patients with fulminant hepatitis, 30 patients with acute hepatitis, and 309 patients without history of hepatic dysfunction or hepatitis in childhood as control. RNA was extracted from each serum sample, and HEV specific reverse-transcriptase polymerase chain reaction was performed. Anti-HEV immunoglobulin (Ig)M and IgG were measured by enzyme-linked immunoadsorbent assay., Results: HEV RNA, anti-HEV IgM, and anti-HEV IgG were not detected in the sera of any of the five patients with fulminant hepatitis. In the 30 patients with acute hepatitis, only one (3.3%) was positive for anti-HEV IgG, and all were negative for anti-HEV IgM and HEV RNA. Of the 309 control patients, 8 (2.6%) were positive for anti-HEV IgG, and 2 (0.6%) were positive for anti-HEV IgM, respectively., Conclusion: The result of patients with fulminant hepatitis suggests that HEV is an unlikely cause of fulminant hepatitis in children. However, the detection rate of anti-HEV IgG shows that a history of HEV infection is not so rare among children in Japan.

Background: In recent years, progressive familial intrahepatic cholestasis has been classified into at least three types by genetic analysis: PFIC1, PFIC2, and MDR3. Liver transplantation is effective for treating patients with this intractable syndrome. Confirming the correct diagnosis is of paramount importance because prognosis after transplantation differs with the genetic type of the disease., Methods: Synthesis of cDNA was accomplished using RNA extracted from liver tissue of two Japanese patients with progressive familial intrahepatic cholestasis. Polymerase chain reaction was performed using 13 primer sets designed for amplification of the bile salt export pump cDNA. Direct sequencing was undertaken, and identified sequences were compared with the sequence for bile salt export pump gene registered with GenBank. In addition, gene sequences for nonprogressive familial intrahepatic cholestasis patients were analyzed., Results: Genetic analysis of patient 1 revealed that substitutions in bile salt export pump protein sequences, namely R575X and E636G, might be the cause of the disease. In patient 2, V330X and R487H might fulfill the same role. Results of gene analysis in parents and cholestatic controls supported these conclusions., Conclusions: Absence or presence of bile salt export protein gene mutations was confirmed as representing a useful prognostic marker for clinical course after liver transplantation.