Metastatic Signet Ring Cell Adenocarcinoma Manifesting as Chronic Leg Pain.

Signet ring cell carcinoma, a type of gastrointestinal system-related cancer, rarely metastasizes to the skeletal muscle. We present signet ring-cell carcinoma in a 28-year-old man who presented with left lower extremity pain and swelling. Imaging showed thickening of the distal esophagus, intestines, and bladder wall. Endoscopy revealed friable gastric mucosa and stenosis in the ascending colon, but biopsies were unrevealing. Leg muscle biopsy showed metastatic adenocarcinoma with focal signet ring features. Carcinoembryonic antigen and cancer antigen 19-9 were elevated. A gastrointestinal primary tumor was suspected. Our case urges clinicians to consider this rare cancer in patients presenting with skeletal muscle mass.

Liver transplant recipient with respiratory failure due to pulmonary fibrosis related to telomere disease requiring lung transplantation.

Short telomere syndrome (STS) is characterized as multiorgan dysfunction presenting with unexplained cytopenias, cryptogenic cirrhosis and pulmonary fibrosis. We present a liver transplant recipient that gradually developed hypoxic respiratory failure attributed to idiopathic pulmonary fibrosis associated telomere disease that culminated in a successful single lung transplantation.

Cholangitis Lenta: What Hepatologists Need to Know.

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Direct-Acting Antiviral Treatment of Patients with Hepatitis C Resolves Serologic and Histopathologic Features of Autoimmune Hepatitis.

Patients with hepatitis C virus (HCV) often have elevated serum markers and histologic features of autoimmune hepatitis (AIH). We evaluated an HCV-positive (HCV+) study group that had elevated serum markers of AIH before starting direct-acting antiviral (DAA) therapy (n = 21) and compared them to an HCV+ control group that did not have laboratory studies suggesting AIH (n = 21). Several patients in the study (17/21) and control (11/21) groups had liver biopsies before DAA treatment, and many were biopsied due to elevated serum markers of AIH. Evaluation of pre-DAA treatment liver biopsies showed histologic features suggestive of AIH in 64.7% (11/17) of the study group and 45.5% (5/11) of the control group. Patients who were HCV+ with elevated serum markers of AIH had significantly increased hepatitis activity (P < 0.001) and slightly increased fibrosis stages (P = 0.039) in their pretreatment liver biopsies compared to controls. We hypothesized that the elevated serum markers and histologic features of AIH would resolve following DAA treatment. Serum markers of AIH in the study group began decreasing by 6 months posttreatment, and 52.4% (11/21) had complete resolution. Alanine aminotransferase levels significantly decreased into the normal range for all patients (21/21). Even patients that had persistence of serum markers of AIH after DAA treatment had normal transaminases. Six patients from the study patient group and 4 patients from the control group had follow-up liver biopsies after DAA treatment, and all biopsies showed resolution of the histologic features of AIH. Conclusion: The majority of HCV+ patients that have serum markers and/or histopathologic features of AIH should initially be treated with DAA.

Evaluation and management of variceal bleeding.

Acute variceal bleeding is one of the most fatal complications of cirrhosis and is responsible for about one-third of cirrhosis-related deaths. Therefore, every effort should be made to emergently resuscitate the patients, start pharmacotherapy as soon as possible and do endoscopic therapy in a timely manner. Despite the recent advances in treatment, mortality rate is still high. We provide a comprehensive review of evaluation and management of variceal bleeding.