Search

Your search keyword '"Benjamin Shneider"' showing total 4 results
Start Over Author "Benjamin Shneider" Search Limiters Available in Library Collection
4 results on '"Benjamin Shneider"'

1. Hypercholesterolemia and changes in lipid and bile acid metabolism in male and female cyp7A1-deficient mice

Author

Sandra K. Erickson, Steven R. Lear, Sean Deane, Sandrine Dubrac, Sandra L. Huling, Lien Nguyen, Jaya S. Bollineni, Sarah Shefer, Hideyuki Hyogo, David E. Cohen, Benjamin Shneider, Ephraim Sehayek, Meena Ananthanarayanan, Natarajan Balasubramaniyan, Fredrick J. Suchy, Ashok K. Batta, and Gerald Salen

Subjects
liver, intestine, lipid synthesis, low density lipoprotein receptors, sterol 27-hydroxylase, bile acid transporters, Biochemistry, QD415-436
Abstract

Cholesterol 7α-hydroxylase, a rate-limiting enzyme for bile acid synthesis, has been implicated in genetic susceptibility to atherosclerosis. The gene, CYP7A1, encoding a protein with this activity, is expressed normally only in hepatocytes and is highly regulated. Our cyp7A1 gene knockout mouse colony, as young adults on a chow diet, is hypercholesterolemic. These mice were characterized extensively to understand how cyp7A1 affects lipid and bile acid homeostasis in different tissue compartments and whether gender plays a modifying role. Both male and female cyp7A1-deficient mice had decreased hepatic LDL receptors, unchanged hepatic cholesterol synthesis, increased intestinal cholesterol synthesis and bile acid transporters, and decreased fecal bile acids but increased fecal sterols. In females, cyp7A1 deficiency also caused changes in hepatic fatty acid metabolism, decreased hepatic canalicular bile acid transporter, Bsep, and gallbladder bile composition altered to a lithogenic profile.Taken together, the data suggest that cyp7A1 deficiency results in a proatherogenic phenotype in both genders and leads to a prolithogenic phenotype in females.

Published
2003
Full Text
View/download PDF

2. Longitudinal Outcomes in Young Patients with Alpha-1-Antitrypsin Deficiency with Native Liver Reveal that Neonatal Cholestasis is a Poor Predictor of Future Portal Hypertension

Author

Jeffrey Teckman, Philip Rosenthal, Kieran Hawthorne, Cathie Spino, Lee M. Bass, Karen F. Murray, Nanda Kerkar, John C. Magee, Saul Karpen, James E. Heubi, Jean P. Molleston, Robert H. Squires, Binita M. Kamath, Stephen L. Guthery, Kathleen M. Loomes, Averell H. Sherker, Ronald J. Sokol, Estella Alonso, Lee Bass, Susan Kelly, Mary Riordan, Hector Melin-Aldana, Jorge Bezerra, Kevin Bove, James Heubi, Alexander Miethke, Greg Tiao, Julie Denlinger, Erin Chapman, Ronald Sokol, Amy Feldman, Cara Mack, Michael Narkewicz, Frederick Suchy, Shikha Sundaram, Johan Van Hove, Benigno Garcia, Mikaela Kauma, Kendra Kocher, Matthew Steinbeiss, Mark Lovell, Kathleen Loomes, David Piccoli, Elizabeth Rand, Pierre Russo, Nancy Spinner, Jessi Erlichman, Samantha Stalford, Dina Pakstis, Sakya King, Robert Squires, Rakesh Sindhi, Veena Venkat, Kathy Bukauskas, Patrick McKiernan, Lori Haberstroh, James Squires, Laura Bull, Joanna Curry, Camille Langlois, Grace Kim, Jeffery Teckman, Vikki Kociela, Rosemary Nagy, Shraddha Patel, Jacqueline Cerkoski, Molly Bozic, Girish Subbarao, Ann Klipsch, Cindy Sawyers, Oscar Cummings, Simon Horslen, Karen Murray, Evelyn Hsu, Kara Cooper, Melissa Young, Laura Finn, Binita Kamath, Vicky Ng, Claudia Quammie, Juan Putra, Deepika Sharma, Aishwarya Parmar, Stephen Guthery, Kyle Jensen, Ann Rutherford, Amy Lowichik, Linda Book, Rebecka Meyers, Tyler Hall, Kasper Wang, Sonia Michail, Danny Thomas, Catherine Goodhue, Rohit Kohli, Larry Wang, Nisreen Soufi, Daniel Thomas, Nitika Gupta, Rene Romero, Miriam B. Vos, Rita Tory, John-Paul Berauer, Carlos Abramowsky, Jeanette McFall, Benjamin Shneider, Sanjiv Harpavat, Paula Hertel, Daniel Leung, Mary Tessier, Deborah Schady, Laurel Cavallo, Diego Olvera, Christina Banks, Cynthia Tsai, Richard Thompson, Edward Doo, Jay Hoofnagle, Averell Sherker, Rebecca Torrance, Sherry Hall, John Magee, Robert Merion, and Wen Ye

