Isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature

<p>Abstract</p> <p>Introduction</p> <p>We report a rare, classic case of isolated angioedema of the bowel due to C1-esterase inhibitor deficiency. It is a rare presentation and very few cases have been reported worldwide. Angioedema has been classified into three categories.</p> <p>Case presentation</p> <p>A 66-year-old Caucasian man presented with a ten-month history of episodic severe cramping abdominal pain, associated with loose stools. A colonoscopy performed during an acute attack revealed nonspecific colitis. Computed tomography of the abdomen performed at the same time showed a thickened small bowel and ascending colon with a moderate amount of free fluid in the abdomen. Levels of C4 (< 8 mg/dL; reference range 15 to 50 mg/dL), CH50 (< 10 U/mL; reference range 29 to 45 U/ml) and C1 inhibitor (< 4 mg/dL; reference range 14 to 30 mg/dL) were all low, supporting a diagnosis of acquired angioedema with isolated bowel involvement. Our patient's symptoms improved with antihistamine and supportive treatment.</p> <p>Conclusion</p> <p>In addition to a detailed comprehensive medical history, laboratory data and imaging studies are required to confirm a diagnosis of angioedema due to C1 esterase inhibitor deficiency.</p

Escalating complexity of endoscopic retrograde cholangiopancreatography over the last decade with increasing reliance on advanced cannulation techniques

At our academic tertiary care medical center, we have noted patients referred for endoscopic retrograde cholangiopancreatography (ERCP) who increasingly require advanced cannulation techniques. This trend is noted despite increased endoscopist experience and annual ERCP volume over the same period. To evaluate this phenomenon of perceived escalation in complexity of cannulation at ERCP and assessed potential underlying factors. Demographic/clinical variables and records of ERCP patients at the beginning (2008), middle (2013) and end (2018) of the last decade were reviewed retrospectively. Cannulation approaches were classified as "standard" or "advanced" and duodenoscope position was labeled as "standard" (short position) or "non-standard" ( long, semi-long). Patients undergoing ERCP were older in 2018 compared to 2008 (69.7 ± 15.2 years 55.1 ± 14.7, < 0.05). Increased ampullary distortion and peri-ampullary diverticula were noted in 2018 ( < 0.001). ERCPs were increasingly performed with a non-standard duodenoscope position, from 2.2% (2008) to 5.6% (2013) and 16.1% (2018) ( < 0.001). Utilization of more than one advanced cannulation technique for a given ERCP increased from 0.7% (2008) to 0.9% (2013) to 6.6% (2018) ( 4 cm, pancreatic uncinate mass, and bilirubin > 10 mg/dL predicted use of advanced cannulation techniques ( < 0.03 for each). Complexity of cannulation at ERCP has sharply increased over the past 5 years, with an increased proportion of elderly patients and those with malignancy requiring advanced cannulation approaches. These data suggest that complexity of cannulation at ERCP may be predicted based on patient/ampulla characteristics. This may inform selection of experienced, high-volume endoscopists to perform these complex procedures