Background: Hepatorenal syndrome (HRS) is a serious complication of advanced chronic liver disease. Different pharmacological therapies have variable efficacy. We performed a systematic review and meta-analysis to compare the efficacy of various drugs in the treatment of HRS. Study: Randomized controlled trials comparing active drug with placebo or comparing 2 different drugs were included in this analysis. Primary study outcome was reversal of HRS. Secondary outcomes were HRS relapse and patient survival. Subgroup analysis was performed on patients with type 1 HRS. Results: Thirteen randomized controlled trial were eligible for analysis. Terlipressin plus albumin was more efficacious than placebo plus albumin (odds ratio=4.72; 95% confidence interval, 1.72-12.93; P=0.003) or midodrine plus albumin and octreotide (odds ratio=5.94; 95% confidence interval, 1.69-20.85; P=0.005), for HRS reversal. However, no significant difference was noted comparing terlipressin plus albumin versus noradrenaline plus albumin, octreotide plus albumin versus placebo plus albumin or noradrenaline plus albumin versus midodrine plus albumin and octreotide. None of the comparisons showed difference on HRS relapse or patient survival. Subgroup analysis revealed that terlipressin was more effective than placebo for type 1 HRS reversal, but no significant differences were noted between any other comparisons, and none of the comparisons showed difference on HRS relapse or patient survival. Conclusions: Intravenous infusion of terlipressin is the most effective medical therapy for reversing HRS. Intravenous infusion of noradrenaline is an acceptable alternative. Studies are needed as basis for developing pharmacological strategies to reduce relapse of HRS and improve patient survival.

Background and study aims Left ventricular assist devices (LVADs) are currently the standard of care in treatment of patients with end-stage heart failure waiting for heart transplant as well as destination therapy for non-transplant candidates. However, patients with LVADs are at increased risk of gastrointestinal bleeding due to the device’s unique effects on hemodynamics. A major source of gastrointestinal bleeding in these patients are gastrointestinal angioectasias located within the small bowel that can only be reached with deep enteroscopy. The goal of our study was to determine the safety and efficacy of single-balloon enteroscopy (SBE) in treating gastrointestinal bleeding in patients with LVADs. Patients and methods We present a retrospective case series performed on patients with LVADs who underwent SBE to treat episodes of gastrointestinal bleeding. All procedures were performed at Emory University Hospital by a single endoscopist. Patient demographics, diagnosis and treatment of gastrointestinal bleeding, episodes of re-bleeding, and procedure-related complications were examined. Results A total of 27 SBE procedures performed in 14 patients were reviewed. SBE was performed in an antegrade approach in 89 % (24/27) of cases. Deep intubation was achieved in all antegrade procedures, with the distal jejunum reached in 79 % (19/24) of cases. The diagnostic yield was 78 %. There were no reported complications associated with the procedures. Conclusions SBE is a safe and effective modality to manage gastrointestinal bleeding in patients with LVADs.

Typical causes of intermittent esophageal dysphagia in a young person include eosinophilic esophagitis, esophageal dysmotility and esophageal rings. We report a 35-year-old male with a one year history of intermittent dysphagia to solid foods. After the endoscopic removal of a food bolus, a barium swallow revealed extrinsic compression of the proximal esophagus. Computed tomography angiogram revealed an aberrant right subclavian artery (ARSA) coursing behind the esophagus, suggesting the diagnosis of dysphagia lusoria. Although rare, dysphagia lusoria represents an important consideration in the differential diagnosis of intermittent esophageal dysphagia in a young adult.

