Background/Aims: The medical term "Health related Quality of Life (HRQOL)", a subjective index of a patient's health status, has been used as an index for the treatment in wide variety of chronic diseases. Although HRQOL is known to decrease in patients with primary biliary cirrhosis (PBC), there has been no study using a disease specific HRQOL questionnaire for PBC. Recently, Jacoby et al. developed a disease specific questionnaire for PBC (named PBC-40) and reported its usefulness in European patients. Because no such report using PBC-40 has been reported from Japan, we evaluated the usefulness of the PBC-40 questionnaire for Japanese patients. Methods: We drafted a Japanese language version of the PBC-40 questionnaire from the original version established by Jacoby, and sent it to 44 patients with PBC at Gunma University Hospital and affiliated hospitals during the period from October through December, 2007. Clinical characteristics and blood chemistry findings were also evaluated and compared with the PBC-40 results. Results: Mean age of the 44 patients who completed the PBC-40 was 60.9 ± 9.9 years old, and male/female ratio was 1:4.5. Of these patients, 27 were asymptomatic PBC while 17 were symptomatic PBC. The mean total score of PBC-40 was 84.0 ± 28.6, with a significant difference between asymptomatic and symptomatic groups of patients: 72.8 ± 18.6 vs 102.1 ± 33.1, P<0.01. Mean score for each category of PBC-40 was as follows; symptoms 2.0, itching 1.9, fatigue 2.2, cognition 2.0, social 2.0 and emotional 2.9. The emotional score was significantly higher than the other 5 categories (P<0.01). All scores for the 6 subcategories were significantly higher in patients with symptomatic PBC than in those with asymptomatic PBC. According to Spearman's rank correlation, the PBC-40 score showed a significantly positive correlation with alkaline phosphatase (ALP) and total bile acids, and a negative correlation with hemoglobin, prothrombin time, and anti-mitochondrial antibody (AMA) titer (P<0.05). A multiple regression analysis indicated that all of the ALP, total bile acids and AMA titer were significant factors which influenced the PBC-40 scores in our patients (P<0.05). Conclusion: The PBC-40 score was thus found to be significantly higher in the symptomatic PBC patients than in asymptomatic patient. This subjective score was well correlated to the objective indices of disease progression in PBC. The Japanese version of PBC-40 would be useful for accurately assessing the HRQOL in Japanese patients with PBC.

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Background/Aims: This study was aimed at seeing whether an ultrasonographic scoring system for fibrosis-related changes of edge, surface and texture of the liver would be useful to predict future development of hepatocellular carcinoma (HCC) in patients with hepatitis B virus-associated chronic liver disease (HBV-CLD). Methods: A total of 376 patients with HBV-CLD were evaluated by the US score at entry, and followed up prospectively (mean observation period was 29.4 months; 4.0-49.9) for the occurrence of HCC by US or helical CT at three-month intervals. Results: The annual incidence of HCC was 1.3%, 2.0%, and 5.0% in non-cirrhosis (US score<6.0), mild cirrhosis (6.0-7.0) and severe cirrhosis (>7.0), respectively. A multivariate proportional hazard model revealed that the middle and high US scores were independently associated with HCC occurrence (p=0.0334, hazard ratio; 5.769 and p=0.0173, hazard ratio; 6.046, respectively). Conclusion: The US scoring was clearly and strongly associated with the occurrence of HCC in patients with HBV-CLD. Such a scoring system is useful in the management of HBV-CLDs.

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Low doses of natural interferon-alpha (IFN-alpha) were administered to 29 consenting patients. These patients had abnormally high values of transaminases despite long-term glycyrrhizic acid product use, or had discontinued drug therapy due to adverse drug reactions. Efficacy, safety and QOL were examined. As a result, ALT values showed significant decreases in ITT analysis. Emergence of anti-IFN antibody was observed in only one patient. Although QOL did not improve significantly with SF-36 in ITT analysis, PPB analysis of patients that completed administration indicated significant improvement in two subscales (PF: physical functioning, BP: bodily pain). Administration was discontinued in 18 patients, with four due to adverse reactions. In conclusion, long-term low-dose administration of natural IFN-alpha for chronic hepatitis C is safe and effective in that it improves ALT values with rare appearance of IFN-neutralizing antibodies.

