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1. Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease

Author

Leung, Daniel H, Devaraj, Sridevi, Goodrich, Nathan P, Chen, Xinpu, Rajapakshe, Deepthi, Ye, Wen, Andreev, Victor, Minard, Charles G, Guffey, Danielle, Molleston, Jean P, Bass, Lee M, Karpen, Saul J, Kamath, Binita M, Wang, Kasper S, Sundaram, Shikha S, Rosenthal, Philip, McKiernan, Patrick, Loomes, Kathleen M, Jensen, M Kyle, Horslen, Simon P, Bezerra, Jorge A, Magee, John C, Merion, Robert M, Sokol, Ronald J, Shneider, Benjamin L, Network, The Childhood Liver Disease Research, Alonso, Estella, Bass, Lee, Kelly, Susan, Riordan, Mary, Melin‐Aldana, Hector, Bezerra, Jorge, Bove, Kevin, Heubi, James, Miethke, Alexander, Tiao, Greg, Denlinger, Julie, Chapman, Erin, Sokol, Ronald, Feldman, Amy, Mack, Cara, Narkewicz, Michael, Suchy, Frederick, Van Hove, Johan, Garcia, Benigno, Kauma, Mikaela, Kocher, Kendra, Steinbeiss, Matthew, Lovell, Mark, Piccoli, David, Rand, Elizabeth, Russo, Pierre, Spinner, Nancy, Erlichman, Jessi, Stalford, Samantha, Pakstis, Dina, King, Sakya, Squires, Robert, Sindhi, Rakesh, Venkat, Veena, Bukauskas, Kathy, Haberstroh, Lori, Squires, James, Bull, Laura, Curry, Joanna, Langlois, Camille, Kim, Grace, Teckman, Jeffery, Kociela, Vikki, Nagy, Rosemary, Patel, Shraddha, Cerkoski, Jacqueline, Bozic, Molly, Subbarao, Girish, Klipsch, Ann, Sawyers, Cindy, Cummings, Oscar, Murray, Karen, Hsu, Evelyn, Cooper, Kara, Young, Melissa, Finn, Laura, Ng, Vicky, Quammie, Claudia, Putra, Juan, Sharma, Deepika, Parmar, Aishwarya, Guthery, Stephen, Jensen, Kyle, Rutherford, Ann, Lowichik, Amy, Book, Linda, and Meyers, Rebecka

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Chronic Liver Disease and Cirrhosis, Rare Diseases, Pediatric, Liver Disease, Digestive Diseases, Oral and gastrointestinal, Humans, Child, Liver, Matrix Metalloproteinase 7, Endoglin, Interleukin-8, Cholestasis, Liver Cirrhosis, Liver Diseases, Biomarkers, Alagille Syndrome, Elasticity Imaging Techniques, Childhood Liver Disease Research Network, Medical Biochemistry and Metabolomics, Immunology, Gastroenterology & Hepatology, Clinical sciences
Abstract

Background and aimsDetailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis.Approach and resultsA targeted enzyme-linked immunosorbent assay-based panel of nine biomarkers (lysyl oxidase, tissue inhibitor matrix metalloproteinase (MMP) 1, connective tissue growth factor [CTGF], IL-8, endoglin, periostin, Mac-2-binding protein, MMP-3, and MMP-7) was examined in children with biliary atresia (BA; n = 187), alpha-1 antitrypsin deficiency (A1AT; n = 78), and Alagille syndrome (ALGS; n = 65) and correlated with liver stiffness (LSM) and biochemical measures of liver disease. Median age and LSM were 9 years and 9.5 kPa. After adjusting for covariates, there were positive correlations among LSM and endoglin ( p = 0.04) and IL-8 ( p

