Search

Your search keyword '"Horslen, S."' showing total 268 results
Start Over Author "Horslen, S."
268 results on '"Horslen, S."'

1. Outcome of Children with Transjugular Intrahepatic Portosystemic Shunt: A Meta-Analysis of Individual Patient Data

Author

Deniz, S, Schinner, R, Monroe, E, Horslen, S, Srinivasa, R, Lv, Y, Fan, D, Han, G, Sarma, M, Srivastava, A, Poddar, U, Yadav, R, Hoang, T, Lange, C, Ocal, O, Ricke, J, Seidensticker, M, Lurz, E, Di Giorgio, A, D'Antiga, L, Wildgruber, M, Deniz S., Schinner R., Monroe E. J., Horslen S., Srinivasa R. N., Lv Y., Fan D., Han G., Sarma M. S., Srivastava A., Poddar U., Yadav R., Hoang T. P. T., Lange C. M., Ocal O., Ricke J., Seidensticker M., Lurz E., Di Giorgio A., D'Antiga L., Wildgruber M., Deniz, S, Schinner, R, Monroe, E, Horslen, S, Srinivasa, R, Lv, Y, Fan, D, Han, G, Sarma, M, Srivastava, A, Poddar, U, Yadav, R, Hoang, T, Lange, C, Ocal, O, Ricke, J, Seidensticker, M, Lurz, E, Di Giorgio, A, D'Antiga, L, Wildgruber, M, Deniz S., Schinner R., Monroe E. J., Horslen S., Srinivasa R. N., Lv Y., Fan D., Han G., Sarma M. S., Srivastava A., Poddar U., Yadav R., Hoang T. P. T., Lange C. M., Ocal O., Ricke J., Seidensticker M., Lurz E., Di Giorgio A., D'Antiga L., and Wildgruber M.

Abstract

Purpose: The purpose of the study was to investigate outcome after pediatric transjugular intrahepatic portosystemic shunt (TIPS) with respect to survival Material and Methods: After searching for studies on TIPS in children in Ovid, Medline, Embase, Scopus and Cochrane published between 2000 and 2022, individual patient data were retrieved from five retrospective cohorts. Overall survival (OS) and transplant-free survival (TFS) were calculated using Kaplan–Meier analysis and log-rank test and compared to the indication (ascites vs. variceal bleeding) as well as to the level of obstruction (pre-hepatic vs. hepatic vs. post-hepatic). Additionally, TIPS patency was analyzed. Results: n = 135 pediatric patients were included in the final analysis. Indication for pediatric TIPS creation was heterogeneous among the included studies. TIPS patency decreased from 6 to 24 months, subsequent pediatric liver transplantation was performed in 22/135 (16.3%) of cases. The presence of ascites was related with poorer TFS (HR 2.3, p = 0.023), while variceal bleeding was not associated with impaired survival. Analysis of the level of obstruction (pre-hepatic, hepatic and post-hepatic) failed to prove significantly reduced OS for post-hepatic obstruction (HR 3.2, p = 0.092) and TFS (HR 1.3, p = 0.057). There was no difference in OS and TFS according to age at time of TIPS placement. Conclusions: The presence of ascites associates with impaired survival after TIPS in children, with no differences in survival according to the age of the child. Interventional shunt procedures can be considered feasible for all ages. Level of Evidence: Level 2a. Graphical Abstract: [Figure not available: see fulltext.]

