Hereditary pancreatitis (HP) is a rare cause of acute, recurrent acute, and chronic pancreatitis. It may present similarly to other causes of acute and chronic pancreatitis, and often there has been a protracted evaluation prior to the diagnosis of HP. Since it was first described in 1952, multiple genetic defects that affect the action of digestive enzymes in the pancreas have been implicated. The most common mutations involve the PRSS1, CFTR, SPINK1, and CTRC genes. New mutations in these genes and previously unrecognized mutations in other genes are being discovered due to the increasing use of next-generation genomic sequencing. While the inheritance pathways of these genetic mutations may be variable and complex, sometimes involving coinheritance of other mutations, the clinical presentation of patients tends to be similar. Interactions with environmental triggers often play a role. Patients tend to present at an early age (prior to the second decade of life) and have a significantly increased risk for the development of pancreatic adenocarcinoma. Patients with HP may develop sequelae of chronic pancreatitis such as strictures and fluid collections as well as exocrine and endocrine insufficiency. Management of patients with HP involves avoidance of environmental triggers, surveillance for pancreatic adenocarcinoma, medical therapy for endocrine and exocrine insufficiency, pain management, and endoscopic or surgical treatment for complications. Care for affected patients should be individualized, with an emphasis on early diagnosis and multidisciplinary involvement to develop a comprehensive treatment strategy.

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INTRODUCTION: Little is known about the additive yield of wide-area transepithelial sampling with computer-assisted three-dimensional analysis (WATS-3D) after a thorough examination with advanced imaging. The aim was to evaluate the adjunctive yield of WATS-3D after advanced imaging. METHODS: This is an observational cohort study from January 2017 to December 2018 for consecutive patients who underwent an examination that consists of high-definition white light endoscopy (HDWLE), narrow-band imaging (NBI), volumetric laser endomicroscopy (VLE), and Seattle protocol (SP) biopsies (collectively termed HDWLE-NBI-VLE-SP examination). Raised lesions were removed by endoscopic resection. Areas suspicious for dysplasia on NBI and VLE were biopsied. This was followed by random biopsies and WATS-3D brush biopsies. RESULTS: One hundred thirty-eight cases were included in this study. Thirty-five cases (25% of the total) were identified as some degree of dysplasia on the HDWLE-NBI-VLE-SP examination. Adjunctive use of WATS-3D yielded an additional 12 new cases of dysplasia (9 with crypt dysplasia and 3 with low-grade dysplasia [LGD]), for added yield of 34.3% (=12/35, 95% confidence interval 14.6%–62.2%). When restricting the analysis to LGD and higher, 21 dysplastic cases (15% of the total cases) were identified by HDWLE-NBI-VLE-SP, while WATS-3D found 4 additional new cases (3 with LGD and 1 with high-grade dysplasia) for an added yield of 19% (=4/21, 95% confidence interval 0.6%–45.7%). DISCUSSION: The addition of WATS-3D to an already thorough examination with HDWLE-NBI-VLE-SP may increase the yield of dysplasia detection.

ObjectiveVolumetric laser endomicroscopy (VLE) is an advanced imaging modality used in Barrett’s oesophagus (BE) to help identify dysplasia in the oesophagus. VLE criteria exist for oesophageal dysplasia but not for dysplasia in the gastric cardia. The aim of this study was to determine if there are in vivo VLE features that can predict gastric cardia dysplasia in BE.DesignThis was a single-centre observational cohort study from August 2016 to August 2018. Patients were included if they had BE, were undergoing a VLE exam as standard of care, and had a suspicious target laser marked at the gastric cardia. The following VLE features were correlated to histology to determine if an association existed between histology subtype and VLE feature: wide crypts, irregular surface, one large isolated gland, multiple glands, and complex glands.ResultsA total of 110 in vivo gastric cardia targets in 77 patients with BE were analysed. The following abnormalities were identified: 61 wide crypts, 34 isolated glands, 16 irregular surfaces, 15 multiple glands, and 11 complex glands. Complex glands were the only VLE feature that correlated to any histology subtype. They were present in 71% of targets with high-grade dysplasia (HGD), T1a cancer or T1b cancer and had a sensitivity, specificity, and accuracy of 71%, 99%, and 85%, respectively. Of the 10 patients with complex glands on VLE and HGD/cancer on histology, 4 had a normal-appearing mucosa (40%) on endoscopy.ConclusionIdentification of complex glands on VLE may aid in detection of HGD or early cancer in the gastric cardia in BE. This is particularly important, as dysplasia at the gastric cardia can be difficult to see endoscopically.

This study suggests that tobacco exposure is independently associated with pancreatic exocrine insufficiency in patients without a prior diagnosis of pancreatic disease. Tobacco exposure seems to have greater detrimental effects on pancreatic function than alcohol in this population.

INTRODUCTION: Wide-area trans-epithelial sampling with computer-assisted three-dimensional analysis (WATS-3D) has been used for dysplasia detection in endoscopic surveillance of Barrett’s esophagus (BE). Little is known about the additive yield of WATS after a thorough exam with advanced imaging that consists of high definition white light endoscopy (HDWLE), narrow band imaging (NBI), volumetric laser endomicroscopy (VLE) with laser marking, and Seattle protocol biopsies (collectively termed HDWLE-NBI-VLE-SP exam). The aim of this study was to evaluate the adjunctive yield of WATS-3D after an HDWLE-NBI-VLE-SP exam. METHODS: This is a single center observational cohort study from January 2017 to December 2018. Charts were abstracted for consecutive patients that underwent an advanced imaging exam. Raised lesions were removed by endoscopic resection. Areas suspicious for dysplasia on NBI and VLE were biopsied or resected. This was followed by random biopsies. Finally WATS-3D brush biopsies were performed. RESULTS: One hundred and thirty eight cases were included in this study. Thirty-five cases (25% of the total ) were identified as some degree of dysplasia on the HDWLE-NBI-VLE-SP exam. Of these, 14 were reported as indefinite for dysplasia, 11 low-grade dysplasia (LGD), 8 high-grade dysplasia (HGD) or intramucosal cancer, and 2 T1b cancers. Adjunctive use of WATS-3D yielded an additional 12 new cases of dysplasia (9 with crypt dysplasia and 3 with LGD), for added yield of 34.3% (=12/35, 95% CI 14.6-62.2%). In addition, 8 cases of dysplasia found on the HDWLE-NBI-VLE-SP exam were upgraded by WATS-3D to a higher grade of dysplasia (5 with crypt dysplasia and 3 with HGD). Thus the absolute increase provided by WATS was 8.7% (=12/138) for a new diagnosis of dysplasia and was 14.5% (=20/138) when combining both new and upgraded diagnosis of dysplasia grade. CONCLUSION: The addition of WATS-3D to an already thorough exam with HDWLE-NBI-VLE-SP can increase the yield of dysplasia detection.