A Fatal Case of Erdheim-Chester Disease with Hepatic Involvement.

Erdheim-Chester disease (ECD) is a rare form of systemic histiocytosis, typically presenting with striking osseous involvement characterized by bilateral osteosclerosis and involvement of organs such as the lung, pituitary gland, heart, and brain. Liver involvement with ECD is extremely uncommon. We report a 56-year-old woman presenting with newly diagnosed cirrhosis and signs concerning for intra-abdominal malignancy, including omental caking and peritoneal thickening. Liver biopsy demonstrated xanthogranulomatous infiltration from ECD. The patient showed initial improvement with interferon therapy, but she developed severe depression, which led to the discontinuation of the treatment. Shortly afterward, she died from progressive liver dysfunction resulting in hepatorenal syndrome.

Double balloon enteroscopy: effective and minimally invasive method for removal of retained video capsules.

BACKGROUND AND AIM: Prior case series document removal of retained video capsules predominantly via surgical intervention. Data on endoscopic removal of retained capsules are limited. Our aim was to describe an endoscopic method of retrieval using double balloon enteroscopy (DBE). METHODS: A retrospective case series examination found 10 patients who underwent DBE for retrieval of a retained video capsule at two large tertiary referral academic centers from May 2007 to June 2013. RESULTS: Mean age of patients was 64.9 ± 18.1 years (four females, six males). Five patients failed to pass the capsule as a result of an ileal or jejunal stricture (one patient with ulcerative colitis; four patients with Crohn's disease); two patients had a small bowel stricture as a result of non-steroidal anti-inflammatory drug enteropathy; one patient had intermittent partial small bowel obstruction without evidence of a stricture; one patient had an obstructing malignant jejunal mass and one patient had a small bowel stricture as a result of radiation enteritis. Endoscopic removal via DBE was successful in eight of 10 patients (80%). The remaining two patients underwent surgical removal of the retained capsule. The two failed cases of capsule retrieval were both patients with suspected ileal disease. CONCLUSIONS: The most common cause of capsule retention was underlying Crohn's disease. DBE is an effective and minimally invasive method of capsule retrieval, including those patients with ileal disease, which has not been previously described. DBE can prevent unnecessary surgery while providing endoscopic therapy of inflammatory strictures by dilation.

Predictors of colorectal cancer testing using the California Health Inventory Survey.

AIM: To identify key variables associated with colon cancer testing using the 2009 California Health Inventory Survey (CHIS). METHODS: The CHIS has been conducted biennially since 2001 using a two-stage, geographically stratified random-digit-dial sample design to produce a representative sample of the entire State. For this study we used survey data from 2001-2009 inclusive. We restricted our analysis to White, Black, and Hispanic/Latinos aged 50-80 years. Weighted data was used to calculate the proportion of participants who underwent some form of colon cancer testing (colonoscopy, flexible sigmoidoscopy or fecal occult blood testing) within the previous 5 years stratified by race/ethnicity. For inferential analysis, boot-strapping with replacement was performed on the weighted sample to attain variance estimates at the 95%CI. For mean differences among categories we used t-tests and for comparisons of categorical data we used Pearson's χ(2). Binary logistic regression was used to identify independent variables associated with undergoing some form of testing. Trend analysis was performed to determine rates of testing over the study period stratified by race. RESULTS: The CHIS database for 2009 had 30 857 unique respondents corresponding to a weighted sample size of 10.6 million Californians. Overall, 63.0% (63.0-63.1) underwent a colon cancer test within the previous 5 years; with 70.5% (70.5%-70.6%) of this subset having undergone colonoscopy. That is 44.5% (44.4%-44.5%) of all individuals 50-80 underwent colonoscopy. By multivariable regression, those tested were more likely to be male (OR = 1.06; 95%CI: 1.06-1.06), Black (OR = 1.30; 95%CI: 1.30-1.31), have a family member with colon cancer (OR = 1.71; 95%CI: 1.70-1.72), and have health insurance (OR = 2.71; 95%CI: 2.70-2.72). Progressive levels above the poverty line were also associated with receiving a test (100%-199%: 1.21; 1.20-1.21), (200%-299%:1.41; 1.40-1.42), (> 300:1.69; 1.68-1.70). The strongest variable was physician recommendation (OR = 3.90; 95%CI: 3.88-3.91). For the Hispanic/Latino group, additional variables associated with testing were success of physician-patient communication (OR = 2.44; 95%CI: 2.40-2.48) and naturalized citizenship status (OR = 1.91; 95%CI: 1.89-1.93). Trend analysis demonstrated increased colon cancer testing for all racial/ethnic subgroups from 2001-2009 although the rate remained considerably lower for the Hispanic/Latino subgroup. CONCLUSION: Using CHIS we identified California citizens most likely to undergo colon cancer testing. The strongest variable associated with testing for all groups was physician recommendation.

Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism.

Angiotensin II (AngII) mediates progression of aortic aneurysm, but the relative contribution of its type 1 (AT1) and type 2 (AT2) receptors remains unknown. We show that loss of AT2 expression accelerates the aberrant growth and rupture of the aorta in a mouse model of Marfan syndrome (MFS). The selective AT1 receptor blocker (ARB) losartan abrogated aneurysm progression in the mice; full protection required intact AT2 signaling. The angiotensin-converting enzyme inhibitor (ACEi) enalapril, which limits signaling through both receptors, was less effective. Both drugs attenuated canonical transforming growth factor-ß (TGFß) signaling in the aorta, but losartan uniquely inhibited TGFß-mediated activation of extracellular signal-regulated kinase (ERK), by allowing continued signaling through AT2. These data highlight the protective nature of AT2 signaling and potentially inform the choice of therapies in MFS and related disorders.