Published
2023

2. Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency

Author

Felzen, A. van Wessel, D.B.E. Gonzales, E. Thompson, R.J. Jankowska, I. Shneider, B.L. Sokal, E. Grammatikopoulos, T. Kadaristiana, A. Jacquemin, E. Spraul, A. Lipiński, P. Czubkowski, P. Rock, N. Shagrani, M. Broering, D. Nicastro, E. Kelly, D. Nebbia, G. Arnell, H. Fischler, B. Hulscher, J.B.F. Serranti, D. Arikan, C. Polat, E. Debray, D. Lacaille, F. Goncalves, C. Hierro, L. Muñoz Bartolo, G. Mozer-Glassberg, Y. Azaz, A. Brecelj, J. Dezsőfi, A. Calvo, P.L. Grabhorn, E. Hartleif, S. van der Woerd, W.J. Kamath, B.M. Wang, J.-S. Li, L. Durmaz, Ö. Kerkar, N. Jørgensen, M.H. Fischer, R. Jimenez-Rivera, C. Alam, S. Cananzi, M. Laverdure, N. Ferreira, C.T. Guerrero, F.O. Wang, H. Sency, V. Kim, K.M. Chen, H.-L. de Carvalho, E. Fabre, A. Bernabeu, J.Q. Zellos, A. Alonso, E.M. Sokol, R.J. Suchy, F.J. Loomes, K.M. McKiernan, P.J. Rosenthal, P. Turmelle, Y. Horslen, S. Schwarz, K. Bezerra, J.A. Wang, K. Hansen, B.E. Verkade, H.J. the NAtural course Prognosis of PFIC Effect of biliary Diversion (NAPPED) Consortium and Felzen, A. van Wessel, D.B.E. Gonzales, E. Thompson, R.J. Jankowska, I. Shneider, B.L. Sokal, E. Grammatikopoulos, T. Kadaristiana, A. Jacquemin, E. Spraul, A. Lipiński, P. Czubkowski, P. Rock, N. Shagrani, M. Broering, D. Nicastro, E. Kelly, D. Nebbia, G. Arnell, H. Fischler, B. Hulscher, J.B.F. Serranti, D. Arikan, C. Polat, E. Debray, D. Lacaille, F. Goncalves, C. Hierro, L. Muñoz Bartolo, G. Mozer-Glassberg, Y. Azaz, A. Brecelj, J. Dezsőfi, A. Calvo, P.L. Grabhorn, E. Hartleif, S. van der Woerd, W.J. Kamath, B.M. Wang, J.-S. Li, L. Durmaz, Ö. Kerkar, N. Jørgensen, M.H. Fischer, R. Jimenez-Rivera, C. Alam, S. Cananzi, M. Laverdure, N. Ferreira, C.T. Guerrero, F.O. Wang, H. Sency, V. Kim, K.M. Chen, H.-L. de Carvalho, E. Fabre, A. Bernabeu, J.Q. Zellos, A. Alonso, E.M. Sokol, R.J. Suchy, F.J. Loomes, K.M. McKiernan, P.J. Rosenthal, P. Turmelle, Y. Horslen, S. Schwarz, K. Bezerra, J.A. Wang, K. Hansen, B.E. Verkade, H.J. the NAtural course Prognosis of PFIC Effect of biliary Diversion (NAPPED) Consortium

Abstract

Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p <0.001). Without siEHC, NLS in the BSEP1/3 group was similar to that in BSEP3/3, but considerably lower than in BSEP1/1 (at age 10 years: 38%, 30%, and 71%, respectively; p = 0.003). After siEHC, BSEP1/3 and BSEP3/3 were associated with similarly low NLS, while NLS was much higher in BSEP1/1 (10 years after siEHC, 27%, 14%, and 92%, respectively; p <0.001). Conclusions: Individuals with BSEP deficiency with one p.E297G or p.D482G mutation and one PPTM have a similarly severe disease course and low responsiveness to siEHC as those with two PPTMs. This identifies a considerable subgroup of patients who are unlikely to benefit from interruption of the enterohepatic circulation by either surgical or ileal bile acid transporter inhibitor treatment. Impact and implications: This manuscript defines the clinical features and prognosis of individuals with BSEP deficiency involving the combination of one relatively mild and one very severe BSEP deficiency mutat

Published
2023

3. Solid Organ Transplantation From Hepatitis B Virus–Positive Donors: Consensus Guidelines for Recipient Management

Author

Huprikar, S., Danziger-Isakov, L., Ahn, J., Naugler, S., Blumberg, E., Avery, R.K., Koval, C., Lease, E.D., Pillai, A., Doucette, K.E., Levitsky, J., Morris, M.I., Lu, K., McDermott, J.K., Mone, T., Orlowski, J.P., Dadhania, D.M., Abbott, K., Horslen, S., Laskin, B.L., Mougdil, A., Venkat, V.L., Korenblat, K., Kumar, V., Grossi, P., Bloom, R.D., Brown, K., Kotton, C.N., and Kumar, D.

Published
2015
Full Text
View/download PDF

8. Intestine

Author

Smith, J. M., Skeans, M. A., Horslen, S. P., Edwards, E. B., Harper, A. M., Snyder, J. J., Israni, A. K., and Kasiske, B. L.

