Erin W. Thackeray

Colon Neoplasms Develop Early in the Course of Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

BACKGROUND & AIMS Colon cancer surveillance guidelines for patients with inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC) suggest annual colonoscopy once the diagnosis of concomitant disease is made, but there is little evidence to support this recommendation. We conducted a retrospective review of patients with colonic neoplasms (colon cancer or dysplasia) to test this guidelines validity and determined when colonic neoplasms occurred in the population. METHODS Records were retrospectively reviewed from 54 patients with IBD, PSC, and colonic neoplasia for dates of diagnosis of IBD, PSC, and colon neoplasia and descriptive information about the colon neoplasms that developed. RESULTS The occurrence of colon neoplasms within 2 years of diagnosis of IBD and PSC (21.5 per 100 patient years of follow-up) was similar to the occurrence within 8 to 10 years from diagnosis of IBD and PSC (20.4 per 100 patient years of follow-up). The colonic neoplasms that developed in this population were spread throughout the colon. CONCLUSIONS Patients with IBD and PSC have a risk of developing colonic neoplasms soon after the coexistence of the 2 diseases is discovered. This finding supports the current colon cancer surveillance guideline recommendations of yearly colonoscopies for this patient population, beginning at the time of diagnosis of PSC in patients with IBD or with the diagnosis of IBD in patients with PSC.

Likelihood of Malignancy in Gallbladder Polyps and Outcomes Following Cholecystectomy in Primary Sclerosing Cholangitis

OBJECTIVES:Patients with primary sclerosing cholangitis (PSC) have an increased risk for gallbladder cancer. We aimed to define the postoperative outcomes in PSC patients after cholecystectomy and determine if size of a gallbladder lesion on imaging predicts the presence of neoplasia.METHODS:We conducted a retrospective review of patients with PSC who underwent cholecystectomy at Mayo Clinic between 1 January 1995 and 31 December 2008. Patients with a prior history of a liver transplant or cholangiocarcinoma were excluded.RESULTS:A total of 57 patients were included in our primary analysis during the early postoperative period. The most common indication for undergoing a cholecystectomy was the presence of a gallbladder polyp or mass. The sensitivity and specificity of a gallbladder lesion of 0.80 cm and the presence of gallbladder neoplasia was 100% (95% confidence interval (CI) 77–100%) and 70% (95% CI 35–93%), respectively. Of the patients, 23 (40%) had an early postoperative complication. The Child-Pugh score was the only predictor of postoperative outcomes in the multivariate model (odds ratio 1.78, 95% CI 1.11–3.12, P=0.02).CONCLUSIONS:Cholecystectomy in patients with PSC is associated with a high morbidity. Gallbladder polyps <0.80 cm are unlikely to be malignant and observation of these small polyps should be considered. A higher Child-Pugh score was associated with early postoperative complications.

Hepatic iron overload or cirrhosis may occur in acquired copper deficiency and is likely mediated by hypoceruloplasminemia.

Background The metabolic fates of copper and iron are closely linked through ceruloplasmin and hephaestin. Ceruloplasmin is the principal copper carrying protein and decreases in acquired copper deficiency. Congenital absence of ceruloplasmin (aceruloplasminemia) results in tissue iron overload. Animal studies suggest hypoceruloplasminemia and impaired hephaestin function result in tissue iron accumulation. Objectives There are no data on hepatic function, pathology, and iron status in patients with acquired copper deficiency. This report studies these issues in 4 patients with acquired copper deficiency. Study This is a retrospective review of hepatic status (imaging, liver function tests, liver biopsy) in 4 patients with neurologic and hematologic manifestations of acquired copper deficiency who also had imaging and/or pathologic evidence of hepatic dysfunction. Results Two patients (cases 1 and 2) showed imaging evidence of cirrhosis and pathologic evidence of cirrhosis or advanced fibrosis. Two patients (cases 3 and 4) had pathologic evidence of hepatic iron overload. All patients had some evidence of abnormality on liver function tests. Conclusions Acquired copper deficiency causes a secondary ceruloplasmin deficiency which can result in hepatic iron overload and/or cirrhosis.

The Effects of Gender and Age on Evaluation of Trainees and Faculty in Gastroenterology

OBJECTIVES:We studied whether differences exist in evaluation scores of faculty and trainees in gastroenterology (GI) based on the gender of the evaluator or evaluatee, or the evaluator–evaluatee gender pairing.METHODS:We examined evaluations of faculty and trainees (GI fellows and internal medicine residents rotating on GI services), using mixed linear models to assess effects of the four possible evaluator–evaluatee gender pairings. Potential confounding variables were adjusted for, and random effects were used to account for repeated assessments.RESULTS:For internal medicine (IM) residents, no difference in evaluation scores based on gender was found. Resident age was negatively associated with performance rating, while percentage correct on the in-training examination (ITE) was positively associated. For GI fellows, the interaction between evaluator and evaluatee gender was significant. Fellow age and international medical graduate (IMG) status were negatively associated with performance rating, while ITE percentage correct was positively associated. For faculty, no difference was found in evaluation scores by IM residents based on the gender of the evaluated faculty or the evaluating resident, although the interaction between the evaluator and the evaluatee gender was significant. Gender had a significant marginal effect on faculty scores by GI fellows, with female faculty receiving lower scores. The interaction between evaluator and evaluatee gender was also significant for evaluations by fellows. Faculty age was negatively associated with performance rating.DISCUSSION:Gender, age, and ITE performance are associated with evaluation scores of GI trainees and faculty at our institution. The interaction of evaluator and evaluatee gender appears to play a more critical role in evaluation scoring than the gender of the evaluatee or evaluator in isolation.

A Rare Cause of Abdominal Pain and Ascites

Question: A 52-year-old man was admitted to the hospital for progressive abdominal pain and distension over 3 months. Physical examination demonstrated an ill-appearing man with a distended and tense abdomen along with palpation tenderness and flank dullness. There was moderate bilateral lower extremity pitting edema. Laboratory studies revealed a hemoglobin (normal values) of 11.4 g/dL (13.5–17.5); leukocytes, 26.5 109/L (3.5–10.5 109); platelets, 705 109/L (150 – 450 109); and albumin, 2.8 g/dL (3.5–5), with normal international normalized ratio and liver biochemistries. An ultrasound-guided paracentesis removed 4 L of clear yellow fluid with analysis showing total nucleated cells of 1315/ L (22% neutrophils), total protein of 3.7 g/dL (47% albumin), and no malignant cells on cytology. Computed tomography demonstrated multiple large, ill-defined abdominal masses with peritoneal implants and large volume ascites (Figure A) without an apparent organ-based primary. An anterior peritoneal mass was biopsied (Figure B) and immunohistochemical stains strongly positive for desmin (Figure C). What is the diagnosis? See the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.