Published
2023

4. 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

Teckman, Jeffrey, Rosenthal, Philip, Hawthorne, Kieran, Spino, Cathie, Bass, Lee M, Murray, Karen F, Kerkar, Nanda, Magee, John C, Karpen, Saul, Heubi, James E, Molleston, Jean P, Squires, Robert H, Kamath, Binita M, Guthery, Stephen L, Loomes, Kathleen M, Sherker, Averell H, Sokol, Ronald J, ChiLDReN, Alonso, Estella, Bass, Lee, Kelly, Susan, Riordan, Mary, Melin-Aldana, Hector, Bezerra, Jorge, Bove, Kevin, Heubi, James, Miethke, Alexander, Tiao, Greg, Denlinger, Julie, Chapman, Erin, Sokol, Ronald, Feldman, Amy, Mack, Cara, Narkewicz, Michael, Suchy, Frederick, Sundaram, Shikha, Van Hove, Johan, Garcia, Benigno, Kauma, Mikaela, Kocher, Kendra, Steinbeiss, Matthew, Lovell, Mark, Loomes, Kathleen, Piccoli, David, Rand, Elizabeth, Russo, Pierre, Spinner, Nancy, Erlichman, Jessi, Stalford, Samantha, Pakstis, Dina, King, Sakya, Squires, Robert, Sindhi, Rakesh, Venkat, Veena, Bukauskas, Kathy, McKiernan, Patrick, Haberstroh, Lori, Squires, James, Bull, Laura, Curry, Joanna, Langlois, Camille, Kim, Grace, Teckman, Jeffery, Kociela, Vikki, Nagy, Rosemary, Patel, Shraddha, Cerkoski, Jacqueline, Bozic, Molly, Subbarao, Girish, Klipsch, Ann, Sawyers, Cindy, Cummings, Oscar, Horslen, Simon, Murray, Karen, Hsu, Evelyn, Cooper, Kara, Young, Melissa, Finn, Laura, Kamath, Binita, Ng, Vicky, Quammie, Claudia, Putra, Juan, Sharma, Deepika, Parmar, Aishwarya, Guthery, Stephen, Jensen, Kyle, Rutherford, Ann, Lowichik, Amy, Book, Linda, Meyers, Rebecka, Hall, Tyler, Wang, Kasper, Michail, Sonia, Thomas, Danny, Goodhue, Catherine, Kohli, Rohit, Wang, Larry, and Soufi, Nisreen

Subjects
Paediatrics, Biomedical and Clinical Sciences, Digestive Diseases, Liver Disease, Pediatric, Hypertension, Clinical Research, Rare Diseases, Transplantation, Chronic Liver Disease and Cirrhosis, 2.1 Biological and endogenous factors, Aetiology, Oral and gastrointestinal, Good Health and Well Being, Adolescent, Adult, Child, Child, Preschool, Cholestasis, Intrahepatic, Disease Progression, Female, Humans, Hypertension, Portal, Infant, Infant, Newborn, Liver Transplantation, Longitudinal Studies, Male, Young Adult, alpha 1-Antitrypsin Deficiency, ChiLDReN, cirrhosis, jaundice, liver enzymes, liver transplant, metabolic liver disease, Human Movement and Sports Sciences, Paediatrics and Reproductive Medicine, Pediatrics
Abstract

ObjectivesTo 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 designThe 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.ResultsWe 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.ConclusionsPortal 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

5. Split liver transplantation is utilized infrequently and concentrated at few transplant centers in the United States.

Author

Ge, Jin, Perito, Emily R, Bucuvalas, John, Gilroy, Richard, Hsu, Evelyn K, Roberts, John P, and Lai, Jennifer C

Subjects
Humans, Liver Transplantation, Transplants, Graft Survival, Adult, Child, Tissue Donors, Living Donors, Tissue and Organ Procurement, United States, Organ Procurement and Transplantation Network, disparities, donors and donation, ethics and public policy, health services and outcomes research, liver transplant: split, liver transplantation/hepatology, organ allocation, organ procurement and allocation, pediatrics, Organ Transplantation, Transplantation, Digestive Diseases, Liver Disease, liver transplantation, hepatology, liver transplant, split, Medical and Health Sciences, Surgery
Abstract

