Search Results - "Shneider, BL"

Severe Bile Salt Export Pump Deficiency: 82 Different ABCB11 Mutations in 109 Families by Strautnieks, Sandra S, Byrne, Jane A, Pawlikowska, Ludmila, Cebecauerová, Dita, Rayner, Anne, Dutton, Laura, Meier, Yvonne, Antoniou, Anthony, Stieger, Bruno, Arnell, Henrik, Özçay, Figen, Al–Hussaini, Hussa F, Bassas, Atif F, Verkade, Henkjan J, Fischler, Björn, Németh, Antal, Kotalová, Radana, Shneider, Benjamin L, Cielecka–Kuszyk, Joanna, McClean, Patricia, Whitington, Peter F, Sokal, Étienne, Jirsa, Milan, Wali, Sami H, Jankowska, Irena, Pawłowska, Joanna, Mieli–Vergani, Giorgina, Knisely, A.S, Bull, Laura N, Thompson, Richard J

“…Background & Aims: Patients with severe bile salt export pump (BSEP) deficiency present as infants with progressive cholestatic liver disease. We characterized…”
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A Systematic Review of Immunosupressant Adherence Interventions In Transplant Recipients: Decoding the Streetlight Effect by Duncan, S, Annunziato, RA, Dunphy, C, Rudow-LaPointe, D, Shneider, BL, Shemesh, E

“…Nonadherence to immunosuppressant medications is an important risk factor for graft dysfunction. To evaluate the effectiveness of adherence-enhancing…”
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Intestinal Bile Acid Transport: Biology, Physiology, and Pathophysiology by Shneider, Benjamin L.

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Medication Adherence in Pediatric and Adolescent Liver Transplant Recipients by Shemesh, Eyal, Shneider, Benjamin L, Savitzky, Jill K, Arnott, Lindsay, Gondolesi, Gabriel E, Krieger, Nancy R, Kerkar, Nanda, Magid, Margret S, Stuber, Margaret L, Schmeidler, James, Yehuda, Rachel, Emre, Sukru

“…Nonadherence to medications is a leading cause of morbidity in children and adolescents who have had a transplant, yet there are no published data about the…”
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Hepatocyte nuclear factor-1α is an essential regulator of bile acid and plasma cholesterol metabolism by Stoffel, Markus, Shih, David Q, Bussen, Markus, Sehayek, Ephraim, Ananthanarayanan, Meenakshisundaram, Shneider, Benjamin L, Suchy, Frederick J, Shefer, Sarah, Bollileni, Jaya S, Gonzalez, Frank J, Breslow, Jan L

“…Maturity-onset diabetes of the young type 3 (MODY3) is caused by haploinsufficiency of hepatocyte nuclear factor-1 alpha (encoded by TCF1). Tcf1 super(-/-)…”
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One hundred nine living donor liver transplants in adults and children: a single-center experience by Miller, C M, Gondolesi, G E, Florman, S, Matsumoto, C, Muñoz, L, Yoshizumi, T, Artis, T, Fishbein, T M, Sheiner, P A, Kim-Schluger, L, Schiano, T, Shneider, B L, Emre, S, Schwartz, M E

“…To summarize the evolution of a living donor liver transplant program and the authors' experience with 109 cases. The authors' institution began to offer…”
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Liver Receptor Homologue-1 Mediates Species- and Cell Line-specific Bile Acid-dependent Negative Feedback Regulation of the Apical Sodium-dependent Bile Acid Transporter by Chen, Frank, Ma, Lin, Dawson, Paul A., Sinal, Christopher J., Sehayek, Ephraim, Gonzalez, Frank J., Breslow, Jan, Ananthanarayanan, M., Shneider, Benjamin L.