Subjects
Adult, Male, medicine.medical_specialty, Cirrhosis, Adolescent, medicine.medical_treatment, Cholestasis, Intrahepatic, Liver transplantation, Article, Young Adult, 03 medical and health sciences, Liver disease, 0302 clinical medicine, Cholestasis, alpha 1-Antitrypsin Deficiency, 030225 pediatrics, Internal medicine, Hypertension, Portal, medicine, Humans, Longitudinal Studies, 030212 general & internal medicine, Neonatal cholestasis, Child, Alpha 1-antitrypsin deficiency, business.industry, Infant, Newborn, Infant, medicine.disease, Liver Transplantation, Transplantation, Child, Preschool, Pediatrics, Perinatology and Child Health, Disease Progression, Portal hypertension, Female, business
Abstract

Objectives To identify predictors of portal hypertension, liver transplantation, and death in North American youth with alpha-1-antitrypsin (AAT) deficiency, and compare with patients with AAT deficiency elsewhere. Study design The Childhood Liver Disease Research Network Longitudinal Observational Study of Genetic Causes of Intrahepatic Cholestasis is a prospective, cohort study of pediatric cholestatic liver diseases, including AAT deficiency, enrolling PIZZ and PISZ subjects 0-25 years of age seen since November 2007 at 17 tertiary care centers in the US and Canada. Data from standard-of-care baseline and annual follow-up visits were recorded from medical records, history, physical examination, and laboratory studies. Participants with portal hypertension were identified based on data collected. Results We enrolled 350 participants (60% male) with a native liver; 278 (79%) entered the cohort without portal hypertension and 18 developed portal hypertension during follow-up. Thirty participants required liver transplantation; 2 patients died during 1077 person-years of follow-up. There was no difference in participants with or without preceding neonatal cholestasis progressing to transplantation or death during the study (12% vs 7%; P = .09), or in experiencing portal hypertension (28% vs 21%; P = .16); the hazard ratio for neonatal cholestasis leading to portal hypertension was P = .04. Development of portal hypertension was associated with a reduced height Z-score. Conclusions Portal hypertension in youth with AAT deficiency impacts growth measures. Progression to liver transplantation is slow and death is rare, but the risk of complications and severe liver disease progression persists throughout childhood. A history of neonatal cholestasis is a weak predictor of severe disease.

Published
2020
Full Text
View/download PDF

3. Diagnosis and management of primary sclerosing cholangitis

Author

Roger, Chapman, Johan, Fevery, Anthony, Kalloo, David M, Nagorney, Kirsten Muri, Boberg, Benjamin, Shneider, Gregory J, Gores, and Kerry N, Whitt

Subjects
medicine.medical_specialty, Hepatology, business.industry, Cholangitis, Sclerosing, MEDLINE, Constriction, Pathologic, Inflammatory Bowel Diseases, medicine.disease, Primary sclerosing cholangitis, Cholangiocarcinoma, Bile Ducts, Intrahepatic, Text mining, Bile Duct Neoplasms, medicine, Humans, Secondary sclerosing cholangitis, Radiology, business, Algorithms
Published
2010

4. Reply

Author

Gerald Salen, A.S. Knisely, Benjamin Shneider, Laura Bull, and D.H. Morton

Subjects
Hepatology, Gastroenterology
Published
2001
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results