A 41 year-old female without significant medical history was admitted to our tertiary care medical center with one month history of solid and liquid food dysphagia and a two-day history of intractable nonbloody emesis followed by severe epigastric and substernal chest pain. An EKG revealed sinus tachycardia and standard laboratory parameters were unremarkable. An emergent CT scan revealed pneumatosis of the proximal stomach, gastroesophageal junction, and distal esophagus without evidence of free air (Figure 1). Symptoms resolved with medical therapy and an urgent esophagogastroduodenoscopy (EGD) was planned. A standard 9 mm gastroscope could not be advanced past an esophageal web at 15 cm from incisors. A 4.9 mm gastroscope was used to intubate the esophagus and traverse the web. At 35 cm, a large, left esophageal wall tear was visualized and extended 5 cm distally to a false lumen located 2 cm proximal to gastroesophageal junction (Figure 2). The findings were suggestive of esophageal dissection without perforation. The stomach and duodenum appeared normal. An esophageal overtube was placed in standard fashion to “dilate” the proximal web and to act as a working channel. An 11 mm x 6 mm, type t Over-The-Scope-Clip (OTSC, Ovesco Endoscopy) system was attached to endoscope tip and advanced into the esophagus through the overtube. The edges of the false lumen were approximated carefully and the clip was deployed closing the defect while maintaining luminal patency (Figure 3). Post-procedure water soluble esophagram revealed no esophageal leak and confirmed patency of esophageal lumen. The patient tolerated a liquid diet, and three days later a repeat EGD revealed the clip in place, with no stenosis of true lumen. Patient's diet was advanced to soft foods and she was discharged with GI clinic follow-up.Figure 1Figure 2Figure 3Boerhaave's syndrome is a rupture of the esophageal wall caused by repetitive vomiting and both the syndrome and its treatment with emergent surgical repair are associated with significant morbidity and mortality. The OTSC device aggregates a large amount of tissue to facilitate closure of perforations, fistulas and anastomotic leaks. Endoscopic closure of esophageal perforation using the OTSC clip system is a minimally invasive, easy to use, robust and successful method that has been described in a few case reports and should be considered in all cases of Boerhaave's syndrome.

A 35-year-old Caucasian male was admitted to our tertiary care institution after he presented with complaints of the sensation of food stuck in his chest for 2 days following the consumption of pork. He denied choking, aspiration, associated dyspnea, problems initiating a swallow or odynophagia. He admitted to having food impactions in the past, requiring esophagogastroduodenoscopy (EGD) for removal. Prior esophageal biopsies did not reveal evidence of eosinophils. His past medical history included chronic tension headaches but he denied a history of atopy or asthma. On initial evaluation, he was handling his oral secretions. Vital signs were within normal limits, and physical examination was unremarkable. Laboratory studies were within normal limits. Glucagon was administered in the emergency room without clinical improvement. EGD revealed a large food bolus lodged in the proximal esophagus which was successfully removed endoscopically. The underlying mucosa appeared friable but no strictures or rings were visualized. Post-endoscopy esophagram revealed a smooth narrowing at the pharyngoesophageal junction consistent with a cricopharyngeal bar (Figure 1). There appeared to be extrinsic compression of the proximal and mid-esophagus from a vascular structure. A computed tomography (CT) angiography of the chest demonstrated an aberrant right subclavian artery (ARSA) passing behind the esophagus, resulting in compression of the posterior aspect of the thoracic esophagus (Figure 2). These findings, in conjunction with the patient's history, suggested the diagnosis of dysphagia lusoria. The patient was referred to vascular surgery clinic for consideration of surgical correction. A reconstruction of the CT images was performed as shown in Figure 3. Dysphagia lusoria results from a congenital abnormality in the development of the aortic arch and its branches causing extrinsic compression of the esophagus. In the majority of cases, the causative vessel is an ARSA originating from a left-sided aortic arch. ARSA originates from the proximal portion of the descending thoracic aorta and courses posterior to the esophagus in 80% of cases. This abnormal course led to the term lusorian artery from the Latin lusus naturae, or “freak of nature.” Based on retrospective data, 30-40% of patients with ARSA will develop dysphagia. Management for mild symptoms is conservative, but for moderate-to-severe symptoms surgical intervention is recommended.Figure 1Figure 2Figure 3

AIM To examine the association of PNPLA3 polymorphisms in chronic hepatitis C patients and development of liver disease spectrum. METHODS Literature was searched systematically from PubMed/MEDLINE, EMBASE, and Cochrane search engines for full-length articles written in English that examined PNPLA3 polymorphism in chronic hepatitis C (CHC) patients. Studies evaluating the association of PNPLA3 polymorphism spectrum (fatty liver, steatohepatitis, cirrhosis, and hepatocellular carcinoma) of CHC were included. Pooled data are reported as OR with 95%CI. Our study endpoint was the risk of the entire liver disease spectrum including: Steatosis/fatty liver, cirrhosis, and hepatocellular carcinoma in CHC patients with PNPLA3 polymorphisms. RESULTS Of 380 studies identified, a total of 53 studies were included for full-text review. Nineteen on chronic hepatitis C were eligible for analysis. Pooled ORs for rs738409 GG compared to CC and CG among patients with fatty liver was 2.214 (95%CI: 1.719-2.853). ORs among advanced fibrosis/cirrhosis were 1.762 (95%CI: 1.258-2.468). Similar odds ratios among hepatocellular carcinoma patients were 2.002 (95%CI: 1.519-2.639). Pooled ORs for rs738409 GG and CG compared to CC among patients with fatty liver were 1.750 (95%CI: 1.542-1.986). Pooled ORs for advanced fibrosis/cirrhosis patients were 1.613 (95%CI: 1.211-2.147). All analyses were homogenous and without publication bias except one. The associations were maintained after adjusting for publication bias and heterogeneity. CONCLUSION PNPLA3 polymorphisms have strong association with increased risk and severity of the liver disease spectrum in CHC patients.