“…Intestinal reclamation of bile salts is mediated in large part by the apical sodium-dependent bile acid transporter (ASBT). The bile acid responsiveness of…”
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Rapamycin Successfully Treats Post‐Transplant Autoimmune Hepatitis by Kerkar, Nanda, Dugan, Christina, Rumbo, Carolina, Morotti, Raffaella A., Gondolesi, Gabriel, Shneider, Benjamin L., Emre, Sukru

“…Rapamycin (Rapa), one of the newer immunosuppressants has been found to control and prevent autoimmune features in animal models. This is the first report…”
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Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity by Chen, Frank, Ananthanarayanan, M., Emre, Sukru, Neimark, Ezequiel, Bull, Laura N., Knisely, A.S., Strautnieks, Sandra S., Thompson, Richard J., Magid, Margret S., Gordon, Ronald, Balasubramanian, N., Suchy, Frederick J., Shneider, Benjamin L.

“…Background & Aims : The mechanisms by which mutations in the familial intrahepatic cholestasis-1 gene cause Byler’s disease (progressive familial intrahepatic…”
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ATPase Class I Type 8B Member 1 and Protein Kinase C ζ Induce the Expression of the Canalicular Bile Salt Export Pump in Human Hepatocytes by Chen, Frank, Ellis, Ewa, Strom, Stephen C, Shneider, Benjamin L

“…The exact molecular mechanism(s) of the disease that results from defects in the ATPase Class I Type 8B Member 1 gene remains controversial. Prior…”
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National and regional analysis of exceptions to the pediatric end‐stage liver disease scoring system (2003–2004) by Shneider, Benjamin L., Suchy, Frederick J., Emre, Sukru

“…Since February 2002, the Pediatric End Stage Liver Disease (PELD) scoring system has been utilized as a means of prioritizing children for liver…”
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Comparison of Parent and Child Reports of Emotional Trauma Symptoms in Pediatric Outpatient Settings by Shemesh, Eyal, Newcorn, Jeffrey H, Rockmore, Lori, Shneider, Benjamin L, Emre, Sukru, Gelb, Bruce D, Rapaport, Robert, Noone, Sally A, Annunziato, Rachel, Schmeidler, James, Yehuda, Rachel

“…Exposure to emotionally traumatic events is common among children who are treated in pediatric medical care settings, and it is important to recognize the…”
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Intrahepatic cholestasis : Summary of an American association for the study of liver diseases single- topic conference by BALISTRERI, William F, BEZERRA, Jorge A, JANSEN, Peter, KARPEN, Saul J, SHNEIDER, Benjamin L, SUCHY, Frederick J

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Liver disease in autosomal recessive polycystic kidney disease by Shneider, Benjamin L., Magid, Margret S.

“…:  Hepatic complications occur in a significant proportion of children with autosomal recessive polycystic kidney disease (ARPKD). PKHD1/fibrocystin, the…”
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Hepatic Overexpression of Murine Abcb11 Increases Hepatobiliary Lipid Secretion and Reduces Hepatic Steatosis by Figge, Anne, Lammert, Frank, Paigen, Beverly, Henkel, Anne, Matern, Siegfried, Korstanje, Ron, Shneider, Benjamin L., Chen, Frank, Stoltenberg, Erik, Spatz, Kathryn, Hoda, Farzana, Cohen, David E., Green, Richard M.

“…Abcb11 encodes for the liver bile salt export pump, which is rate-limiting for hepatobiliary bile salt secretion. We employed transthyretin-Abcb11 and…”
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A Pilot Study of Posttraumatic Stress and Nonadherence in Pediatric Liver Transplant Recipients by Shemesh, Eyal, Lurie, Susan, Stuber, Margaret L, Emre, Sukru, Patel, Yogita, Vohra, Pankaj, Aromando, Marie, Shneider, Benjamin L

“…Symptoms of posttraumatic stress disorder (PTSD) were described in survivors of life-threatening diseases, the trauma being the experiences associated with the…”
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FXR-activating ligands inhibit rabbit ASBT expression via FXR-SHP-FTF cascade by Li, Hai, Chen, Frank, Shang, Quan, Pan, Luxing, Shneider, Benjamin L, Chiang, John Y L, Forman, Barry M, Ananthanarayanan, M, Tint, G Stephen, Salen, Gerald, Xu, Guorong

