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Autoimmune enteropathy (AIE) is a rare immune disorder of the gut seldom found in adults and characterized by uncontrollable diarrhea resulting in malabsorption. While AIE is known to be pan-enteric, virtually all cases have presented with altered duodenal histology following known patterns with or without macroscopic change. We describe a unique case of seronegative AIE lacking typical duodenal manifestations in a 43-year-old female. To our knowledge, this is the first report of AIE lacking usual duodenal histologic changes, which resulted in missed diagnosis for years. Ultimately, crypt epithelial apoptosis, mononuclear inflammation of the lamina propria, and goblet cell loss of intestinal mucosa besides the duodenum clinched the diagnosis of AIE. Colonic histologic abnormalities consistent with AIE in the setting of diarrhea with malnutrition despite duodenal sparing should prompt suspicion for AIE given the pan-enteric nature of this disease. more...

Melanoma-associated retinopathy (MAR) is a paraneoplastic syndrome that involves the production of autoantibodies which can cross-react with retinal epitopes leading to visual symptoms. Autoantibodies can target intracellular proteins, and only a few are directed against membrane proteins. This discrepancy in autoantibody–protein target can translate into different immune responses (T-cell mediated vs B-cell mediated). Historically, treatment of MAR has focused on surgical reduction or immunosuppressive medication, mainly glucocorticoids. However, tumor resection is not relevant in metastatic melanoma in which MAR is mostly encountered. Moreover, the use of glucocorticoids can reduce the efficacy of immunotherapy. We report the first case to our knowledge with subjective resolution of visual symptoms and objective evidence of normalization of electroretinogram of MAR with undetectable autoantibodies after administration of programmed death-1 (PD-1) inhibitor (pembrolizumab) without the use of surgical reduction or systemic immunosuppression. This case highlights the potential improvement and resolution of negative autoantibody MAR with the use of PD-1 inhibitors and emphasizes the importance of multidisciplinary approach and team discussion to avoid interventions that can decrease immunotherapy-mediated anti-tumor effect. more...

The treatment of immunotherapy relapsed cutaneous melanoma constitutes a challenge in both research and clinical practice fields given the lack of effective therapeutic options. Homologous recombination deficiency (HRD) has been identified in several solid cancers including cutaneous melanoma. However, the utility of medications targeting HRD cancer cells is an uncharted territory in melanoma. Moreover, preclinical evidence suggests a synergistic role of combining immune checkpoint blockade (ICB) with drugs targeting HRD cancer cells such as PARP inhibitors. Here, we present a case study of a patient with immunotherapy relapsed melanoma who was found to have detected HRD and was treated with nivolumab (ICB) and olaparib (PARP inhibitors). more...

Autoimmune enteropathy (AIE) is a rare immune disorder of the gut seldom found in adults and characterized by uncontrollable diarrhea resulting in malabsorption. While AIE is known to be pan-enteric, virtually all cases have presented with altered duodenal histology following known patterns with or without macroscopic change. We describe a unique case of seronegative AIE lacking typical duodenal manifestations in a 43-year-old female. To our knowledge, this is the first report of AIE lacking usual duodenal histologic changes, which resulted in missed diagnosis for years. Ultimately, crypt epithelial apoptosis, mononuclear inflammation of the lamina propria, and goblet cell loss of intestinal mucosa besides the duodenum clinched the diagnosis of AIE. Colonic histologic abnormalities consistent with AIE in the setting of diarrhea with malnutrition despite duodenal sparing should prompt suspicion for AIE given the pan-enteric nature of this disease. more...

Background. Minor papilla (MiP) cannulation is frequently performed using specialized small-caliber accessories. Outcomes data for MiP cannulation with standard-sized accessories are lacking. Methods. This is a case series describing MiP cannulation outcomes in consecutive patients treated by two endoscopists between July 2005 and November 2008 at two tertiary referral centers. MiP cannulation was attempted using a 4.4 Fr tip sphincterotome loaded with a , 260 cm hydrophilic-tip guidewire, using a wire-guided technique under physician control. Results. 25 patients were identified (14 women, mean age 45). Procedure indications included recurrent acute pancreatitis in 16 patients (64%) and chronic pancreatitis in 2 (8%), among other indications. MiP cannulation was successful in 24 patients (96%). Sphincterotomy followed by pancreatic stent placement was performed in 21 patients (84%). Mild post-ERCP pancreatitis occurred in 3 patients (12%). Conclusion. Physician-controlled wire-guided MiP cannulation using a 4.4 Fr sphincterotome and guidewire is an effective and safe technique. more...