Symptomatic gastrointestinal (GI) amyloidosis is a rare manifestation of systemic amyloid light chain amyloidosis. Further, the presentation of primary GI amyloidosis without previously diagnosed systemic amyloidosis is exceptionally rare. We describe a case of a patient presenting with abdominal pain, nausea, vomiting, and weight loss later to be diagnosed with localized GI amyloidosis from underlying multiple myeloma. Unfortunately, the GI disease was insurmountable leading to her death. Amyloidosis exerts its pathology at the level of the mucosa, soft tissue, nerves, and vascular supply of the GI tract. No specific treatments for the GI complications of amyloidosis are available and supportive measures are universally employed. GI amyloidosis is not only infrequently amenable to systemic and symptomatic treatment, but has a negative impact on both quality of life and survival.

Objectives: Brush cytology has a low sensitivity for the diagnosis of cholangiocarcinoma. This study aimed to compare the standard approach (brush cytology) with a triple modality approach utilizing brush cytology, forceps biopsy and fluorescence in situ hybridization in terms of sensitivity and specificity for the diagnosis of cholangiocarcinoma. Methods: In a retrospective study at a single academic center, 50 patients underwent triple modality testing. Additionally, 61 patients underwent brush cytology alone. Intervention was endoscopic retrograde cholangiopancreatography with brush cytology, fluorescence in situ hybridization, and forceps biopsy. The main outcome measures included sensitivity, specificity, positive predictive value and negative predictive value. Results: Overall, 50 patients underwent triple tissue sampling, and 61 patients underwent brush cytology alone. Twenty-two patients were eventually diagnosed with cholangiocarcinoma. Brush cytology had a sensitivity of 42%, specificity of 100%, positive predictive value of 100% and negative predictive value of 88%. Triple tissue sampling had an overall sensitivity of 82%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 87%. Within the triple test group, brush cytology had a sensitivity of 27%, forceps biopsy had a sensitivity of 50%, and fluorescence in situ hybridization analysis had a sensitivity of 59%. Conclusions: A triple modality approach results in a marked increase in sensitivity for the diagnosis of cholangiocarcinoma compared with single modality testing such as brush cytology and should be considered in the evaluation of indeterminate or suspicious biliary strictures.

Purpose: Health maintenance is a crucial component to the comprehensive care that patients with Inflammatory Bowel Disease (IBD) require. In fact, gastroenterologists typically assume the role of the primary care physicians when it comes to preventative care in these patients. This study examines the ability of gastroenterologists in the community setting to document adherence to the recommended preventative care measures for IBD patients. We reviewed the charts of IBD patients on biologics (infliximab, adalimumab, or certolizumab pegol) and non-biologics to determine compliance with yearly flu vaccination, the pneumococcal vaccine every five years, hepatitis B serology testing, and bone densitometry scans. Methods: We performed a retrospective study of IBD being treated with infliximab, adalimumab, or certolizumab pegol by the Wake Endoscopy Center group of gastroenterologists from January 1, 2009 to January 1, 2013. We also evaluated 300 connective patients not on biologics (control) during that time frame. Each patient's chart was examined for documentation of a yearly flu vaccination, quinquennial pneumococcal vaccine, hepatitis B serology, and bone densitometry since January 1, 2009. Results: In our database, 197 patients received infliximab therapy: 132 with Crohn's Disease (CD), 65 with UC since January 1, 2009. Our results showed that 24% of IBD patients (CD and UC combined) on infliximab received their yearly flu vaccine, 7% received the pneumococcal vaccine every 5 years. Sixty-two percent had hepatitis B serology testing, 8% of CD patients received a bone densitometry scan, 20% of UC patients received one. Our database included 300 patients not receiving biologics, 102 with CD, and 198 with UC. Of these patients, only 1.3% of these patients received a yearly flu vaccine, 1% received the pneumococcal vaccine in the last 5 years, and 6% had hepatitis B serology testing; only 3% of CD patients and 2% of UC patients received bone densitometry scans.Table: Table. Biologic vs non-biologic therapyConclusion: Many IBD patients in the community setting are not receiving standard preventative care. Patients receiving biologics are more likely to have better adherence compared to those not on biologics. Poor documentation and physician oversight may be some potential causes of our overall disappointing results, as well as associations with frequency of therapy and office visits. Gastroenterologists treating IBD should be more proactive with health maintenance and diligent with proper documentation.

