Disease severity prognostication in primary sclerosing cholangitis: a validation of the Anali scores and comparison with the potential functional stricture.

Objectives: Our aim was twofold. First, to validate Anali scores with and without gadolinium (ANALI and ANALI) in primary sclerosing cholangitis (PSC) patients. Second, to compare the ANALIs prognostic ability with the recently-proposed potential functional stricture (PFS).

Spontaneous perforation of the common bile duct: an uncommon complication of primary sclerosing cholangitis.

Objectives: To describe the MR features and prognosis of patients with an uncommon complication of primary sclerosing cholangitis (PSC) characterized by a spontaneous perforation of the common bile duct (CBD) resulting in a peri-biliary collection and a pseudo-cystic appearance of the CBD.

Methods: A single-center cohort of 263 patients with PSC who had at least two MRIs between 2003 and 2022 and a minimum follow-up of 1 year was retrospectively analyzed. MRI data (characteristics of CBD perforation and MR features of PSC) and clinical data were assessed.

Focal loss of mural stratification as a radiological predictor for small bowel adenocarcinoma in Crohn's disease.

Aim: Patients with Crohn's disease (CD) are at higher risk of small bowel adenocarcinoma (SBA). We aimed to identify radiological predictors of SBA in CD.

Methods: We conducted a retrospective case-control study at two tertiary inflammatory bowel disease centers and identified CD patients diagnosed with SBA between 2003 and 2019.

Chyluria: non-enhanced MR lymphography.

Chyluria is an uncommon medical condition resulting from an abnormal communication between the abdominal lymphatic system and the urinary tract, which results in the presence of chyle in the urine, making it appear milky white. Proper diagnosis is demonstrated by the concentration of urinary lipids. Worldwide, chyluria is most commonly associated with the parasite Wuchereria bancrofti.

ERCP 2.0: Biliary 3D-reconstruction in patients with malignant hilar stricture.

Background: Endoscopic retrograde cholangiopancreatography (ERCP) for malignant hilar strictures is challenging. The correlation between Magnetic resonance cholangiopancreatography (MRCP) and per ERCP 2D fluoroscopic images is not obvious. The aim of this study was to evaluate the feasibility and potential usefulness of MRCP-based handmade biliary 3D reconstruction in this setting.

Protective potential of the gallbladder in primary sclerosing cholangitis.

Background & Aims: Gallbladder enlargement is common in patients with primary sclerosing cholangitis (PSC). The gallbladder may confer hepatoprotection against bile acid overload, through the sequestration and cholecystohepatic shunt of bile acids. The aim of this study was to assess the potential impact of the gallbladder on disease features and bile acid homeostasis in PSC.

JAK2V617F mutation drives vascular resident macrophages toward a pathogenic phenotype and promotes dissecting aortic aneurysm.

JAK2V617F mutation is associated with an increased risk for athero-thrombotic cardiovascular disease, but its role in aortic disease development and complications remains unknown. In a cohort of patients with myeloproliferative neoplasm, JAK2V617F mutation was identified as an independent risk factor for dilation of both the ascending and descending thoracic aorta. Using single-cell RNA-seq, complementary genetically-modified mouse models, as well as pharmacological approaches, we found that JAK2V617F mutation was associated with a pathogenic pro-inflammatory phenotype of perivascular tissue-resident macrophages, which promoted deleterious aortic wall remodeling at early stages, and dissecting aneurysm through the recruitment of circulating monocytes at later stages.

Quantitative magnetic resonance cholangiopancreatography metrics are associated with disease severity and outcomes in people with primary sclerosing cholangitis.

Background & Aims: People with primary sclerosing cholangitis (PSC) have a variable and often progressive disease course that is associated with biliary and parenchymal changes. These changes are typically assessed by magnetic resonance imaging (MRI), including qualitative assessment of magnetic resonance cholangiopancreatography (MRCP). Our aim was to study the association of novel objective quantitative MRCP metrics with prognostic scores and patient outcomes.

variant is associated with hepatobiliary MR abnormalities in people with low-phospholipid-associated cholelithiasis syndrome.

Background & Aims: The low-phospholipid-associated cholelithiasis (LPAC) syndrome is a recently described peculiar form of cholelithiasis associated with the ATP-binding-cassette subfamily B, member 4 () gene deficiency. The purpose of our study was to analyse the relationship between magnetic resonance (MR) features and the genetic status of in people with LPAC syndrome.

Methods: A total of 233 individuals with proven LPAC syndrome were enrolled between January 2003 and June 2018 in a retrospective single-centre study.