Split liver transplantation (SLT) is 1 strategy for maximizing the number of deceased donor liver transplants. Recent reports suggest that utilization of SLT in the United States remains low. We examined deceased donor offers that were ultimately split between 2010 and 2014. SLTs were categorized as "primary" and "secondary" transplants. We analyzed allocation patterns and used logistic regression to evaluate factors associated with secondary split discard. Four hundred eighteen livers were split: 54% from adult, 46% from pediatric donors. Of the 227 adult donor livers split, 61% met United Network for Organ Sharing "optimal" split criteria. A total of 770 recipients (418 primary and 352 secondary) were transplanted, indicating 16% discard. Ninety-two percent of the 418 primary recipients were children, and 47% were accepted on the first offer. Eighty-seven percent of the 352 secondary recipients were adults, and 7% were accepted on the first offer. Of the 352 pairs, 99% were transplanted in the same region, 36% at the same center. In logistic regression, shorter donor height was associated with secondary discard (odds ratio 0.97 per cm, 95% CI 0.94-1.00, P = .02). SLT volume by center was not predictive of secondary discard. Current policy proposals that incentivize SLT in the United States could increase the number of transplants to children and adults.

Published
2020

6. International Liver Transplantation Society Global Census: First Look at Pediatric Liver Transplantation Activity Around the World

Author

Rodriguez-Davalos, Manuel I., Lopez-Verdugo, Fidel, Kasahara, Mureo, Muiesan, Paolo, Reddy, Mettu S., Flores-Huidobro Martinez, Angel, Xia, Qiang, Hong, Johnny C., Niemann, Claus U., Seda-Neto, Joao, Miloh, Tamir A., Yi, Nam-Joon, Mazariegos, George V., Ng, Vicky L., Esquivel, Carlos O., Lerut, Jan, Rela, Mohamed, Botha, Jean, Mehta, Naimish N., Bakar Hafeez Bhatti, Abu, Zamora-Valdes, Daniel, Anand, Superina, Riccardo, de Kleine, Ruben, Matamoros, Maria A., Bedregal-Mendoza, Tayana, Steve Bynon, J., Rodriguez-Sancho, Luis C., Langnas, Alan, Dhawan, Anil, Ogura, Yasuhiro, Vianna, Rodrigo, Ilyassova, Bibigul, Tantemsapya, Niramol, Bellemare, Sarah, Ovchinsky, Nadia, Bhalerao, Suryabhan, Balci, Deniz, Dip, Marcelo, Imventarza, Oscar, Mangus, Richard S., Fawcett, Jonathan, Antonini, Teresa M., Shrimal, Anurag, Zhu, Zhi-Jun, Rivera, Jairo, Dayangac, Murat, McCall, John, Utz Melere, Melina, Stormon, Michael, Mizuta, Koichi, Gao, Wei, Hening Rahayatri, Tri, Costaguta, Alejandro, Gautier, Sergei, Kazemi, Kourosh, Martinez-Castillo, Jorge, Apinyachon, Worapot, Rastogi, Mukul, Restrepo, Juan C., Takahito, Yagi, Ravichandra, Srinivasa, Vanin, Anabel, Vieira, Sandra, Luque, Carlos, Gondolesi, Gabriel, Hashimoto, Koji, Yazigi, Nada, Lebel, Sylvie, Mavis, Alisha, Arnon, Ronen, Chavez-Vargas, Ricardo, Elisofon, Scott, Desai, Dev M., Okajima, Hideaki, Chinnakotla, Srinath, Hsu, Evelyn, Reyes, Jorge, Orgoi, Sergelen, Raut, Vikram, Hee Oh, Seak, Valentino, Pamela, Emre, Sukru, Spada, Marco, Leung, Dan, Charco, Ramon, Ericzon, Bo-Goran, Fischer, Ryan, Del Rio-Martin, Juan V., Cillo, Umberto, Soliman, Hossam, Varela-Fascinetto, Gustavo, de Villa, Vanessa, Haberal, Mehmet, Covarrubias, Joshue, Colledan, Michele, de Ville de Goyet, Jean, Desai, Chirag, Sundaram, Shikha, Fronek, Jiri, Nadig, Satish N., Mali, Vidyadhar P., Tandoi, Francesco, Kohli, Rohit, Mohan, Neelam, Goyal, Neerav, Santhosh, Drisya, Debray, Dominique, Durmaz, Ozlem, De Bruyne, Ruth, Kaliciński, Piotr, Hierro, Loreto, Venick, Robert, Perito, Emily, Mozer-Glassberg, Yael, Chen, Chao-Long, Rand, Elizabeth, Nikeghbalian, Saman, Kelly, Deirdre, Boillot, Olivier, and Book, Linda S.

