Learn More
The very low density lipoprotein (VLDL) receptor is a recently cloned member of the low density lipoprotein (LDL) receptor family that mediates the binding and uptake of VLDL when overexpressed in animal cells. Its sequence is 94% identical in humans and rabbits and 84% identical in humans and chickens, implying a conserved function. Its high level(More)
Mice deficient in cholesterol 7alpha-hydroxylase, the rate-limiting enzyme of bile acid biosynthesis, were constructed by targeted disruption of the Cyp7 gene. The introduced mutation removed exons 3-5 of the gene and gave rise to a null allele that encoded no immunoreactive or enzymatically active protein. Heterozygous carriers of the disrupted gene(More)
PURPOSE The aim of the study was to characterize enterocolitis in the Ednrb-null (Ednrb-/-) mouse with aganglionosis of the colon and to develop and validate a semiquantitative histopathologic grading system to assess enterocolitis. METHODS We isolated colon and ileal specimens of Ednrb-/- and control mice (Ednrb+/+) and performed histochemical staining(More)
We used advanced spectral imaging for intrasurgical decision making in a preclinical study, on a mouse model of Hirschsprung's Disease. Our imaging device sampled areas from normal and abnormal (aganglionic) colon in these animals. Spectral segmentation and classification of the resulting images showed a clear distinction between the normal and aganglionic(More)
We present a case of a child born with a birthmark over the lumbar spine, which harbored a pinhole-sized opening. At 6 months of age the child presented with fever of unknown origin. Subsequent lower extremity pain resulted in imaging studies that revealed a spinal mass with extension into the posterior mediastinum. At operation, the child was found to have(More)
PURPOSE Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohn's disease (CD). It is unknown whether children who present with PF at the time of diagnosis have a different course than those that develop PF while on therapy. METHODS From a prospective, single institution observational registry of children diagnosed(More)
PURPOSE Our aim was to compare outcomes of children undergoing laparoscopic cholecystectomy with laparoscopic common bile duct exploration (LC+CBDE) to those undergoing laparoscopic cholecystectomy with adjunctive endoscopic retrograde cholangiopancreatography (LC+ERCP). METHODS We performed a two-center retrospective chart review of all children (<18(More)
A new member of the low density lipoprotein receptor gene family that binds and internalizes very low density lipoprotein (VLDL) particles was previously cloned and characterized from the rabbit and human. The physiological role of this putative VLDL receptor is not known, but its tissue distribution and ligand specificity suggest a possible role in the(More)
Development of potentially life-threatening enterocolitis is the most frequent complication in children with Hirschsprung disease (HSCR), even after definitive corrective surgery. Intestinal microbiota likely contribute to the etiology of enterocolitis, so the aim of this study was to compare the fecal bacterial and fungal communities of children who(More)
Hirschsprung-associated enterocolitis remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications, such as stricture formation, prophylaxis with rectal washouts, and identification of(More)