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Background: Primary sclerosing cholangitis (PSC) is an immune-mediated disease that has an unfavorable prognosis and needs a liver transplant (LT). The aim of this paper was to show the usefulness of the Majoie classification on magnetic resonance cholangiopancreatography (MRCP) images in assessing the prognosis in adult patients with PSC. Methods: Our work presents a retrospective monocentric study performed on 64 adult patients with PSC of the large bile ducts. Two radiologists evaluated the MRCP of diagnosis and calculated MRCP scores using the Majoie classification. Liver-related outcome (LT or liver-related death) was marked as a primary endpoint. Results: Univariate analysis showed that patients with more severe lesions (sum score of intrahepatic and extrahepatic ducts > 3) had a lower age at diagnosis, of 37.2 years, complicated with liver cirrhosis (53.1% of patients) and recurrent cholangitis (28.1%) p < 0.05, without significant differences in mortality, association with IBD or LT. Concordance analysis between MRCP prognostic scores and progression to a PSC-related event showed a moderate relationship (c-statistic 0.662), and a good AUROC was observed for the UKPSC score (0.893) and the MRS (0.936). Conclusions: In the study, we observed a good correlation between the imaging scores based on the Majoie classification and the evolution of the patients. These scores were outperformed by the UKPSC, MRS, and PREsTo clinical models. Their utility was best in predicting recurrent cholangitis. [ABSTRACT FROM AUTHOR]

Introduction. Hereditary colorectal cancer (HCRC) syndromes represent a relatively diverse group of disorders that exhibit different patterns of inheritance. We aim to create the first registry of this type for this category of patients in order for them to have access to better healthcare and to give them a new perspective Keyword: colorectal cancer, hereditary syndromes, cancer registry Materials and Methods. We started collecting data since August 2021. We made an extensive examination consisting of personal infromation and important clinical data. All patients introduced in the study had lower and upper GI examinations performed, and also CT exams when indicated by guidelines. For Lynch Syndrome we used the Amsterdam and Bethesda criteria, while for familial adenomatous polyposis syndrome we combined the colonoscopy results with family history. We also collected family information in order to be able to perform screening testing for them depending on their respective syndrome. Results. We managed to introduce 26 patients that have either a genetic, histological or clinical diagnosis. Out of these, some are only family members that haven't been genetically tested and aren't diagnosed yet, but still have to be followed. We have 22 index patients, and are currently actively following other family members at high risk. We have 6 Lynch Syndrome families, 2 Peutz-Jeghers families, 3 Attenuated FAP Syndrome families, 2 MAP families, 7 FAP families and one Juvenile polyposis syndrome family with a sex distribution male to female of 9:17, (34.61% male, 65.39% female). Both patients with MAP have had genetic testing done, and also the Juvenile polyposis patient. Currently, family members are being introduced into the study in order to perform screening examinations according to guidelines and create an efficient timetable that can help prevent complications and diagnosis of advanced tumours. Conclusion. While challenging, it is clear that there is an urgent need for an electronic registry in order to offer this category of pacients a better life expectancy and a better quality of life. [ABSTRACT FROM AUTHOR]