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A 41-year-old man was admitted to our hospital for a generalized itching and abnormal liver function test values in late May, 2005. On admission, he was negative for all of the hepatitis virus marker, antinuclear antibody, anti-mitochondrial M2 antibody, anti-LKM1 antibody, and anti-smooth muscle antibody. Histological findings of liver biopsy on this admission were consistent with acute hepatitis. His condition improved immediately after prednisolone (PSL) was administered orally. However, when PSL was discontinued during ambulatory treatment, the liver dysfunction worsened, and the patient was readmitted to our hospital in August. Liver biopsy this time revealed both a severe lymphocytic infiltration in the portal area and evidence of interface hepatitis. On the basis of these findings together with positive antinuclear antibodies at 160×, the patient was diagnosed with autoimmune hepatitis. The liver dysfunction improved immediately after administration of PSL was restarted. This case is intriguing because it illustrates the early phase of the development of autoimmune hepatitis.

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Here we report a case of gastric varices successfully treated by dual balloon-occluded embolotherapy (DBOE). A male in his mid-50s was admitted to our hospital because of bleeding from Lg-cf type gastric varices. The main inflow vessel was the left gastric vein with outflow by a gastro-renal shunt. Percutaneous transhepatic portal obliteration (PTO) via the left gastric vein and balloon-occluded retrograde transvenous obliteration (BRTO) via the gastro-renal shunt were performed simultaneously as DBOE using 5% ethanolamine oleate with iopamidol. Upper gastrointestinal endoscopy three months after DBOE showed the gastric varices had disappeared. Thus, DBOE provides a reliable and safe procedure for ruptured gastric varices where the inflow vessel is the left gastric vein.

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A 79 years old male was notified of a liver tumor at a medical checkup in April 2002: the tumor was visualized by ultrasonography as a hyper-isoechoic lesion with hypo-echoic rim. Ultrasonic contrast enhancement employing Levovist^TM revealed that the tumor was penetrated by Glisson's capsule, with a characteristic fish eye image of the tumor in portal phase. SPIO-MRI of T2-weighted imaging visualized the tumor as a three-layer structure with concentric circles. Without definite preoperative diagnosis, the patient underwent hepatic S6-subsegmentectomy in July 2002. The tumor, 2.8 cm × 2.5 cm in size, was diagnosed ultimately as an epithelioid hemangioendothelioma (EHE) based on positive immunohistological results with antibodies against CD34, Factor VIII, etc. Since hepatic EHE is rare by itself, let alone for the precise analyses of preoperative image-diagnosis findings in comparison with postoperative histological findings, here we report our own case in discussion with those in literatures.

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We investigated the gender difference in responsiveness to 48-week interferon (IFN) and ribavirin combination treatment in patients infected with hepatitis C virus (HCV) genotype 1b and high baseline viral loads. In patients under 50 years old, the sustained viral response (SVR) rate showed no difference by gender. However, in patients over 50 years old, the SVR rate in male was significantly higher than that in female. Multiple regression analysis revealed several factors that are independently associated with better response to the combination therapy: male, no IFN-dose reduction, ICG R15 less than 15% and ribavirin dose/body weight ratio at no less than 11.0. Further investigation would be needed to explain why the effect of the combination therapy varies by gender in those older than 50 years old.

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We have innovated new imaging technique, Defect Re-perfusion Imaging, in the Sonazoid-enhanced contrast ultrasonography (US) for hepatocellular carcinoma (HCC), which is not clearly demonstrated on B mode US. Firstly, perfusion defect can be detected on post-vascular phase imaging 10 min after intravenous injection of Sonazoid. Second, re-injection of Sonazoid clearly shows arterial hypervascularity within the defect portion if the nodule is typical HCC. Since this technique is extremely useful in the detection, treatment guidance and evaluation of treatment response for ultrasound ill-defined HCC nodules, we propose this technique should be widely used in the clinical practice of HCC.

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