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This is the English version of the guidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd–Chiari syndrome, which were established and revised in 2018 by the Aberrant Portal Hemodynamics Study Group under the jurisdiction of the Ministry of Health, Labor, and Welfare in Japan. These guidelines are excerpts, and the full version consists of 86 clinical questions and explanations, totaling 183 pages in Japanese. [ABSTRACT FROM AUTHOR]

Aim: Although the Fontan procedure has improved the survival of patients with single‐ventricle heart disease, the long‐term consequences of the procedure have been a concern. This study aimed to explore the patients' postoperative clinical characteristics, including a diagnosis of Fontan‐associated liver disease (FALD). Methods: A nationwide Japanese epidemiological survey of post‐Fontan patients was undertaken in 2021. The survey targets were selected from all departments of pediatrics, pediatric surgery, cardiology, cardiovascular surgery, and gastroenterology using stratified random sampling by the number of beds. Each department was asked to complete a mail‐back questionnaire on the numbers of patients and their clinical characteristics. The diagnosis of FALD was made by each attending physician. Results: The estimated number of post‐Fontan patients was 7810 (95% confidence interval, 5430−10 200) in 2020, with a period prevalence of 61.9 per million. During the follow‐up of 13.8 years after the Fontan procedure, 40% of patients were diagnosed with FALD. An elevated γ‐glutamyl transpeptidase level was the most common finding leading to the FALD diagnosis (41%), and 45% of the patients also showed liver fibrosis. Compared with non‐FALD patients, FALD patients were older, had longer duration since the Fontan procedure, and had more severe cardiac or liver conditions. However, more than half of the non‐FALD patients had elevated liver enzyme levels, suggesting underestimation of the number of FALD patients. Conclusions: In 2020, approximately 40% of post‐Fontan patients underwent follow‐up with a diagnosis of FALD, although the lack of established diagnostic criteria for FALD could affect the reported prevalence of FALD. [ABSTRACT FROM AUTHOR]

Aim: There are few data regarding the safety and effectiveness of severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) vaccines in patients with intractable hepatobiliary diseases. We conducted a multicenter, questionnaire‐based, cross‐sectional study to determine the safety and effectiveness of the SARS‐CoV‐2 vaccines in Japanese patients with intractable hepatobiliary disease. Methods: Patients aged ≥18 years with autoimmune hepatitis (AIH), primary biliary cholangitis, primary sclerosing cholangitis, Budd–Chiari syndrome, idiopathic portal hypertension, and extrahepatic portal vein obstruction at each center were consecutively invited to join the study. Participants were asked to complete a questionnaire regarding their characteristics, vaccination status, post‐vaccination adverse effects, and SARS‐CoV‐2 infection. Additionally, liver disease status, treatment regimens, and liver function test values pre‐ and post‐vaccination were collected. Results: The survey was conducted from September 2021 to May 2022, and 528 patients (220 AIH, 251 primary biliary cholangitis, 6 AIH– primary biliary cholangitis/primary sclerosing cholangitis overlap, 39 primary sclerosing cholangitis, 4 Budd–Chiari syndrome, 5 idiopathic portal hypertension, and 3 extrahepatic portal vein obstruction) participated in the study. Post‐vaccination adverse effects were comparable to those observed in the general population. Post‐vaccination liver injuries classified as grade 1 or higher were observed in 83 cases (16%), whereas grades 2 and 3 were observed in only six cases (1.1%); AIH‐like liver injury requiring treatment was not observed. Overall, 12 patients (2.3%) were infected with SARS‐CoV‐2, and only one patient was infected 6 months after the second vaccination. Conclusion: SARS‐CoV‐2 vaccines demonstrated satisfactory safety and effectiveness in Japanese patients with intractable hepatobiliary diseases. [ABSTRACT FROM AUTHOR]

Aim Methods Results Conclusion Fontan‐associated liver disease (FALD) is a complication after Fontan surgery, and a common cause of liver tumors and cirrhosis. However, no diagnostic criteria for FALD have been established, leading to an underestimation of its prevalence.We conducted a national survey to elucidate the characteristics of FALD by collecting data from high‐volume centers managing patients who had undergone the Fontan surgery in Japan. In total, 1168 patients were enrolled in the study. First, we examined typical liver findings on ultrasonography after the Fontan surgery. Next, we proposed diagnostic criteria for FALD and advanced FALD based on blood tests, imaging, liver tumors, and pathological examinations. We investigated the sensitivity of histologically diagnosed FALD and advanced FALD based on criteria for blood or imaging tests.Hepatomegaly, hepatic venous dilatation, caudate lobe enlargement, splenomegaly, liver atrophy, ascites, hepatocellular carcinoma, and hepatic tumors other than hepatocellular carcinoma were observed in 37.7%, 29.9%, 18.4%, 33.2%, 3.2%, 6.0%, 0.85%, and 10.0% of patients, respectively. Typical ultrasound findings of FALD included hepatomegaly, hepatic vein dilatation, and splenomegaly, reflecting liver congestion. With the progression of fibrosis, caudate lobe enlargement and splenomegaly became more prominent. Based on these findings, we proposed diagnostic criteria for FALD. Using these criteria, FALD was diagnosed in 1014 (86.8%) of the patients, and all patients with a pathological diagnosis of FALD were successfully identified. Eight patients were found to have pathological cirrhosis, and all were diagnosed with advanced FALD using our criteria based on blood tests or imaging.Our diagnostic criteria facilitate detection of FALD or advanced FALD after the Fontan surgery. The accuracy of these criteria should be further evaluated. [ABSTRACT FROM AUTHOR]