Propofol is an effective sedative in advanced endoscopy. However, the incidence of sedation-related complications is unclear. We sought to define the frequency of sedation-related adverse events, particularly the rate of airway modifications (AMs), with propofol use during advanced endoscopy. We also evaluated independent predictors of AMs.Patients undergoing sedation with propofol for advanced endoscopic procedures, including endoscopic retrograde cholangiopancreatography, endoscopic ultrasound, and small-bowel enteroscopy, were studied prospectively. Sedative dosing was determined by a certified registered nurse anesthetist with the goal of achieving deep sedation. Sedation-related complications included AMs, hypoxemia (pulse oximetry [SpO(2)]90%), hypotension requiring vasopressors, and early procedure termination. AMs were defined as chin lift, modified face mask ventilation, and nasal airway. We performed a regression analysis to compare characteristics of patients requiring AMs (AM+) with those who did not (AM-).A total of 799 patients were enrolled over 7 months. Procedures included endoscopic ultrasound (423), endoscopic retrograde cholangiopancreatography (336), and small-bowel enteroscopy (40). A total of 87.2% of patients showed no response to endoscopic intubation. Hypoxemia occurred in 12.8%, hypotension in 0.5%, and premature termination in 0.6% of the patients. No patients required bag-mask ventilation or endotracheal intubation. There were 154 AMs performed in 115 (14.4%) patients, including chin lift (12.1%), modified face mask ventilation (3.6%), and nasal airway (3.5%). Body mass index, male sex, and American Society of Anesthesiologists class of 3 or higher were independent predictors of AMs.Propofol can be used safely for advanced endoscopic procedures when administered by a trained professional. Independent predictors of AMs included male sex, American Society of Anesthesiologists class of 3 or higher, and increased body mass index. more...

Objective Existing research is inconsistent on whether clinical experience is associated with improved management of type 2 diabetes mellitus. We sought to determine whether meeting diabetes quality indicators improves as general internal medicine physicians progress from first to last year of residency. Methods We performed a chart abstraction of electronic health records data covering the period from September 2008 to August 2011. In all, 352 patient records were abstracted and linked to year of resident provider. Type 2 diabetes quality indicators included glycated hemoglobin (A1C), low-density lipoprotein, diastolic and systolic blood pressure control, obtaining urine microalbumin or prescription for angiotensin-converting enzyme inhibitor or angiotensin receptor blocker and documented foot and eye examinations. Chi-square tests and logistic regression analysis were used to determine whether year of residency was associated with quality of care indices before and after adjusting for patient age, gender, race, body mass index and cigarette smoking. Results Urine microalbumin was the most often met indicator (76.9%), and the least often met indicator was documented eye examination (37.4%). Results of adjusted analysis indicated that the odds of A1C, low-density lipoprotein control, obtaining urine microalbumin and documented eye and foot examinations were greater among patients of second- and third-year residents compared with those of first-year residents (odds ratios range, 1.26–5.12). Urine microalbumin was the indicator most often in optimal control and least often met indicators were eye and foot examinations. Conclusions We observed improvement in quality of diabetes care throughout residency. However, the low prevalence of several quality indicators indicates a need for additional training and quality improvement. more...

Background Successful cannulation of the common bile duct (CBD) remains the benchmark for ERCP. Use of a pancreatic duct (PD) stent to facilitate biliary cannulation has been described, although the majority of patients require precut sphincterotomy to achieve CBD cannulation. Objective To report the performance characteristics of using a PD stent in conjunction with physician-controlled wire-guided cannulation (WGC) to facilitate bile duct cannulation. Design Retrospective cohort. Setting Two tertiary care, academic medical centers. Patients All undergoing ERCP with native papillae. Intervention In cases of difficult biliary access in which the PD is cannulated, a pancreatic stent is placed. After this, physician-controlled WGC is attempted by using the PD stent to direct the sphincterotome into the biliary orifice. If cannulation is unsuccessful after several minutes, a precut sphincterotomy is performed over the PD stent or the procedure is terminated. Main Outcome Measurements Frequency of successful bile duct cannulation and precut sphincterotomy. Results A total of 2345 ERCPs were identified, 1544 with native papillae. Among these, CBD and PD cannulation failed in 16 (1.0%) patients, whereas 76 (4.9%) patients received a PD stent to facilitate biliary cannulation. Successful cannulation was achieved in 71 (93.4%) of 76 patients, 60 (78.9%) of whom did not require precut sphincterotomy. Complications included mild post-ERCP pancreatitis in 4 (5.3%) and aspiration in 1 (1.3%). Precut sphincterotomy was complicated by hemorrhage, controlled during the procedure in 2 (13.3%) of 15. Conclusions Physician-controlled WGC over a PD stent facilitates biliary cannulation while maintaining a low rate of precut sphincterotomy. more...