Published
2023
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7. Predicting ideal outcome after pediatric liver transplantation: An exploratory study using machine learning analyses to leverage Studies of Pediatric Liver Transplantation Data

Author

Wadhwani, Sharad Indur, Hsu, Evelyn K, Shaffer, Michele L, Anand, Ravinder, Ng, Vicky Lee, and Bucuvalas, John C

Subjects
Transplantation, Liver Disease, Clinical Research, Digestive Diseases, Organ Transplantation, Adolescent, Adult, Algorithms, Anastomosis, Surgical, Biliary Tract Surgical Procedures, Child, Child, Preschool, Humans, Infant, Liver Failure, Liver Transplantation, Machine Learning, Pediatrics, Predictive Value of Tests, Prospective Studies, Registries, Risk Assessment, Risk Factors, Software, Treatment Outcome, Young Adult, ideal outcome, machine learning, pediatric liver transplant, Paediatrics and Reproductive Medicine, Surgery
Abstract

Machine learning analyses allow for the consideration of numerous variables in order to accommodate complex relationships that would not otherwise be apparent in traditional statistical methods to better classify patient risk. The SPLIT registry data were analyzed to determine whether baseline demographic factors and clinical/biochemical factors in the first-year post-transplant could predict ideal outcome at 3 years (IO-3) after LT. Participants who received their first, isolated LT between 2002 and 2006 and had follow-up data 3 years post-LT were included. IO-3 was defined as alive at 3 years, normal ALT (

Published
2019

8. Deceased Pediatric Donor Livers: How Current Policy Drives Allocation and Transplantation

Author

Ge, Jin, Hsu, Evelyn K, Bucuvalas, John, and Lai, Jennifer C

Subjects
Pediatric, Organ Transplantation, Transplantation, Chronic Liver Disease and Cirrhosis, Liver Disease, Digestive Diseases, Good Health and Well Being, Adolescent, Cadaver, Child, End Stage Liver Disease, Female, Humans, Liver Transplantation, Male, Policy, Tissue Donors, Tissue and Organ Procurement, Medical Biochemistry and Metabolomics, Clinical Sciences, Immunology, Gastroenterology & Hepatology
Abstract

Each year, approximately 60 children, representing 12% of waitlist candidates, die awaiting liver transplantation. The current allocation algorithm for pediatric donor livers prioritizes local/regional adults over national children. We attempted to better understand the impact of the present algorithm on pediatric candidates. We analyzed pediatric donor liver offers from 2010 to 2014. Donors and recipients were classified based on age. We mapped allocation and acceptance patterns and used subgroup analyses to explore the significance of donor service areas (DSAs) with low pediatric transplant volumes. We used Cox proportional hazard regressions to evaluate posttransplantation outcomes: 3,318 pediatric donor livers were transplanted into 3,482 recipients, and 45% (1,569) were adults. Of the 1,569 adults, 25% (390) received a pediatric organ that was never offered to children; 52% (204) of these 390 pediatric organs originated in the 37 DSAs, with ≤25 pediatric liver transplantations; 278 children died or were delisted due to illness during the same time, with higher mortality rates in the 37 DSAs (10% versus 6%, P < 0.01). Compared to adults, pediatric recipients aged 15 before being offered to local/regional + circle non-status 1A adults.