Poor post-vaccination production of antibody against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a concern among solid organ transplant (SOT) recipients. Furthermore, the timing and kinetics of antibody titers after the second vaccine dose are unknown. We conducted a multicenter prospective observational study that included 614 SOT recipients: 460 kidney, 53 heart, 50 liver, 20 lung, and 31 simultaneous pancreas–kidney (SPK). The participants received two doses of the mRNA vaccine (Pfizer BNT162b2 or Moderna mRNA-1273), as indicated. Serum samples were collected before the first and second vaccinations and at 1, 3, and 6 months after the second vaccine dose, which were then assessed for SARS-CoV-2 antibodies. The overall seropositivity rate was 43% at 1 month after administration of the second vaccine dose; it gradually increased to 68% at 3 months after second dose administration and to 70% at 6 months. In addition, recipient of kidney, lung or SPK transplants had lower antibody titers at the 3- and 6-month time points than did the other recipients. SOT recipients acquired SARS-CoV-2 S-IgG antibodies slowly, and the peak titer differed significantly from that of the general population. • Solid organ transplant recipients acquired SARS-CoV-2 antibodies slowly after vaccination. [ABSTRACT FROM AUTHOR]

Background/aims: Treatment using tumor necrosis factor-α (TNF-α) inhibitors is one of the risk factors for active tuberculosis (TB) in patients with Crohn's disease (CD). Biologics, such as ustekinumab (UST) and vedolizumab (VDZ), are less likely to cause opportunistic infections. However, large-scale studies for active TB and biologics other than TNF-α inhibitors are limited. We aimed to investigate the association between biologics and active TB utilizing a Japanese medical claims database., Methods: We analyzed retrospectively the association of the risk of active TB development with treatment using TNF-α inhibitors and other biologics (UST and VDZ) in patients with CD using the Japanese Medical Data Vision (MDV) database between April 2008 and June 2022. The durations of each biologic and biologic-free treatment were calculated for each patient. Univariate and multivariate analyses were performed using the Cox proportional hazards model, with the utilization of biologics considered as time-dependent covariates., Results: We included 28,811 patients with CD in MDV database. Finally, 17,169 patients were analyzed. In total, 7,064 patients were categorized as biologic-naïve, while 10,105 were classified as biologic-experienced. Seventeen patients developed active TB, including 7 on infliximab, 5 on adalimumab, and 5 on no biologics. None of the patients treated with UST and VDZ developed active TB. Multivariate analysis suggested that TNF-α inhibitors were the risk factors for active TB (hazard ratio, 3.66; P= 0.020)., Conclusions: TNF-α inhibitors, but not UST or VDZ, are risk factors for active TB in Japanese patients with CD.

Introduction: Elucidating the epidemiological picture in the early phase of a pandemic is crucial to strengthening preparedness and public health responses to future emerging infectious diseases. Using data from the "Osaka Prefectural Novel Coronavirus Response Status Management System," we evaluated factors associated with mortality among patients with novel coronavirus disease 2019 (COVID-19) in Osaka Prefecture, Japan., Methods: The study periods were from January 29 to June 13, 2020 (first surge), from June 14 to October 9, 2020 (second surge), and from October 10 to December 24, 2020 (up to the middle of the third surge). The odds ratios (ORs) and 95% confidence intervals (95% CIs) for mortality were calculated using logistic regression models., Results: Of the 14,864 patients with COVID-19 (8,207 men, 6,657 women) registered, 297 (2%) died. The ORs for mortality were significantly higher in men (OR = 2.00, 95% CI = 1.54-2.60) than in women, in 70- to 79-year-olds (OR = 25.4, 95% CI = 16.8-38.2) and ≥80-year-olds (OR = 78.1, 95% CI = 53.3-114) than in 0- to 69-year-olds ( P for trend < 0.001), and in those with underlying diseases (OR = 1.74, 95% CI = 1.34-2.27) than in those without. The ORs for the second surge (OR = 0.42, 95% CI = 0.31-0.57) and third surge (OR = 0.41, 95% CI = 0.29-0.58) decreased compared with the first surge. Detailed evaluation of underlying diseases by time period showed that "Diseases of the blood and blood-forming organs and certain disorders involving immune mechanisms," "Endocrine, nutritional, and metabolic diseases," "Diseases of the genitourinary system," and "Diseases of the respiratory system" were associated with increased risk of mortality., Conclusions: Among those affected early in the COVID-19 epidemic, male sex, older age, first-surge infection, and underlying medical conditions were significantly associated with mortality. Our findings are expected to provide a useful reference for future countermeasures in the early stages of pandemics involving unknown emerging infectious diseases., Competing Interests: None, (Copyright © Japan Medical Association.)