Published
2016
Full Text
View/download PDF

11. Similarities and differences in allocation policies for pediatric liver transplantation across the world

Author

Fischler, B, Baumann, U, D'Agostino, D, D'Antiga, L, Dezsofi, A, Debray, D, Durmaz, O, Evans, H, Frauca, E, Hadzic, N, Jahnel, J, Loveland, J, Mclin, V, Ng, V, Nobili, V, Pawłowska, J, Sharif, K, Smets, F, Verkade, H, Hsu, E, Horslen, S, Bucuvalas, J, Fischler B., Baumann U., D'Agostino D., D'Antiga L., Dezsofi A., Debray D., Durmaz O., Evans H., Frauca E., Hadzic N., Jahnel J., Loveland J., McLin V., Ng V. L., Nobili V., Pawłowska J., Sharif K., Smets F., Verkade H. J., Hsu E., Horslen S., Bucuvalas J., Fischler, B, Baumann, U, D'Agostino, D, D'Antiga, L, Dezsofi, A, Debray, D, Durmaz, O, Evans, H, Frauca, E, Hadzic, N, Jahnel, J, Loveland, J, Mclin, V, Ng, V, Nobili, V, Pawłowska, J, Sharif, K, Smets, F, Verkade, H, Hsu, E, Horslen, S, Bucuvalas, J, Fischler B., Baumann U., D'Agostino D., D'Antiga L., Dezsofi A., Debray D., Durmaz O., Evans H., Frauca E., Hadzic N., Jahnel J., Loveland J., McLin V., Ng V. L., Nobili V., Pawłowska J., Sharif K., Smets F., Verkade H. J., Hsu E., Horslen S., and Bucuvalas J.

Abstract

Objectives:We aimed to investigate national allocation policies for pediatric liver transplantation (LT).Method:A survey was prepared by the European Society for Paediatric Gastroenterology Hepatology and Nutrition Hepatology Committee in collaboration with the North American Studies of Pediatric Liver Transplantation consortium. The survey was sent to pediatric hepatologists and transplant surgeons worldwide. National data were obtained from centrally based registries.Results:Replies were obtained from 15 countries from 5 of the world continents. Overall donation rate varied between 9 and 35 per million inhabitants. The number of pediatric LTs was 4 to 9 per million inhabitants younger than 18 years for 13 of the 15 respondents. In children younger than 2 years mortality on the waiting list (WL) varied between 0 and 20%. In the same age group, there were large differences in the ratio of living donor LT to deceased donor LT and in the ratio of split liver segments to whole liver. These differences were associated with possible discrepancies in WL mortality.Conclusions:Similarities but also differences between countries were detected. The described data may be of importance when trying to reduce WL mortality in the youngest children.

Published
2019

12. The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children

Author

Deneau, M.R. Mack, C. Perito, E.R. Ricciuto, A. Valentino, P.L. Amin, M. Amir, A.Z. Aumar, M. Auth, M. Broderick, A. DiGuglielmo, M. Draijer, L.G. Tavares Fagundes, E.D. El-Matary, W. Ferrari, F. Furuya, K.N. Gupta, N. Hochberg, J.T. Homan, M. Horslen, S. Iorio, R. Jensen, M.K. Jonas, M.M. Kamath, B.M. Kerkar, N. Kim, K.M. Kolho, K.-L. Koot, B.G.P. Laborda, T.J. Lee, C.K. Loomes, K.M. Martinez, M. Miethke, A. Miloh, T. Mogul, D. Mohammad, S. Mohan, P. Moroz, S. Ovchinsky, N. Palle, S. Papadopoulou, A. Rao, G. Rodrigues Ferreira, A. Sathya, P. Schwarz, K.B. Shah, U. Shteyer, E. Singh, R. Smolka, V. Soufi, N. Tanaka, A. Varier, R. Vitola, B. Woynarowski, M. Zerofsky, M. Zizzo, A. Guthery, S.L.

Abstract

Background and Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC. Approach and Results: We used retrospective data from the Pediatric PSC Consortium. The training cohort contained 1,012 patients from 40 centers. We generated a multivariate risk index (Sclerosing Cholangitis Outcomes in Pediatrics [SCOPE] index) that contained total bilirubin, albumin, platelet count, gamma glutamyltransferase, and cholangiography to predict a primary outcome of liver transplantation or death (TD) and a broader secondary outcome that included portal hypertensive, biliary, and cancer complications termed hepatobiliary complications (HBCs). The model stratified patients as low, medium, or high risk based on progression to TD at rates of