Purpose: Biliary injury may be a clinically dangerous complication of cholecystectomy and liver transplantation, as bile leak can lead to ileus, bilomas, cholangitis, and even sepsis and subsequent death. Although endoscopic retrograde cholangiopancreatography (ERCP) has proven itself a useful and arguably preferable diagnostic and therapeutic alternative to surgery, there has been little data on the long-term success and complications encountered in these patients, especially as related to the etiology of the bile leak. Methods: A retrospective chart review was conducted on patients undergoing ERCP for suspected bile leak. All ERCPs were performed by three endoscopists at two clinical sites at an academic center between December 2008 and August 2011. Results: A total of 40 ERCPs met criteria for review, with 31 for initial evaluation and management and 9 for repeated attempts at management. There were 17 females and 14 males with a mean age of 51.6 years. Average time to follow up was 661 days. Cholecystectomy was the most frequent cause of suspected biliary leak (16/31), followed by orthotopic liver transplantation (12/31), and liver metastases from primary colon or rectal cancer (3/31). Within the 16 patient cholecystectomy group, actual bile leak was found in 11 patients, with seven of those having successful resolution of bile leak on first attempt. Post ERCP pancreatitis was found in two patients, with another patient developing a persistent biloma, and another patient developing a candida infection of the biliary system. There were two patients requiring CT drains. One patient required an exploratory laparotomy with pancreatic abscess drainage. Among the 12 patient transplant group, bile leak was found in 10 patients and was successfully resolved on first attempt in two patients. One patient developed post ERCP pancreatitis. One patient developed cholangitis. CT drains were placed in two patients. There were four patients requiring exploratory laparotomy with Roux en Y procedures to resolve the leak. Within the three patient cancer group, one patient had no leak, one patient had resolution of leak on first attempt, and the last patient required second ERCP to resolve the leak. There were no complications in this group. Conclusion: Over the long term, ERCP is an effective and safe tool for detection and management of biliary leak, resolving biliary leak and helping to avoid surgery in the majority of patients. Successful resolution of bile leak on first attempt was more common in the cholecystectomy group. Eventual surgical intervention occurred more commonly in the transplant group. CT drain placement and infectious complications appeared with equal frequency between the cholecystectomy and transplant groups.

Purpose: There is a growing trend to utilize propofol for outpatient endoscopic procedures leading to increased costs for endoscopy. The costs can be reduced by using a nurse anesthetist without the need for a physician anesthesiologist. Our study compares complication rates of propofol (P) given by a nurse anesthetist (NA) without supervision of a physician anesthesiologist vs. standard conscious sedation (CS) given by an attending gastroenteriologist (G). Methods: In an open-access endoscopy setting, a set of criterion was given to a scheduling team for assigning patients to undergo endoscopy via propofol (P) administrated by a nurse anesthetist (NA). These included higher than average risk patients defined as patients with history of regular use of narcotics or benzodiazepines, history of documented anxiety disorder, BMI >40, as well as patients reporting difficulty with prior sedation. These patients were assigned to receive propofol administrated by a nurse anesthetist. Lower or average risk patients were typically given conscious sedation (CS)administrated by an attending gastroenterologist (G). Results: We performed a retrospective study looking at complication rates in our endoscopy suite over a period of one year (May 2010 to April 2011). During this time frame, we reviewed electronic charts of 6330 patients and recorded all complications related to sedation. Of 2093 patients receiving propofol administrated by a nurse anesthetist, there were no complications related to sedation. Of 4237 patients receiving conscious sedation administrated by a gastroenterologist, there were five complications related to sedation. Of these five patients, three required airway management until improved oxygenation was seen, and two required narcan for reversal of sedation. No patients required intubation, and no other major complications occurred. Conclusion: The administration of propofol by a nurse anesthetist without an attending anesthesiologist is very safe in the open access outpatient endoscopy suite setting. Our findings reveal comparable or lower complication rates vs. standard conscious sedation, despite potentially more difficult patients in the propofol group. Hence, there is probably no role for a physician anesthesiologist in the endoscopy suite setting, which can potentially decrease the costs of outpatient endoscopy.Table: Complication rates of nurse anesthetist vs gastroenterologist