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Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end‐stage liver disease. Mizoribine, a purine antimetabolite, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we undertook a preliminary clinical study in children with PSC. Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated. The primary end‐points of our study included improvement of aspartate aminotransferase, alanine aminotransferase, and γ‐glutamyltransferase. Liver histology, immunostaining studies of the liver, and magnetic resonance cholangiopancreatography (MRCP) were also assessed. We have treated four PSC children: two treatment‐naïve patients (cases 1 and 2), and two with established liver cirrhosis (cases 3 and 4). Both case 1 and 2 showed a normalization of liver enzymes and case 2 showed an improvement in MRCP findings. Cases 3 and 4 also showed an improvement in varices, MRCP findings, and liver histology. The combination therapy may be effective for some children with PSC and autoimmune features. By ameliorating both parenchymal inflammation and cholangiopathy of PSC, the therapy might improve the prognosis for patients. It awaits further prospective studies to confirm the efficacy of this therapy in patients with PSC. [ABSTRACT FROM AUTHOR]

Abstract: Mother‐to‐child transmission of the hepatitis B virus (HBV) is a major concern for infected mothers, especially after their first child has become an HBV carrier despite immunoprophylaxis. Eight mothers whose first child had become an HBV carrier despite immunoprophylaxis were referred for antiviral therapy during the subsequent pregnancy. All pregnant women were seropositive for both the hepatitis B surface antigen and hepatitis B e antigen. In the treatment group (three receiving lamivudine and two receiving tenofovir), mother‐to‐child transmission of the HBV was successfully prevented in all infants (5/5). On the other hand, two of three infants became HBV carriers in the untreated group. There were no significant adverse effects in either mothers or infants. Antiviral therapy using lamivudine and tenofovir during the second pregnancy successfully prevented mother‐to‐child transmission of the HBV for high‐risk mothers. [ABSTRACT FROM AUTHOR]

Background and Aim Childhood-onset inflammatory bowel disease (IBD) is characterized by extensive intestinal involvement and rapid early progression. Infliximab (IFX), cyclosporin (CYA), and tacrolimus (FK506) are increasingly used to treat pediatric IBD; however, their long-term effects and adverse events have not been properly investigated in pediatric patients. The aim of this study was to characterize the effects of these biologics and immunomodulators on pediatric IBD patients in Japan. Additionally, we assessed IFX use in pediatric patients with Crohn's disease (CD). Methods A national survey of IFX, adalimumab, CYA, and FK506 use in pediatric IBD patients (< 17 years of age) was sent to 683 facilities in Japan from December 2012 to March 2013. Secondary questionnaires were sent to pediatric and adult practitioners with the aim of assessing the effectiveness and safety of IFX for pediatric CD patients. Results The response rate for the primary survey was 61.2% ( N = 418). Among 871 pediatric CD patients, 284 (31.5%), 24, 4, and 15 received IFX (31.5%), adalimumab, CYA, and FK506, respectively, from 2000 to 2012. According to the secondary survey, extensive colitis (L3, Paris classification) was diagnosed in 69.4% of pediatric CD patients who received IFX. Regarding the effectiveness of IFX in this population, 54.7% (99/181) of patients were in remission, and 42.0% (76/181) were on maintenance therapy. However, 32.0% (58/181) of patients experienced adverse events, and one patient died of septic shock. Conclusions Infliximab is reasonably safe and effective in pediatric CD patients and should therefore be administered in refractory cases. [ABSTRACT FROM AUTHOR]

Objectives. In short-term observations, interferon (IFN) therapy has been shown to be effective in producing both biochemical and virological responses in children with chronic hepatitis B virus (HBV) infection. However, in long-term follow up, no studies have shown a clear advantage of IFN therapy during childhood. We conducted a retrospective study on the sustained effect of IFN therapy among a Japanese pediatric population. Methods and subjects. A retrospective study was performed on 155 children with chronic HBV infection who were followed in two affiliated hospitals during the period from 1986 to 2013. Results. The 155 patients comprised 97 males and 58 female. Infection route was maternal transmission in 96/155 patients. HBV genotype was A in 17, B in 6, and C in 51 patients. IFN therapy was performed in 48 patients. One year after the completion of IFN therapy, normalization of alanine aminotransferase (ALT) and lower viral levels (<104 copies/ml) was observed in 43 and 29 patients, respectively. The sustained effects of IFN therapy were evaluated by comparison between 43 hepatitis B e-antigen (HBeAg)-positive patients treated with IFN and 67 patients with chronic hepatitis B observed without IFN therapy. A Cox's proportional hazard analysis showed a higher seroconversion rate in the IFN group than in the untreated group ( p = 0.003). Similarly, there were higher rates of ALT normalization and lower viral levels in the IFN group than in the untreated group ( p = 0.001 for both). Conclusion. IFN therapy showed sustained effects for achieving ALT normalization and HBeAg seroconversion and for reducing the viral load in children with chronic hepatitis B. [ABSTRACT FROM AUTHOR]

