Endoscopic Management of Primary Sclerosing Cholangitis.

Primary sclerosing cholangitis (PSC) remains a rare but potentially devastating chronic, cholestatic liver disease. PSC causes obstruction of intra- and/or extra-hepatic bile ducts by inflammation and fibrosis, leading to biliary obstruction, cirrhosis and portal hypertension with all associated sequelae. The most dreaded consequence of PSC is cholangiocarcinoma, occurring in 10-20% of patients with PSC, and with population-based estimates of a 398-fold increased risk of cholangiocarcinoma in patients with PSC compared to the general population. We use the 4-D approach to endoscopic evaluation and management of PSC based on currently available evidence. After laboratory testing with liver chemistries and high-quality cross-sectional imaging with MRCP, the first D is Dominant stricture diagnosis and evaluation. Second, Dilation of strictures found during ERCP is performed using balloon dilation to as many segments as possible. Third, Dysplasia and cholangiocarcinoma diagnosis is performed by separated brushings for conventional cytology and fluorescence in situ hybridization (FISH), and consideration for direct cholangioscopy with SpyGlass™. Fourt and finally, Dosing of antibiotics is critical to prevent peri-procedural cholangitis. The aim of this review article is to explore endoscopic tools and techniques for the diagnosis and management of PSC and provide a practical approach for clinicians.

Endoscopic Ultrasound Evaluation of Portal Pressure.

Portal hypertension is a complex syndrome with multiple clinical manifestations that develop in a variety of conditions and diseases. The spectrum of portal hypertension manifestations is wide and dependent on the physiologic site of increased portal resistance (pre-, post-, and sinusoidal or intrahepatic), as well as the presence of hepatocellular dysfunction.

Early Detection of Pancreatic Cancer: Risk Factors and the Current State of Screening Modalities.

Pancreatic cancer (PC) is expected to become the second leading cause of cancer-related mortality in the United States within the next decade. Patients often present at late stages of the disease, when they become symptomatic; in many cases, these patients have unresectable disease that is associated with a poor prognosis. Considering the low incidence of PC in the general population, routine screening of average-risk individuals is not feasible and not recommended. Individuals with familial germline mutations or familial PC are at higher risk of developing PC. Improving detection of PC at an earlier stage entails the recognition of high-risk individuals who may benefit from a long-term screening program. This article identifies patients who may be at increased risk of developing PC, discusses PC screening recommendations, and compares imaging-based modalities and biomarkers for early detection of PC.

Small adenocarcinomas of the esophagogastric junction: association with intestinal metaplasia and dysplasia.

OBJECTIVES: Intestinal metaplasia in Barrett's esophagus predisposes to esophageal adenocarcinoma. Intestinal metaplasia of the cardia is a common finding in persons without cancer. Many adenocarcinomas of the esophagogastric junction are large enough to obliterate any underlying intestinal metaplasia. To estimate how often adenocarcinoma of the esophagogastric junction arises in intestinal metaplasia, we studied small adenocarcinomas of the esophagogastric junction. METHODS: Resection patients had adenocarcinomas 2 cm or smaller, within 2 cm of the esophagogastric junction. Age- and sex-matched controls had resection for squamous carcinoma. Saved and new histological slides from the esophagogastric junction were examined, with additional stains. RESULTS: Intestinal metaplasia was found in 86% (19/22) of adenocarcinoma cases, versus 32% (7/22) of controls (p < 0.001). Intestinal metaplasia with high or low grade dysplasia was associated with 64% (14/22) of adenocarcinomas and with 5% (1/22) of controls (p < 0.001). Excluding four cases with long and three with short Barrett's esophagus, 80% (12/15) of adenocarcinomas had associated intestinal metaplasia, 53% (8/15) with dysplasia. Most adenocarcinoma cases had the incomplete type of intestinal metaplasia with a Barrett type cytokeratin 7/20 staining pattern. Helicobacter pylori were seen in one adenocarcinoma and five control cases. CONCLUSIONS: Most adenocarcinomas of the esophagogastic junction arise in the background of intestinal metaplasia, sometimes in an endoscopically visible Barrett's esophagus, more often in small areas of intestinal metaplasia of the cardia. In cases of adenocarcinoma, the intestinal metaplasia resembled that found in Barrett's esophagus, and was not associated with H. pylori.

Clinical distinctions and pathogenic implications of type 1 autoimmune hepatitis in Brazil and the United States.

BACKGROUND/AIMS: Type 1 autoimmune hepatitis has a strong genetic predisposition that varies among different ethnic groups. Our aims were to determine if the clinical manifestations differed between patients with type 1 autoimmune hepatitis from Brazil and the United States and if classical disease could be associated with region-specific susceptibility markers. METHODS: The clinical manifestations and genetic risk factors of 161 patients from the United States were compared to those of 115 patients from Brazil. RESULTS: The patients from Brazil had earlier disease onset, lower frequency of concurrent immune diseases, higher serum levels of aspartate aminotransferase and gamma-globulin, greater occurrence of smooth muscle antibodies, and lower frequency of antinuclear antibodies than the patients from the United States. Human leukocyte antigen (HLA) DR13 and DRB1*1301 occurred more commonly in the Brazilian patients and HLA DR4 less often. Normal subjects from each country had similar frequencies of HLA DR13 and DR3. CONCLUSIONS: Type 1 autoimmune hepatitis in Brazil has different features at presentation than the disease in Caucasoid patients from the United States, and it is associated with HLA DR13. Background populations in each country have similar frequencies of HLA DR13 and DR3, and region-specific etiologic factors may determine the HLA association.