PTEN deficiency induces an extrahepatic cholangitis-cholangiocarcinoma continuum via aurora kinase A in mice.

Background & Aims: The PTEN-AKT pathway is frequently altered in extrahepatic cholangiocarcinoma (eCCA). We aimed to evaluate the role of PTEN in the pathogenesis of eCCA and identify novel therapeutic targets for this disease.

Methods: The Pten gene was genetically deleted using the Cre-loxp system in biliary epithelial cells.

A clinical review of congenital hepatic fibrosis diagnosed in adulthood: presentation, complications, and outcomes.

Introduction And Objectives: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described.

Hereditary transthyretin amyloidosis presenting with spontaneous periorbital purpura: a case report.

Background: Amyloidosis is a systemic disorder of abnormal protein folding and deposition resulting in a range of symptoms including neuropathy, heart failure, renal disease, and dermatologic findings. The two most common types of amyloidosis that affect the heart are transthyretin (ATTR) amyloidosis and light chain (AL) amyloidosis, which vary in clinical presentation. Skin findings such as periorbital purpura are considered more specific for AL amyloidosis.

Non-invasive detection of cardiac allograft rejection among heart transplant recipients using an electrocardiogram based deep learning model.

Aims: Current non-invasive screening methods for cardiac allograft rejection have shown limited discrimination and are yet to be broadly integrated into heart transplant care. Given electrocardiogram (ECG) changes have been reported with severe cardiac allograft rejection, this study aimed to develop a deep-learning model, a form of artificial intelligence, to detect allograft rejection using the 12-lead ECG (AI-ECG).

Methods And Results: Heart transplant recipients were identified across three Mayo Clinic sites between 1998 and 2021.

Hepatoportal Sclerosis-A Clinicopathologic Review of 28 Cases.

Background And Aims: The aim of this study was to review a large series of cases with hepatoportal sclerosis (HPS) as a pathologically recognizable entity in liver tissue specimens and describe the associated clinical and radiographic manifestations, along with the outcomes of this entity.

Methods: Data were collected through a retrospective chart review.

Results: Twenty-eight patients were identified that had pathologically defined HPS.

Angiosarcoma: A Rare Malignancy Linked to Chemical Exposures.

Angiosarcoma is an exceptionally rare malignancy that accounts for less than 1% of all sarcomas. This case describes a 90-year-old male veteran who presented with a hematoma from a traumatic head injury. This then progressed to an ulcerated bleeding lesion that measured 2.

Normal ESR, CRP and Platelet Count in Giant Cell Arteritis and Polymyalgia Rheumatica: A Diagnostic Conundrum.

Unlabelled: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are both rheumatological diseases of the elderly with a strong association with each other and which rarely present with normal inflammatory markers. Here we present the case of a 61-year-old Caucasian woman who had typical symptoms of both diseases. At the time of presentation, her blood work showed normal inflammatory markers, but because of the high clinical suspicion for GCA, a temporal artery biopsy was done which was positive for giant cells and disruption of the internal elastic lamina.

Gout gone awry: The importance of proper diagnosis.

Gout is the best-known type of arthritis with a prevalence of 1%-3% in the western world (Therapeutic Advances in Musculoskeletal Disease, 6, 2014 and 131; Journal of Advanced Research, 8, 2017 and 495). Although it is well understood, there is growing evidence of the misdiagnosis of gout from other forms of arthritis. These errors lead to delay in accurate diagnosis (Journal of Advanced Research, 8, 2017 and 495).

Umbralisib-Induced Immune-Mediated Colitis: A Concerning Adverse Effect of the Novel PI3Kδ/CK1ε Inhibitor.

Novel chemotherapeutic agents are developed to treat recurrent/relapsed lymphoid malignancies. Umbralisib, a novel phosphatidylinositol 3-kinase inhibitor with a selective isoform binding, has shown an improved efficacy and safety profile in clinical trials. Immune-mediated colitis, a frequently observed dose-limiting adverse event of phosphatidylinositol 3-kinase inhibitors, has been mostly observed at supratherapeutic doses in the trials, with grade 1 or 2 diarrhea being the most common adverse event at the therapeutic dose (800 mg PO QD).

Creutzfeldt-Jakob disease as a cause of dementia.

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder belonging to the family of transmissible spongiform encephalopathies. The disease is believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. Our patient is an 84-year-old Caucasian man who presented to the geriatric clinic for evaluation of short-term memory loss and decreased concentration which started 3 months prior to initial evaluation.

Sequential Protocol Biopsies Post-Liver Transplant From Donors With Moderate Macrosteatosis: What Happens to the Fat?

The number of steatotic deceased donor livers encountered has continued to rise as a result of the obesity epidemic. Little is known about the histological characteristics of moderately macrosteatotic livers over time in the recipient following liver transplantation (LT). All recipients undergoing LT at Mayo Clinic Florida with donor livers with moderate macrosteatosis (30%-60%) from 2000-2017 were identified (n = 96).

Evaluating the Adoption of Laboratory Practice Guidelines.

Context.—: To date, the College of American Pathologists (CAP) has developed 17 laboratory practice guidelines (LPGs) including updates. In 2013, the CAP was awarded a 5-year cooperative agreement grant from the United States Centers for Disease Control and Prevention to increase the effectiveness of LPGs.

Does Donor Allograft Microsteatosis Matter? Comparison of Outcomes in Liver Transplantation With a Propensity-Matched Cohort.

It has been suggested that microsteatosis does not negatively impact graft survival following liver transplantation (LT). The present study represents the largest series on donor livers with significant microsteatosis and investigates the impact of microsteatosis on perioperative factors such as postreperfusion syndrome (PRS), early allograft dysfunction (EAD), and postoperative renal dysfunction. Clinical outcomes of all patients undergoing LT with donor livers with isolated microsteatosis (≥30%; n = 239) between 2000 and 2017 were compared with a propensity score-matched cohort of patients undergoing LT with donor livers with no steatosis (n = 239).

Antithymocyte Globulin Use for Corticosteroid Nonresponsive Rejection After Liver Transplantation.

Background And Aims: Acute cellular rejection after liver transplantation usually responds to intravenous corticosteroids, yet some episodes are corticosteroid-nonresponsive. We report our experience using antithymocyte globulin therapy for corticosteroid-nonresponsive acute cellular rejection in liver transplant recipients.

Methods: From January 1, 2002 to January 1, 2010, 1436 patients underwent 1548 liver or liver with other organ transplantations at our institution.

Warfarin-induced spontaneous iliopsoas hematoma - An unusual complication.

Warfarin is a commonly prescribed anticoagulant with a narrow therapeutic window and high potential for serious complications. Spontaneous psoas hematoma is a rare complication of warfarin which can result in significant neurological deficits.

Liver transplantation for intrahepatic cholangiocarcinoma.

Although hepatocellular carcinoma (HCC) has become a common indication for liver transplantation (LT), intrahepatic cholangiocarcinoma (ICC) and combined hepatocellular carcinoma-cholangiocarcinoma (cHCC-CCA) are historically contraindicated due to their aggressive behavior. On the basis of recent experiences, some groups have proposed a clinical trial investigating the role of LT for patients with early cholangiocarcinoma (CCA), defined as a single lesion ≤ 2 cm. The purpose of this study is to assess the clinicopathologic features and outcomes following LT for patients who were initially diagnosed with HCC and subsequently found to have either ICC or cHCC-CCA on explant.