Aim The objective of the current study was to find baseline predictive factors of response to therapy with pegylated interferon and ribavirin ( PEG-IFN/ RBV therapy) in children and adolescents with chronic hepatitis C. Methods IL28B genotype and mutations in the core of hepatitis C virus ( HCV) were analyzed in 30 patients treated with PEG-IFN/ RBV for HCV infection. The initial rate of decrease in the viral load was assessed during the first 2 weeks of treatment. Results IL28B major allele was seen more frequently in patients with sustained virologic response ( SVR) than in non- SVR patients ( P < 0.001). There was no difference between these two groups in frequency of Core 70 mutation. Among patients with genotype-1, SVR was achieved in more patients ( P = 0.007) in the IL28B major allele group than in those in the minor allele group. The early decrements in the viral load (log/2 weeks) were 3.80 ± 0.86 in the genotype-2 major allele group, 1.82 ± 0.84 in the genotype-1 major allele group, and 0.41 ± 0.33 in the genotype-1 minor allele group. Conclusions Among pediatric patients with HCV infection the effectiveness of PEG-IFN/ RBV therapy may be lower in the group with genotype-1 IL28B minor alleles than in other groups with IL28B major allele. Treatment strategy should be carefully implemented in patients with IL28B unfavorable type. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To estimate an efficacy of intravenous methylprednisolone pulse therapy (m-PSL) and the occurrence of corticosteroid-related side effects in children with autoimmune hepatitis (AIH), compared with oral corticosteroid therapy. Study design: The subjects consisted of nine children with AIH. Patients were divided into two groups. Group 1 was consisted of four patients who were treated with conventional oral prednisone therapy without m-PSL. Group 2 was consisted of five children who were treated with m-PSL as an induction therapy and maintained on oral prednisone. Results: The median duration needed for normalization of alanine aminotransferase was 285 days (range 18–617 days) in group 1 and 54 days (range 15–116 days) in group 2. The most frequent side effects were obesity and growth retardation. The Z-score in group 1 tended to be lower than in group 2 and body mass index in group 1 tended to be higher than in group 2. Conclusion: The m-PSL may become a safe and effective therapy for children with AIH. Further study is needed to establish the appropriate therapy for AIH in children. [Copyright &y& Elsevier]

The article presents a study that investigated the efficacy of proton pump inhibitor (PPI)-based triple eradication therapy in Japanese children suffering from Helicobacter pylori infection. Triple therapies consisting of a proton pump inhibitor and two antibiotics have been shown to be effective for eradicating Helicobacter pylori in children. The study also examined the influence of clarithromycin resistance on eradication success, ulcer healing, and clinical symptoms in Japanese children. Results of the study found that a PPI plus amoxicillin and metronidazole triple therapy is reasonably effective as the first-line eradication therapy in most Japanese children with Helicobacter pylori infection.

This paper introduces the guidelines for treatment of ulcerative colitis in children, created by the working group of the Japanese Society for Pediatric Gastroenterology, Hepatology and Nutrition (Chair: Yuichiro Yamashiro) and the Japanese Society for Pediatric Inflammatory Bowel Disease (IBD) (Chair: Akio Kobayashi). The ideas of the working group, with regard to the fundamental differences in medical treatment between children and adults, included: (1) for children, intensive medical treatment including appropriate systemic management is important during the acute phase of illness. (2) Treatment with steriods, which can cause growth disturbances, should not be continued for long periods of time. (3) Pulsed steriod therapy, selective removal of blood cells, and intravenous infusion of cyclosporin should be included in the therapeutic option for severe and fluminant cases. [ABSTRACT FROM AUTHOR]