Published
2019

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

Author

Harlow, Kathryn E, Africa, Jonathan A, Wells, Alan, Belt, Patricia H, Behling, Cynthia A, Jain, Ajay K, Molleston, Jean P, Newton, Kimberly P, Rosenthal, Philip, Vos, Miriam B, Xanthakos, Stavra A, Lavine, Joel E, Schwimmer, Jeffrey B, Network, Nonalcoholic Steatohepatitis Clinical Research, Abrams, Stephanie H, Barlow, Sarah, Himes, Ryan, Krisnamurthy, Rajesh, Maldonado, Leanel, Mahabir, Rory, Carr, April, Bernstein, Kimberlee, Bramlage, Kristin, Cecil, Kim, DeVore, Stephanie, Kohli, Rohit, Lake, Kathleen, Podberesky, Daniel, Towbin, Alex, Behr, Gerald, Lefkowitch, Jay H, Mencin, Ali, Reynoso, Elena, Alazraki, Adina, Cleeton, Rebecca, Cordero, Maria, Hernandez, Albert, Karpen, Saul, Munos, Jessica Cruz, Raviele, Nicholas, Bozic, Molly, Cummings, Oscar W, Klipsch, Ann, Ragozzino, Emily, Sandrasegaran, Kumar, Subbarao, Girish, Walker, Laura, Kafka, Kimberly, Scheimann, Ann, Ito, Joy, Fishbein, Mark H, Mohammad, Saeed, Rigsby, Cynthia, Sharda, Lisa, Whitington, Peter F, Cattoor, Theresa, Derdoy, Jose, Freebersyser, Janet, King, Debra, Lai, Jinping, Osmack, Pat, Siegner, Joan, Stewart, Susan, Torretta, Susan, Wriston, Kristina, Baker, Susan S, Lopez-Graham, Diana, Williams, Sonja, Zhu, Lixin, Awai, Hannah, Bross, Craig, Collins, Jennifer, Durelle, Janis, Middleton, Michael, Paiz, Melissa, Sirlin, Claude, Ugalde-Nicalo, Patricia, Villarreal, Mariana Dominguez, Aouizerat, Bradley, Courtier, Jesse, Ferrell, Linda D, Feier, Natasha, Gill, Ryan, Langlois, Camille, Perito, Emily Rothbaum, Tsai, Patrika, Cooper, Kara, Horslen, Simon, Hsu, Evelyn, Murray, Karen, Otto, Randolph, Yeh, Matthew, Young, Melissa, Brunt, Elizabeth M, Fowler, Kathryn, Kleiner, David E, Brown, Sherry, Doo, Edward C, and Hoofnagle, Jay H

Subjects
Paediatrics, Biomedical and Clinical Sciences, Pediatric, Chronic Liver Disease and Cirrhosis, Cardiovascular, Health Disparities, Obesity, Minority Health, Prevention, Liver Disease, Digestive Diseases, Nutrition, 2.4 Surveillance and distribution, Metabolic and endocrine, Good Health and Well Being, Child, Cholesterol, LDL, Diet, Female, Humans, Hypercholesterolemia, Hypertriglyceridemia, Life Style, Longitudinal Studies, Male, Non-alcoholic Fatty Liver Disease, Triglycerides, Nonalcoholic Steatohepatitis Clinical Research Network, NAFLD, cardiovascular, diet, dyslipidemia, pediatric, statin, Human Movement and Sports Sciences, Paediatrics and Reproductive Medicine, Pediatrics
Abstract

ObjectiveTo 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 designThis 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.ResultsThere 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.ConclusionsMore 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

