Changing clinical and molecular characteristics of hepatitis E virus infection in Mie Prefecture, Japan: Disappearance of indigenous subtype 3e strains.

AIM: To evaluate the clinical and molecular characteristics of hepatitis E virus (HEV) infection in Mie Prefecture, Japan, from 2004 through 2018. METHODS: The clinical information of hepatitis E cases was collected from 21 medical institutions in Mie Prefecture. The nucleotide sequences of infecting HEV strains were determined for cases with available serum samples. The origins or transmission routes were inferred from phylogenetic analyses of the nucleotide sequences. RESULTS: Fifty-three patients were diagnosed with HEV infection. The number of cases increased each year through 2012 and then decreased. Analyses of the clinical characteristics of the cases indicated that even mild cases were detected in the latter 10 years of the study. Nucleotide sequence analyses were undertaken on 38 of the 53 cases. The HEV subtype 3e (HEV-3e) strains identified for 13 cases were closely related to a swine HEV-3e strain that was isolated from the liver of a pig bred in Mie Prefecture. The number of cases infected with the indigenous Mie HEV-3e strains increased until 2012 but have not been reported since 2014. In the latter half of the study, cases involving various HEV strains of different genotypes and subtypes emerged. CONCLUSIONS: The disappearance of indigenous Mie HEV-3e strains appeared to be the primary cause for the decrease in hepatitis E cases in Mie Prefecture. The disappearance might have been associated with improved hygienic conditions on pig farms or the closure of contaminated farms. The results suggest that indigenous HEV strains can be eradicated by appropriate management.

Validity of Japanese version of SARC-F questionnaire in patients with chronic liver disease.

BACKGROUND AND AIM: We aimed to examine the validity of the Japanese version of SARC-F questionnaire (SARC-F-J) that employs the diagnostic criteria for sarcopenia established by the Japan Society of Hepatology in patients with chronic liver disease. METHODS: Subjects were outpatients at the Department of Hepatology at the Japanese Red Cross Ise Hospital, Japan. Evaluations were performed using the following self-administered questionnaires: SARC-F-J, Tokyo Metropolitan Institute of Gerontology Index of Competence (TMIG-IC), the Japanese version of the Falls Efficacy Scale (FES), and Kaigo-Yobo Checklist (CL). Based on the diagnostic criteria of the Japan Society of Hepatology, we diagnosed sarcopenia from the skeletal muscle index calculated using the iliopsoas muscle area at the third lumbar vertebra on computed tomography and from grip strength. To evaluate construct validity, we calculated the sensitivity, specificity, and positive and negative predictive values of SARC-F-J that used the diagnostic criteria of the Japan Society of Hepatology as reference. Furthermore, to evaluate convergent validity, we calculated Pearson's correlation coefficients between SARC-F-J and TMIG-IC, FES, and CL. RESULTS: A total of 140 subjects were included in the analysis set. Sensitivity and specificity were 16.3% and 45.0% and 95.3% and 90.8% for men and women, respectively. The positive predictive value was 81.8% for both, whereas the negative predictive value was 47.1% and 64.5% for men and women, respectively. A significant correlation was seen between SARC-F-J and TMIG-IC, FES, and CL. CONCLUSIONS: We believe that the SARC-F-J is a valid tool for patients with chronic liver disease.

A 10-year Follow-up Study of a Japanese Family with Ferroportin Disease A: Mild Iron Overload with Mild Hyperferritinemia Co-occurring with Hyperhepcidinemia May Be Benign.

This is a 10-year follow-up study of a family with ferroportin disease A. The proband, a 59-year-old man showed no noteworthy findings with the exception of an abnormal iron level. The proband's 90-year-old father showed reduced abilities in gait and cognition; however, with the exception of his iron level, his biochemistry results were almost normal. Brain imaging showed age-matched atrophy and iron deposition. In both patients, the serum levels of ferritin and hepcidin25, and liver computed tomography scores declined over a 10-year period. These changes were mainly due to a habitual change to a low-iron diet. The iron disorder in this family was not associated with major organ damage.

[A case of adult-onset type II citrullinemia (CTLN2) triggered by an overseas travel].

A 43-year-old male presented with abnormal behavior and consciousness disturbance on the day after traveling abroad and was admitted to our hospital. Laboratory tests showed hyperammonemia and hypercitrullinemia. The electro-encephalogram showed frontal dominant bilateral slow δ burst. He had a peculiar taste for nuts. But he didn't take nuts during the overseas travel for 3 days. The family history revealed that his younger brother died of a status epilepticus of unknown cause at the age of 29. These findings were compatible with hepatic encephalopathy due to adult-onset type II citrullinemia (CTLN2). Gene analysis provided a definite diagnosis of CTLN2. Diet and drug therapy have improved his condition. He is due to have liver transplantation which is the only established radical treatment for CTLN2 if his condition becomes worse. The present case shows that cessation of the habitual intake of nuts only for 3 days could lead to onset of CTLN2.