Published
2021

13. Impact of genotype, serum bile acids, and surgical biliary diversion on native liver survival in FIC1 deficiency

Author

Arıkan, Çiğdem (ORCID 0000-0002-0794-2741 & YÖK ID 240198), van Wessel, D. B. E.; Thompson, R. J.; Gonzales, E.; Jankowska, I.; Shneider, B. L.; Sokal, E.; Grammatikopoulos, T.; Kadaristiana, A.; Jacquemin, E.; Spraul, A.; Lipiński, 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. B. F.; Serranti, D.; Debray, D.; Lacaille, F.; Goncalves, C.; Hierro, L.; Muñoz Bartolo, G.; Mozer-Glassberg, Y.; Azaz, A.; Brecelj, J.; Dezsőfi, A.; Luigi Calvo, P.; Krebs-Schmitt, D.; Hartleif, S.; van der Woerd, W. L.; Wang, J. S.; Li, L. T.; Durmaz, Ö.; Kerkar, N.; Hørby Jørgensen, M.; Fischer, R.; Jimenez-Rivera, C.; Alam, S.; Cananzi, M.; Laverdure, N.; Ferreira, C. T.; Ordonez, F.; Wang, H.; Sency, V.; Kim, K. M.; Chen, H. L.; Carvalho, E.; Fabre, A.; Quintero Bernabeu, J.; Alonso, E. M.; Sokol, R. J.; Suchy, F. J.; Loomes, K. M.; McKiernan, P. J.; Rosenthal, P.; Turmelle, Y.; Rao, G. S.; Horslen, S.; Kamath, B. M.; Rogalidou, M.; Karnsakul, W. W.; Hansen, B.; Verkade, H. J., Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM), School of Medicine, Arıkan, Çiğdem (ORCID 0000-0002-0794-2741 & YÖK ID 240198), van Wessel, D. B. E.; Thompson, R. J.; Gonzales, E.; Jankowska, I.; Shneider, B. L.; Sokal, E.; Grammatikopoulos, T.; Kadaristiana, A.; Jacquemin, E.; Spraul, A.; Lipiński, 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. B. F.; Serranti, D.; Debray, D.; Lacaille, F.; Goncalves, C.; Hierro, L.; Muñoz Bartolo, G.; Mozer-Glassberg, Y.; Azaz, A.; Brecelj, J.; Dezsőfi, A.; Luigi Calvo, P.; Krebs-Schmitt, D.; Hartleif, S.; van der Woerd, W. L.; Wang, J. S.; Li, L. T.; Durmaz, Ö.; Kerkar, N.; Hørby Jørgensen, M.; Fischer, R.; Jimenez-Rivera, C.; Alam, S.; Cananzi, M.; Laverdure, N.; Ferreira, C. T.; Ordonez, F.; Wang, H.; Sency, V.; Kim, K. M.; Chen, H. L.; Carvalho, E.; Fabre, A.; Quintero Bernabeu, J.; Alonso, E. M.; Sokol, R. J.; Suchy, F. J.; Loomes, K. M.; McKiernan, P. J.; Rosenthal, P.; Turmelle, Y.; Rao, G. S.; Horslen, S.; Kamath, B. M.; Rogalidou, M.; Karnsakul, W. W.; Hansen, B.; Verkade, H. J., Koç University Research Center for Translational Medicine (KUTTAM) / Koç Üniversitesi Translasyonel Tıp Araştırma Merkezi (KUTTAM), and School of Medicine

Abstract

University of Colorado; Baylor College of Medicine; Children’s Hospital of Philadelphia; Children’s Hospital of Pittsburgh; St Louis Children’s Hospital; Riley Hospital for Children; Seattle Children’s Hospital; M.D./Ph.D. Scholarship from the University of Groningen; European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Networking Grant 2019; ChilDReN National Institutes of Health Grants; Ann & Robert H. Lurie Children’s Hospital; Albireo and Mirum Pharmaceuticals

Published
2021

21. Indications for lung transplantation

Author

Dunn, S, Horslen, S, Dunn, S ( S ), Horslen, S ( S ), Benden, C, Josephson, M, Hanna, B, Dunn, S, Horslen, S, Dunn, S ( S ), Horslen, S ( S ), Benden, C, Josephson, M, and Hanna, B