11. Analysis of Liver Offers to Pediatric Candidates on the Transplant Wait List

Author

Hsu, Evelyn K, Shaffer, Michele L, Gao, Lucy, Sonnenday, Christopher, Volk, Michael L, Bucuvalas, John, and Lai, Jennifer C

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Digestive Diseases, Pediatric, Organ Transplantation, Chronic Liver Disease and Cirrhosis, Transplantation, Liver Disease, Good Health and Well Being, Adolescent, Adult, Age Factors, Child, Child, Preschool, Clinical Decision-Making, Donor Selection, End Stage Liver Disease, Female, Health Services Needs and Demand, Humans, Infant, Liver Transplantation, Male, Patient Dropouts, Registries, Risk Assessment, Risk Factors, Severity of Illness Index, Time Factors, Tissue Donors, Treatment Outcome, United States, Waiting Lists, Young Adult, Liver Transplant, Allocation, Organ Offers, Neurosciences, Paediatrics and Reproductive Medicine, Gastroenterology & Hepatology, Clinical sciences, Nutrition and dietetics
Abstract

Background & aimsApproximately 10% of children on the liver transplant wait-list in the United States die every year. We examined deceased donor liver offer acceptance patterns and their contribution to pediatric wait-list mortality.MethodsWe performed a retrospective cohort study of children on the US liver transplant wait-list from 2007 through 2014 using national transplant registry databases. We determined the frequency, patterns of acceptance, and donor and recipient characteristics associated with deceased donor liver organ offers for children who died or were delisted compared with those who underwent transplantation. Children who died or were delisted were classified by the number of donor liver offers (0 vs 1 or more), limiting analyses to offers of livers that were ultimately transplanted into pediatric recipients. The primary outcome was death or delisting on the wait-list.ResultsAmong 3852 pediatric liver transplant candidates, children who died or were delisted received a median 1 pediatric liver offer (inter-quartile range, 0-2) and waited a median 33 days before removal from the wait-list. Of 11,328 donor livers offered to children, 2533 (12%) were transplanted into children; 1179 of these (47%) were immediately accepted and 1354 (53%) were initially refused and eventually accepted for another child. Of 27,831 adults, 1667 (6.0%; median, 55 years) received livers from donors younger than 18 years (median, 15 years), most (97%) allocated locally or regionally. Of children who died or were delisted, 173 (55%) received an offer of 1 or more liver that was subsequently transplanted into another pediatric recipient, and 143 (45%) died or were delisted with no offers.ConclusionsAmong pediatric liver transplant candidates in the US, children who died or were delisted received a median 1 pediatric liver offer and waited a median of 33 days. Of livers transplanted into children, 47% were immediately accepted and 53% were initially refused and eventually accepted for another child. Of children who died or were delisted, 55% received an offer of 1 or more liver that was subsequently transplanted into another pediatric recipient, and 45% died or were delisted with no offers. Pediatric prioritization in the allocation and development of improved risk stratification systems is required to reduce wait-list mortality among children.

Published
2017

14. Pushing the Limits of In Situ Split Liver Procurement to Overcome Donor Distance and Graft Size Challenges for 8‐Week‐Old Pediatric Recipient.

Author

Kwon, Yong K., Valentino, Pamela L., Saarela, Katelyn M., Wendel, Danielle R., Hsu, Evelyn K., Kaufman, Daniel M., Dick, Andre A. S., and Healey, Patrick J.

Subjects
CHILD patients, ORGAN donors, LIVER transplantation, CRITICALLY ill, DATABASES
Abstract

