Thrombotic Storm With Budd-Chiari Syndrome in a Pediatric Patient With Ulcerative Colitis.

Ulcerative colitis is associated with an increased risk of thromboembolic phenomena. Thrombotic storm defined by the development of multiple thrombi in multiple locations within a short period of time is a rare condition that is potentially life threatening. We present a 14-year-old adolescent boy with an ulcerative colitis flare complicated by Budd-Chiari syndrome and thrombotic storm.

Granulocyte macrophage-colony-stimulating factor autoantibodies and increased intestinal permeability in Crohn disease.

Background: Alterations in intestinal permeability have been implicated in the pathogenesis of Crohn disease (CD). We have reported that granulocyte macrophage-colony-stimulating factor (GM-CSF) is required for mucosal barrier function in mice, and elevated neutralizing GM-CSF autoantibodies (Ab) are associated with stricturing ileal disease and surgery in patients with CD. We hypothesized that children with CD with elevated GM-CSF Ab would exhibit increased intestinal permeability.

Innate dysfunction promotes linear growth failure in pediatric Crohn's disease and growth hormone resistance in murine ileitis.

Background: Growth failure remains a common complication of pediatric Crohn's disease (CD) and has been associated with small bowel involvement and need for surgery. We have reported that patients with elevated (≥ 1.6 μg/mL) granulocyte macrophage colony stimulating factor autoantibodies (GM-CSF Ab) are more likely to experience complicated ileal disease requiring surgery.

Crohn's disease limited to the appendix: a case report in a pediatric patient.

In the original description of Crohn's disease, the appendix was not believed to be involved in the inflammatory process. Later on, case reports started to appear in publications demonstrating that the appendix could be involved in the inflammatory changes of Crohn's disease, and it could also be the primary or the sole manifestation of the disease. Being that appendectomies are one of the most common procedures performed by pediatric surgeons, the knowledge about this diagnosis, all be it rare, is important.

Lipopolysaccharide exposure is linked to activation of the acute phase response and growth failure in pediatric Crohn's disease and murine colitis.

Background: Systemic exposure to lipopolysaccharide (LPS) has been linked to clinical disease activity in adults with inflammatory bowel disease (IBD). We hypothesized that markers of LPS exposure and the acute phase response (APR) would be increased in pediatric IBD patients with growth failure, and that LPS signaling would be required for induction of the APR in murine colitis.

Methods: Serum markers of LPS exposure, endotoxin core IgA antibody (EndoCAb), and the APR, LPS binding protein (LBP) were quantified in pediatric IBD patients and controls.