Abstract

Pediatric lung transplant is nowadays an accepted therapy in well-selected children with end-stage pulmonary disease, offering prolonged overall survival and better quality of life. Over 100 pediatric lung transplants are reported to the International Society for Heart and Lung Transplantation (ISHLT) Thoracic Transplant Registry annually. In order to maximize posttransplant survival in children, careful candidate selection is absolutely crucial. Primary diagnostic indications for lung transplantation in children is cystic fibrosis (CF) pulmonary disease overall, followed by pulmonary hypertension and interstitial lung disease and obliterative bronchiolitis. However, there is a varying distribution of diagnostic indications depending on children’s ages. In younger children, surfactant protein deficiencies, congenital heart disease, and idiopathic pulmonary arterial hypertension are the commonest diagnoses, respectively. In older children, CF is the most frequent indication for lung transplantation. Further, marked regional differences exist worldwide regarding indications, reflecting diverse referral patterns and differences in disease management. In North America, half of the children undergoing lung transplantation have CF; in Europe, over two-thirds of children suffer with CF. Today, the ISHLT has international consensus guidelines in place, including general guidance on the selection of pediatric candidates and disease-specifics on referral and timing for listing for lung transplantation

Published
2018

26. Early and Late Factors Impacting Patient and Graft Outcome in Pediatric Liver Transplantation: Summary of an ESPGHAN Monothematic Conference

Author

Mclin, V, Allen, U, Boyer, O, Bucuvalas, J, Colledan, M, Cuturi, M, D'Antiga, L, Debray, D, Dezsofi, A, de Goyet, J, Dhawan, A, Durmaz, O, Falk, C, Feng, S, Fischler, B, Franchi-Abella, S, Frauca, E, Ganschow, R, Gottschalk, S, Hadzic, N, Hierro, L, Horslen, S, Hubscher, S, Karam, V, Kelly, D, Maecker-Kolhoff, B, Mazariegos, G, Mckiernan, P, Melk, A, Nobili, V, Ozgenc, F, Reding, R, Sciveres, M, Sharif, K, Socha, P, Toso, C, Vajro, P, Verma, A, Wildhaber, B, Baumann, U, Mclin VA, Allen U, Boyer O, Bucuvalas J, Colledan M, Cuturi MC, d'Antiga L, Debray D, Dezsofi A, de Goyet JD, Dhawan A, Durmaz O, Falk C, Feng S, Fischler B, Franchi-Abella S, Frauca E, Ganschow R, Gottschalk S, Hadzic N, Hierro L, Horslen S, Hubscher S, Karam V, Kelly D, Maecker-Kolhoff B, Mazariegos G, McKiernan P, Melk A, Nobili V, Ozgenc F, Reding R, Sciveres M, Sharif K, Socha P, Toso C, Vajro P, Verma A, Wildhaber BE, Baumann U, Mclin, V, Allen, U, Boyer, O, Bucuvalas, J, Colledan, M, Cuturi, M, D'Antiga, L, Debray, D, Dezsofi, A, de Goyet, J, Dhawan, A, Durmaz, O, Falk, C, Feng, S, Fischler, B, Franchi-Abella, S, Frauca, E, Ganschow, R, Gottschalk, S, Hadzic, N, Hierro, L, Horslen, S, Hubscher, S, Karam, V, Kelly, D, Maecker-Kolhoff, B, Mazariegos, G, Mckiernan, P, Melk, A, Nobili, V, Ozgenc, F, Reding, R, Sciveres, M, Sharif, K, Socha, P, Toso, C, Vajro, P, Verma, A, Wildhaber, B, Baumann, U, Mclin VA, Allen U, Boyer O, Bucuvalas J, Colledan M, Cuturi MC, d'Antiga L, Debray D, Dezsofi A, de Goyet JD, Dhawan A, Durmaz O, Falk C, Feng S, Fischler B, Franchi-Abella S, Frauca E, Ganschow R, Gottschalk S, Hadzic N, Hierro L, Horslen S, Hubscher S, Karam V, Kelly D, Maecker-Kolhoff B, Mazariegos G, McKiernan P, Melk A, Nobili V, Ozgenc F, Reding R, Sciveres M, Sharif K, Socha P, Toso C, Vajro P, Verma A, Wildhaber BE, and Baumann U

Abstract

As pediatric liver transplantation comes of age, experts gathered to discuss current paradigms and define gaps in knowledge warranting research to further improve patient and graft outcomes. Identified areas ripe for collaborative research include understanding the molecular and cellular mechanisms of tolerance and the role of donor-specific antibodies, considering ways to expand donor pool, minimizing long-term side effects of immunosuppression, and fine-tuning surgical techniques to minimize biliary and vascular complications. Copyright © 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

Published
2017

27. Clinically Actionable Hypercholesterolemia and Hypertriglyceridemia in Children with Nonalcoholic Fatty Liver Disease

