Philip K. Frykman

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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)
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)
The aim of the study was to describe and characterize a novel small spleen phenotype with splenic lymphopenia in the Ednrb-null (Ednrb−/−) mouse with aganglionosis known to also develop enterocolitis. We compared spleen weight as a percent of body weight from Ednrb+/+, Ednrb+/−, and Ednrb−/− mice to quantify our initial observation. Splenic(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)
PURPOSE The effect of timing of onset of necrotizing enterocolitis (NEC) on outcomes has not been determined for the full-term infant. In this study we aimed to characterize the full-term NEC population and to evaluate onset of NEC. METHODS We performed a two-center retrospective review of all full-term infants (≥ 37weeks) with a diagnosis of NEC between(More)
BACKGROUND Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair. PATIENTS AND METHODS Medical records for all cases(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)