“…The regulation of the rabbit apical sodium-dependent bile acid transporter (ASBT) was studied both in vivo and in vitro. New Zealand White rabbits were fed…”
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NATIVE LIVER SURVIVAL IN PATIENTS WITH FIC1 DEFICIENCY: IMPACT OF GENOTYPE, SERUM BILE ACID CONCENTRATIONS AND SURGICAL BILIARY DIVERSION by van Wessel, D, Thompson, RJ, Gonzales, EM, Jankowska, I, Shneider, BL, Sokal, E, Grammatikopoulos, T, Kadaristiana, A, Jacquemin, E, Spraul, A, Lipinski, P, Czubkowski, P, Rock, N, Shagrani, M, Broering, D, Algoufi, T, Mazhar, N, Nicastro, E, Kelly, D, Nebbia, G, Arnell, H, Fischler, B, Hulscher, J, Serranti, D, Arikan, C, Polat, E, Debray, D, Lacaille, F, Goncalves, C, Hierro, L, Bartolo, GM, Mozer-Glassberg, Y, Azaz, A, Brecelj, J, Dezsofi, A, Calvo, PL, Grabhorn, E, Hartleif, S, Van der Woerd, W, Wang, JS, Li, LT, Durmaz, O, Kerkar, N, Jorgensen, MH, Fischer, RT, Jimenez-Rivera, C, Alam, S, Cananzi, M, Laverdure, N, Ferreira, CT, Ferrero, FO, Wang, H, Sency, V, Kim, KM, Chen, HL, Carvalho, E, Fabre, A, Bernabeu, JQ, Alonso, EM, Sokol, RJ, Suchy, FJ, Loomes, KM, McKiernan, PJ, Rosenthal, P, Turmelle, YP, Rao, GS, Horslen, SP, Kamath, BM, Rogalidou, M, Karnsakul, W, Hansen, BE, Verkade, HJ

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THE PRESENCE OF A TRUNCATING MUTATION IN ABCB11 ABROGATES THE BENEFICIAL EFFECT OF A RESIDUAL FUNCTION MUTATION ON THE COURSE OF SEVERE BILE SALT EXPORT PUMP DEFICIENCY by Felzen, A, van Wessel, D, Thompson, RJ, Gonzales, EM, Jankowska, I, Shneider, BL, Sokal, E, Grammatikopoulos, T, Kadaristiana, A, Jacquemin, E, Spraul, A, Lipinski, P, Czubkowski, P, Rock, N, Shagrani, M, Broering, D, Algoufi, T, Mazhar, N, Nicastro, E, Kelly, D, Nebbia, G, Arnell, H, Fischler, B, Hulscher, J, Serranti, D, Arikan, C, Polat, E, Debray, D, Lacaille, F, Goncalves, C, Hierro, L, Bartolo, GM, Mozer-Glassberg, Y, Azaz, A, Brecelj, J, Dezsofi, A, Calvo, PL, Grabhorn, E, Hartleif, S, Van der Woerd, W, Kamath, BM, Wang, JS, Li, LT, Durmaz, O, Kerkar, N, Jorgensen, MH, Fischer, RT, Jimenez-Rivera, C, Alam, S, Cananzi, M, Ruiz, M, Ferreira, CT, Ferrero, FO, Wang, H, Sency, V, Kim, KM, Chen, HL, Carvalho, E, Fabre, A, Bernabeu, JQ, Alonso, EM, Sokol, RJ, Suchy, FJ, Loomes, KM, McKiernan, PJ, Rosenthal, P, Turmelle, Y, Horslen, SP, Schwarz, KB, Bezerra, JA, Wang, KS, Hansen, BE, Verkade, HJ

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Critical analysis of the pediatric end‐stage liver disease scoring system: A single center experience by Shneider, Benjamin L., Neimark, Ezequiel, Frankenberg, Tamara, Arnott, Lindsay, Suchy, Frederick J., Emre, Sukru

“…The Pediatric End‐Stage Liver Disease (PELD) scoring system is a new nationally utilized formula developed to provide a continuous numerical assessment of the…”
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