Author

Harlow, KE, Africa, JA, Wells, A, Belt, PH, Behling, CA, Jain, AK, Molleston, JP, Newton, KP, Rosenthal, P, Vos, MB, Xanthakos, SA, Lavine, JE, Schwimmer, JB, Abrams, SH, Barlow, S, Himes, R, Krisnamurthy, R, Maldonado, L, Mahabir, R, Carr, A, Bernstein, K, Bramlage, K, Cecil, K, DeVore, S, Kohli, R, Lake, K, Podberesky, D, Towbin, A, Behr, G, Lefkowitch, JH, Mencin, A, Reynoso, E, Alazraki, A, Cleeton, R, Cordero, M, Hernandez, A, Karpen, S, Munos, JC, Raviele, N, Bozic, M, Cummings, OW, Klipsch, A, Ragozzino, E, Sandrasegaran, K, Subbarao, G, Walker, L, Kafka, K, Scheimann, A, Ito, J, Fishbein, MH, Mohammad, S, Rigsby, C, Sharda, L, Whitington, PF, Cattoor, T, Derdoy, J, Freebersyser, J, King, D, Lai, J, Osmack, P, Siegner, J, Stewart, S, Torretta, S, Wriston, K, Baker, SS, Lopez-Graham, D, Williams, S, Zhu, L, Awai, H, Bross, C, Collins, J, Durelle, J, Middleton, M, Paiz, M, Sirlin, C, Ugalde-Nicalo, P, Villarreal, MD, Aouizerat, B, Courtier, J, Ferrell, LD, Feier, N, Gill, R, Langlois, C, Perito, ER, Tsai, P, Cooper, K, Horslen, S, Hsu, E, Murray, K, Otto, R, Yeh, M, Young, M, Brunt, EM, and Fowler, K

Abstract

© 2018 Elsevier Inc. Objective: To determine the percentage of children with nonalcoholic fatty liver disease (NAFLD) in whom intervention for low-density lipoprotein cholesterol or triglycerides was indicated based on National Heart, Lung, and Blood Institute guidelines. Study design: This multicenter, longitudinal cohort study included children with NAFLD enrolled in the National Institute of Diabetes and Digestive and Kidney Diseases Nonalcoholic Steatohepatitis Clinical Research Network. Fasting lipid profiles were obtained at diagnosis. Standardized dietary recommendations were provided. After 1 year, lipid profiles were repeated and interpreted according to National Heart, Lung, and Blood Institute Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction. Main outcomes were meeting criteria for clinically actionable dyslipidemia at baseline, and either achieving lipid goal at follow-up or meeting criteria for ongoing intervention. Results: There were 585 participants, with a mean age of 12.8 years. The prevalence of children warranting intervention for low-density lipoprotein cholesterol at baseline was 14%. After 1 year of recommended dietary changes, 51% achieved goal low-density lipoprotein cholesterol, 27% qualified for enhanced dietary and lifestyle modifications, and 22% met criteria for pharmacologic intervention. Elevated triglycerides were more prevalent, with 51% meeting criteria for intervention. At 1 year, 25% achieved goal triglycerides with diet and lifestyle changes, 38% met criteria for advanced dietary modifications, and 37% qualified for antihyperlipidemic medications. Conclusions: More than one-half of children with NAFLD met intervention thresholds for dyslipidemia. Based on the burden of clinically relevant dyslipidemia, lipid screening in children with NAFLD is warranted. Clinicians caring for children with NAFLD should be familiar with lipid management.

Published
2018
Full Text
View/download PDF

31. OPTN/SRTR 2020 Annual Data Report: Intestine

Author

Horslen, S. P., Smith, J. M., Weaver, T., Cafarella, M., and Foutz, J.

Abstract

Despite small increases in additions to the intestine transplant wait‐ list, total waitlist numbers, overall intestine transplant rates, and overall transplants performed from 2019 to 2020, the trend over the last decade is still toward less intestine transplant activity. Waitlist mortality continues to fall for pediatric populations and is relatively stable for adults. While 1‐ year graft survival continues to improve, there has been no noticeable improvement in 3‐ and 5‐year graft survival. Immunosuppression practices continue to favor use of an induction agent followed by tacrolimus‐based regimens. Patient survival at 5 years is currently identical for isolated intestines and liver‐inclusive allograft recipients.

Published
2022
Full Text
View/download PDF

32. OPTN/SRTR 2019 Annual Data Report: Intestine

Author

Horslen, S. P., Smith, J. M., Ahn, Y., Skeans, M. A., Cafarella, M., Noreen, S. M., Snyder, J. J., and Israni, A. K.

