A Case of Paradoxical Arthralgia Following Anti-TNF Monoclonal Antibody Administration in a Patient With New-Onset Pediatric Crohn's Disease.

Anti-TNF antibodies have become a first-line therapy in moderate-to-severe inflammatory bowel diseases. However, there may be some rare paradoxical events and those affecting joints causing severe symptoms need a scrupulous differential diagnosis. When these events occur, it may be necessary to discontinue treatment and shift to another drug class. Herein, we report the case of a 15-year-old boy affected by Crohn's disease, who developed a paradoxical reaction after the second dose of infliximab. Clinical remission was achieved shifting to budesonide and azathioprine and continuing maintenance therapy with azathioprine alone. To date, no other paradoxical events have occurred.

Inadequate Hospital Practices to Prevent Mother-to-Child Transmission of Hepatitis B Virus Infection: A European Survey.

OBJECTIVES: Prevention of vertical transmission of hepatitis B virus (HBV) infection is crucial to eliminate viral hepatitis as a major public health threat by 2030. We aimed to assess the current hospital policies and practices implemented before, at, and after birth, and to evaluate potential barriers to the full application of international guidelines. METHODS: A web-based survey was supported by PENTA Foundation and distributed across Europe from October to December 2021. RESULTS: Overall, 76 centers with delivery departments completed the survey. Hepatitis B surface antigen (HBsAg) maternal screening is performed in the first trimester of pregnancy in 53% of the centers and in the third in 46%. HBsAg positive pregnant women are tested for serologic HBV markers and HBV-DNA in 78% and 63% of the departments; 38% of the HBeAg positive women with high HBV-DNA levels are treated during the last trimester of pregnancy. At birth, 91% of the departments administer HBV vaccine to infants born to HBsAg positive mothers within 12 hours of birth; 74% test women with unknown HBsAg status and 78% of them wait for the maternal testing results before administering HBV vaccine to their newborns. After birth, 47% of the departments provide postvaccination serological testing for infants born to HBsAg positive mothers. The timing of the HBV vaccine schedule varies greatly. CONCLUSIONS: There is significant heterogeneity in the hospital policies and correlated procedures. The implementation of a multidisciplinary clinical pathway is a must if a stronger connection between the prenatal, perinatal, and postnatal phases is to be established.

COVID-19 as a Trigger of Acute-on-Chronic Hepatitis B Presenting With Undetectable INR Due to Hypercoagulability in a 16-Year-Old Girl.

In patients with SarS-CoV2 and chronic Hepatitis B (HBV) co-infection liver injury is associated with a worse prognosis. We report a case of acute chronic liver failure (ACLF) with encephalopathy due to HBV reactivation during COVID-19 with undetectable INR. Thromboelastography showed a profile consistent with a prothrombotic state so INR was not a reliable marker of liver function until plasma infusion. After plasma infusion, indeed, an imbalance of hepatic function was shown by an underlying INR prolongation that was consistent with an ACLF.

New case of syncytial giant-cell variant of hepatocellular carcinoma in a pediatric patient with HNF1B deficiency: does it fit with the syndrome?

BACKGROUND: Hepatocyte nuclear factor 1B (HNF1B) is a member of the homeodomain-containing family of transcription factors located on 17q12. HNF1B deficiency is associated with a clinical syndrome (kidney and urogenital malformations, maturity-onset diabetes of the young, exocrine pancreatic insufficiency) and to an underdiagnosed liver involvement. Differently from HNF1A, the correlation between hepatocellular carcinoma (HCC) and germline HNF1B deficiency has been poorly evaluated. CASE REPORT: Here, we report a novel case of a syndromic HNF1B-deficient paediatric patient that developed HCC with unique histopathological features characterised by neoplastic syncytial giant cells, which was observed only in one additional case of paediatric cholestatic liver disease of unknown origin. CONCLUSIONS: Our case highlights the influence of HNF1B deficiency in liver disease progression and its putative association with a rare yet specific HCC histotype. We hypothesised that HCC could be secondary to the repressive effect of HNF1B variant on the HNF1A transcriptional activity.

Timely Birth Dose Vaccine to Prevent Vertical Transmission of Hepatitis B: A Single Center Experience on the Road to the WHO Elimination Goals in Italy.

Italy was one of the first industrialized countries to implement a program of routine vaccination against hepatitis B virus (HBV) infection. However, currently, no HBV vaccine is administered at birth if the screened mother is HBsAg negative, whilst babies born to HBsAg positive mothers are given vaccine and hepatitis B immunoglobulin, within 12-24 post-delivery hours. A single center retrospective analysis of policies and practices to prevent mother-to-child transmission of HBV was carried out, to evaluate their adherence to HBV care guidelines. Paired maternal-infant medical records for consecutive live births, between January 2015 and December 2019, were reviewed at the AOU Città della Salute e Scienza di Torino, where a total of 235/35,506 babies (0.7%) were born to HBsAg positive mothers. Markers of active viral replication, i.e., HBV DNA level and/or HBeAg, were reported in only 66/235 (28%) of the mothers' medical records. All newborns had immunoprophylaxis at birth: 61% at <12 h, 31% between 12 and 24 h, 7% between 24 and 36 h and 1% at >36 h. In 2019, two cases of vertical HBV transmission occurred, despite timely immunoprophylaxis, as their mothers' viral load was detected too late for antiviral prophylaxis. Missed early identification of pregnant women with high viremia levels or late vaccinations may contribute to perinatal HBV infection. Immunoprophylaxis should be given to babies born to HBsAg positive mothers at the latest within 12 h. In Italy, policies aimed at achieving the WHO 2030 goal of eliminating viral hepatitis should be further implemented.