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Auto-inflammatory Diseases (AIDs) are a group of diseases with a strong genetic component, inducing an inappropriate activation of innate immunity. The patients with pediatric onset will face the transitional care (TC) from a pediatrician to an adult care setting, during the critical phase of the adolescence. That implies a risk of failure and drop out, due to the different approach of pediatrician compared to the adult doctor. To describe the model of TC for AIDs from a paediatric to adult centre of two hospitals in Rome, and to pointing out the different steps emerged from specific experiences.

Key Clinical Message: The perioperative control of ammonia, reduction of stress, and administration of drugs tolerated in type 1 citrullinemia and Brugada pattern allowed the successful and uneventful management of general anesthesia in the study patient. The aim of this study was to report the targeted perioperative management of general anesthesia (GA) adopted for dental extractions in a rare patient with type 1 citrullinemia and Brugada pattern. A male, Caucasian, adult type 1 citrullinemia patient needed dental extractions under GA. The medical history showed neurodevelopmental impairment, growth retardation, epilepsy, and a Type 2 Brugada electrocardiographic pattern in the second precordial lead. The authors focused the anesthesiologic protocol on the prevention of hyperammonemia and fatal arrhythmias. Changes in diet and 10% glucose solution administration prevented protein catabolism due to the fasting period (ammonia was 44 μmol/L preoperatively and 46 μmol/L postoperatively; glycemia was 120 g/dL preoperatively and 153 g/dL postoperatively). The patient received a continuous electrocardiogram, noninvasive blood pressure, pulse oximeter, entropy monitoring, train‐of‐four monitoring, and external biphasic defibrillator pads. Midazolam, remifentanil, and dexamethasone were administered for pre‐anesthesia; thiopental and rocuronium for induction; remifentanil and desflurane for maintenance; sugammadex for decurarization. After the intraligamentary injection of lidocaine 2% with epinephrine 1:100,000 for local anesthesia, the patient developed a transient Type 1 Brugada pattern that lasted a few minutes. The whole procedure lasted 30 min. The patient's discharge to ward occurred 3 h after the end of GA. The perioperative management of ammonia, reduction of stress, and administration of drugs tolerated in Type 1 citrullinemia and Brugada pattern allowed the successful and uneventful administration of GA in the study patient. [ABSTRACT FROM AUTHOR]

Aim: The gold standard of care for pancreatic adenocarcinoma is the integrated treatment of surgery and chemotherapy (ChT), but about 50% of patients present with unresectable disease. Our study evaluated the efficacy in terms of local control, survival and safety of stereotactic body radiation therapy (SBRT) in locally advanced pancreatic cancer (LAPC). Methods: A retrospective study (STEP study) analyzed patients with LAPC treated with a dose of 45 Gy in 6 fractions. Local control (LC), distant progression free survival (DPFS), overall survival (OS) and toxicity were analyzed according to the Kaplan-Meier method. Results: A total of 142 patients were evaluated. Seventy-six patients (53.5%) received induction ChT before SBRT. The median follow-up was 11 months. One-, 2- and 3-year LC rate was 81.9%, 69.1% and 58.5%. Median DPFS was 6.03 months; 1- and 2-year DPFS rate was 19.9% and 4.5%. Median OS was 11.6 months and 1-, 2- and 3-year OS rates were 45.4%, 16.1%, and 9.8%. At univariate analysis, performed by the log-rank test, age < 70 years (p = 0.037), pre-SBRT ChT (p = 0.004) and post-SBRT ChT (p = 0.019) were associated with better OS. No patients experienced G3 toxicity. Conclusion: SBRT represents an effective and safe therapeutic option in the multimodal treatment of patients with LAPC in terms of increased LC. When SBRT was sequentially integrated with ChT, the treatment proved to be promising in terms of OS as well. [ABSTRACT FROM AUTHOR]