Abstract

Intestine transplant can be life‐saving and can improve quality of life for patients with intestinal failure. Medical and surgical advances in treatment of intestinal failure over the past 10 to 15 years have resulted in fewer patients being added to the waiting list for intestine transplant alone or for intestine transplant in combination with liver transplant (and sometimes other organs). Consequently, fewer transplants are being performed. The numbers of listings and transplants fell to new lows in 2019. The number of programs performing transplants in at least one patient in 2019 was the lowest in the last decade, equal to 2014, at 15. Graft failure plateaued over the past decade, but early graft loss has increased in the past 2 years, notably in recipients of a combined liver and intestine allograft. Five‐year patient survival for transplants in 2012‐2014 varied little by graft type.

Published
2021
Full Text
View/download PDF

33. Estimation of fish and ω-3 fatty acid intake in pediatric nonalcoholic fatty liver disease

Author

St-Jules, DE, Watters, CA, Brunt, EM, Wilkens, LR, Novotny, R, Belt, P, Lavine, JE, Abrams, SH, Himes, R, Krisnamurthy, R, Maldonado, L, Morris, B, Bernstein, K, Cecil, K, DeVore, S, Kohli, R, Lake, K, Podberesky, D, Slaughter, C, Xanthakos, S, Behr, G, Mencin, A, Ovchinsky, N, Reynoso, E, Alazraki, A, Cleeton, R, Karpen, S, Raviele, N, Vos, M, Byam, E, Cummings, OW, Fleming, C, Klipsch, A, Molleston, JP, Sandrasegaran, K, Subbarao, G, Pfeifer, K, Scheimann, A, Torbenson, M, Clark, JM, Corless, E, Donithan, M, Isaacson, M, May, KP, Miriel, L, Sternberg, A, Tonascia, J, Ünalp-Arida, A, Van Natta, M, Vaughn, I, Wilson, L, Yates, K, Arnon, R, Boyd, M, Amsden, K, Fishbein, MH, Kirwan, E, Mohammad, S, Quinn, A, Rigsby, C, Whitington, PF, Barlow, S, Derdoy, J, Jain, A, King, D, Osmack, P, Siegner, J, Stewart, S, Romo, D, Arroyo, S, Behling, C, Collins, J, Durelle, J, Middleton, M, Newton, K, Paiz, M, Schwimmer, JB, Sirlin, C, Ugalde-Nicalo, P, Aouizerat, B, Ferrell, LD, Fleck, S, Gill, R, Langlois, C, Perito, ER, Rosenthal, P, Tsai, P, Cooper, K, Horslen, S, Hsu, E, Murray, K, Otto, R, Rich, D, Yeh, M, Young, M, and Fowler, K

Abstract

AIMS:: Fish and ω-3 fatty acids are reported to be beneficial in pediatric nonalcoholic fatty liver disease (NAFLD), but no studies have assessed their relation to histological severity. The objectives of this study were to evaluate the dietary intake of fish and ω-3 fatty acids in children with biopsy-proven NAFLD, and examine their association with serological and histological indicators of disease. METHODS:: This was a cross-sectional analysis of 223 children (6-18 years) who participated in the Treatment of Nonalcoholic Fatty Liver Disease in Children trial or the NAFLD Database study conducted by the Nonalcoholic Steatohepatitis Clinical Research Network. The distribution of fish and ω-3 fatty acid intake was determined from responses to the Block Brief 2000 Food Frequency Questionnaire, and analyzed for associations with serum alanine aminotransferase, histological features of fatty liver disease, and diagnosis of steatohepatitis after adjusting for demographic, anthropometric, and dietary variables. RESULTS:: The minority of subjects consumed the recommended 8 ounces of fish per week (22/223 [10%]) and 200 mg of long-chain ω-3 fatty acids per day (12/223 [5%]). Lack of fish and long-chain ω-3 fatty acid intake was associated with greater portal (P=0.03 and P=0.10, respectively) and lobular inflammation (P=0.09 and P=0.004, respectively) after controlling for potential confounders. CONCLUSIONS:: Fish and ω-3 fatty acid intake was insufficient in children with NAFLD, which may increase susceptibility to hepatic inflammation. Patients with pediatric NAFLD should be encouraged to consume the recommended amount of fish per week. Copyright 2013 by ESPGHAN and NASPGHAN.

Published
2013
Full Text
View/download PDF

34. OPTN/SRTR 2018 Annual Data Report: Intestine

Author

Smith, J. M., Weaver, T., Skeans, M. A., Horslen, S. P., Miller, E., Noreen, S. M., Snyder, J. J., Israni, A. K., and Kasiske, B. L.

Abstract

Despite medical and surgical advances in treatment of intestinal failure, intestine transplant still plays an important role. However, the number of new patients added to the intestine transplant waiting list has decreased over the past decade, reaching a low of 135 in 2018. The number of intestine donors also decreased, reaching a low of 106 in 2018, and the number of intestine transplants performed declined to its lowest level, 104, of which 59% were intestine‐liver transplants. Graft failure has plateaued over the past decade. Patient survival for transplants in 2011‐2013 varied by age and transplant type. Patient survival was lowest for adult intestine‐liver recipients (1‐and 5‐year survival 66.7% and 49.1%, respectively) and highest for pediatric intestine recipients (1‐and 5‐year survival 89.1% and 76.4%, respectively).

Published
2020
Full Text
View/download PDF

43. Dyslipidemia and sustained elevations in transaminases from early childhood are common in lysosomal acid lipase deficiency

Author

Deegan, P., primary, Burton, B., additional, Di Rocco, M., additional, Enns, G., additional, Guardmagna, O., additional, Horslen, S., additional, Hovingh, G., additional, Lobritto, S., additional, Malinova, V., additional, McLin, V., additional, Raiman, J., additional, Santra, S., additional, Sharma, R., additional, Sykut-Cegielska, J., additional, Tripuraneni, R., additional, Valayannopoulos, V., additional, Whitley, C., additional, Eckert, S., additional, and Quinn, A., additional

Published
2014
Full Text
View/download PDF

45. OPTN/SRTR 2017 Annual Data Report: Intestine

Author

Smith, J. M., Weaver, T., Skeans, M. A., Horslen, S. P., Noreen, S. M., Snyder, J. J., Israni, A. K., and Kasiske, B. L.

Abstract

Despite improvements in medical and surgical treatment of intestinal failure, intestine transplant continues to play an important role. In 2017, 109 intestine transplants were performed, 62 in adults and 47 in children, reflecting the changed age distribution over the past decade of candidates waitlisted for intestine and intestine‐liver transplant from largely pediatric to increasing proportions of adults. In 2017, 56.0% of candidates on the intestine list at any time during the year were aged younger than 18 years, with a decrease over time in those aged younger than 6 years and an increase in those aged 6‐17 years. Adults accounted for 44.0% of candidates on the list at any time during the year, with an increase since 2013 in those aged 18‐34 years and a decrease in those aged 35 years or older. By age, the pretransplant mortality rate was highest for adult candidates at 7.9 per 100 waitlist‐years and lowest for pediatric candidates at 3.7 per 100 waitlist‐years. Patient survival varied by age and type of transplant, and was lowest for adult intestine‐liver recipients (1‐ and 5‐year survival 66.7% and 42.6%, respectively) and highest for pediatric intestine recipients (1‐ and 5‐year survival 86.2% and 75.4%, respectively).

Published
2019
Full Text
View/download PDF

50. OPTN/SRTR2016 Annual Data Report: Intestine

Author

Smith, J. M., Weaver, T., Skeans, M. A., Horslen, S. P., Harper, A. M., Snyder, J. J., Israni, A. K., and Kasiske, B. L.

Abstract

Despite improvements in medical and surgical treatment of intestinal failure, intestine transplant continues to play an important role. In 2016, a total of 147 intestine transplants were performed, 80 intestine‐without‐liver and 67 intestine‐liver. Over the past decade, the age distribution of candidates waitlisted for intestine and intestine‐liver transplant shifted from primarily pediatric to increasing proportions of adults. In 2016, 58.2% of candidates on the intestine list at any time during the year were aged younger than 18 years, with a decrease over time in those aged younger than 6 years and an increase in those aged 6‐17 years. Adults accounted for 41.9% of candidates on the list at any time during the year, with a stable proportion of those aged 18‐34 years and a decrease in those aged 35 years or older. By age, pretransplant mortality rate was highest for adult candidates at 11.7 per 100 waitlist years and lowest for children aged younger than 6 years at 2.2 per 100 waitlist years. For intestine transplants with or without a liver in 2009‐2011, 1‐ and 5‐year graft survival was 72.0% and 54.1%, respectively, for recipients aged younger than 18 years, and 70.5% and 44.1%, respectively, for recipients aged 18 years or older.

Published
2018
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results