For resectable pancreatic tumors surgery is the gold standard of care, however more than 50% of patients are unresectable at the time of diagnosis. In patients with bordeline resectable/locally advanced pancreatic cancer, the integration of chemotherapy and chemo-radiation treatment, also as neoadjuvant treatment, is the current therapeutic option. The standard fractionation of the radiotherapy treatment is associated with a significant toxicity rate and with a disappointing overall survival. Higher local control related to the high doses employed, short overall treatment time and sequential integration with systemic therapy, represent the crucial advantages of SBRT over conventional CRT. Methods: After multidisciplinary board evaluation, patients with inoperable pancreatic cancer with maximum tumor diameter ≤ 5cm, N0 and M0 were treated with SBRT. Prescription dose was 45Gy in 6 fractions. Radiotherapy treatment plan was based on 3 phases 4D contrast enhanced simulation CT-scan and if necessary image registration of PET-CT or MRI. ITV of the lesion and Organs at risk were contoured on the simulation CTscan in all phases of the breath. Primary end-point was freedom from local progression (FFLP). Secondary endpoints were overall survival, progression-free survival, and toxicity. Local control was defined according to RECIST v1.1 criteria. Acute and late toxicity was scored according to the NCI Common Terminology Criteria for Adverse Events (CTCAE) v4.0. Results: Between January 2011 and June 2021, 142 patients with pancreatic cancer were treated with SBRT at Humanitas Cancer Center. Median age was 71 years (range 41-91 years). 14 patients (9.8%) presented a borderline resectable disease and 128 patients (90.2%) a locally advanced disease. 76 patients (53.5%) received induction CHT before SBRT, for a median time of 5 months (range 3 - 10 months). FFLP was 81.9%, 69.1% and 58.5% at 1, 2 and 3 years, respectively. Median OS was 11.6 months and 1-2-3 years OS rates were 45.4% (95%CI 37.0 – 53.5), 16.1% (95%CI 10.5 – 22.9) and 9.8% (95%CI 5.4 – 15.7), respectively. CHT administered before SBRT (HR 0.59, 95%CI 0.41–0.84, p=0.004) and CHT post-SBRT (HR 0.63, 95%CI 0.43–0.92, p=0.019) were significantly correlated with OS. No case of Grade 3 toxicity was detected. Conclusions: SBRT is an effective and safe local therapy for selected patients with inoperable pancreatic cancer. Since OS was improved in patients pre-treated with induction CHT, our results suggest that the treatment for these patients must be multimodal and the stereotactic treatment may be a promising therapeutic option in this multi-modality context. [ABSTRACT FROM AUTHOR]

Reconstruction of extrahepatic bile ducts is a staple procedure of HPB surgery. The current standard for most cases is a nonanatomical bilioenteric reconstruction, a satisfactory option for the majority of patients. However, it cannot be used for a small number of selective cases (short bowel syndrome, severe abdominal adhesions), where an anatomical reconstruction with or without an interponate can be used. This review summarizes current knowledge about tissue and material usage for experimental and clinical anatomical bile duct reconstruction in the last 100 years. A Pubmed database was searched for published articles about anatomical extrahepatic bile duct reconstruction in experimental and clinical settings ranging from 1920 to 2022. To date, the truly optimal interponate material has not yet been found. However, evidence reveals important properties of such material, most importantly its biodegradability and neovascularization in the recipient's body. The role of internal bile duct stenting for anatomical reconstruction seems important for the outcome. Anatomical reconstruction of extrahepatic bile ducts is an uncommon but usable technique in unique cases when a nonanatomical reconstruction cannot be done. The optimal properties of interponate material for anatomical bile duct reconstruction have been more clarified, although further research is required., (© 2024 The Author(s). Journal of Hepato‐Biliary‐Pancreatic Sciences published by John Wiley & Sons Australia, Ltd on behalf of Japanese Society of Hepato‐Biliary‐Pancreatic Surgery.)

Introduction: Recently, medical interest has been growing in SARS-CoV-2 infection and its multiorgan involvement, including the liver. Up until now, a few reports have described autoimmune hepatitis (AIH) triggered by SARS-CoV-2 infection, but no data are available about the specific liver inflammatory infiltrate and cluster of differentiation. We report a case of AIH triggered by SARS-CoV-2 infection, with a particular focus on its histological and mainly immunohistochemical features., Case Description: A 60-year-old man, with a history of paucisymptomatic SARS-CoV-2 infection that occurred one month earlier, was admitted for alterations of hepatocellular necrosis and cholestasis indexes. He completed vaccination for SARS-CoV-2 a year earlier. The serologies for hepatotropic viruses were negative. The anti- smooth muscle antibodies (ASMA) and antinuclear antibodies (ANA) results were positive. Anti-liver kidney microsome (anti-LKM) antibodies and antimitochondrial (AMA) were negative. By liver biopsy, haematoxylin-eosin staining highlighted severe portal inflammation with a rich CD38+ plasma cell component, while immunohistochemical staining showed low cell CD4+ count and prevalence of CD8+ and CD3+. After biopsy, the patient started an immunosuppressant regimen, with benefit., Discussion: We can conclude that the patient developed a type 1 AIH triggered by SARS-CoV-2 infection. The presence of CD8 T-cells at immunohistochemical examination suggests different mechanisms from classic AIH. Similar cases are described after AIH triggered by SARS-CoV-2 vaccination., Conclusion: The AIH after SARS-CoV-2 infection developed by the patient showed a histological picture similar to a classic AIH for the abundant presence of plasma cells, and immunohistochemical features similar to those described after SARS-CoV-2-vaccination., Learning Points: Recently, medical interest has been growing in SARS-CoV-2 infection and its multiorgan involvement, including the liver. Underlying mechanisms are not still clear, more likely consisting of an inflammatory and immune mediated process rather than a direct cytopathic damage.Our report describes a rare case of type 1 AIH triggered by SARS-CoV-2 infection, showing a peculiar histological pattern, different from classic AIH, conversely similar to AIH triggered by SARS-CoV-2 vaccination.The mechanisms underlying liver involvement in SARS-CoV-2 infection are still under investigation. Further studies should be encouraged to improve understanding on this focus and to support physicians in its management., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2024.)