Background: Pediatric liver transplantation for small recipients presents significant challenges, particularly in securing suitably sized donor organs. This case report illustrates the feasibility of performing an in situ split procurement in an 18.5‐kg toddler, the smallest recorded case in the OPTN database to date, for a critically ill 8‐week‐old infant recipient. Case Presentation: An 8‐week‐old infant with severe hepatitis of unknown etiology was urgently listed as Status 1A. An organ offer from a 3.5‐year‐old donor, requiring a reduction procedure, became available 1939 nautical miles away. Instead of a back‐table reduction procedure, we performed an in situ split to reduce cold ischemic time given the distance. The recipient surgery was started ahead of the organ's arrival, and the recipient was ready for graft implantation upon the organ's arrival, resulting in a total of 510 min of cold ischemic time. Post‐operatively, the graft did not show signs of significant injury or dysfunction, which expedited recovery from her other medical conditions. Conclusions: In situ split liver procurement is an invaluable tool for pediatric centers as it effectively provides more graft options for pediatric patients on the waitlist. Additionally, in situ split can offer significant benefits in optimizing recipient surgery, especially when the donor is located at an extreme distance. Despite these benefits, in situ split is not currently widely utilized across transplant centers. Addressing the logistical challenges associated with this technique is crucial for broader implementation and improved patient outcomes. [ABSTRACT FROM AUTHOR]

Published
2024
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15. Caregivers' and providers' perspectives of social and medical care after pediatric liver transplant: Results from the multicenter SOCIAL-Tx study.

Author

Wadhwani, Sharad I., Alvarado, Alejandra, Shifman, Holly P., Bautista, Bethany, Yalung, Jared, Squires, James E., Campbell, Kathleen, Ebel, Noelle H., Hsu, Evelyn, Vittorio, Jennifer, Zielsdorf, Shannon, Desai, Dev M., Bucuvalas, John C., Gottlieb, Laura, Kotagal, Uma, Lyles, Courtney R., Ackerman, Sara L., and Lai, Jennifer C.

Published
2024
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16. Diagnostic accuracy of serum matrix metalloproteinase-7 as a biomarker of biliary atresia in a large North American cohort.

Author

Pandurangi, Sindhu, Mourya, Reena, Nalluri, Shreya, Fei, Lin, Dong, Shun, Harpavat, Sanjiv, Guthery, Stephen L., Molleston, Jean P., Rosenthal, Philip, Sokol, Ronald J., Wang, Kasper S., Ng, Vicky, Alonso, Estella M., Hsu, Evelyn K., Karpen, Saul J., Loomes, Kathleen M., Magee, John C., Shneider, Benjamin L., Horslen, Simon P., and Teckman, Jeffrey H.

Published
2024
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17. 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

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

Published
2020
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19. Anti‐spike antibody durability after SARS‐CoV‐2 vaccination in adolescent solid organ transplant recipients

Author

McAteer, John, primary, Kalluri, Divya D., additional, Abedon, Rivka R., additional, Qin, Caroline X., additional, Auerbach, Scott R., additional, Charnaya, Olga, additional, Danziger‐Isakov, Lara A., additional, Ebel, Noelle H., additional, Feldman, Amy G., additional, Hsu, Evelyn K., additional, Mohammad, Saeed, additional, Perito, Emily R., additional, Thomas, Ashley M., additional, Chiang, Teresa P. Y., additional, Garonzik‐Wang, Jacqueline M., additional, Segev, Dorry L., additional, Werbel, William A., additional, and Mogul, Douglas B., additional

Published
2024
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21. In Children With Nonalcoholic Fatty Liver Disease, Cysteamine Bitartrate Delayed Release Improves Liver Enzymes but Does Not Reduce Disease Activity Scores

Author

Schwimmer, Jeffrey B, Lavine, Joel E, Wilson, Laura A, Neuschwander-Tetri, Brent A, Xanthakos, Stavra A, Kohli, Rohit, Barlow, Sarah E, Vos, Miriam B, Karpen, Saul J, Molleston, Jean P, Whitington, Peter F, Rosenthal, Philip, Jain, Ajay K, Murray, Karen F, Brunt, Elizabeth M, Kleiner, David E, Van Natta, Mark L, Clark, Jeanne M, Tonascia, James, Doo, Edward, Abrams, Stephanie H, Barlow, Sarah, Himes, Ryan, Krisnamurthy, Rajesh, Maldonado, Leanel, Mahabir, Rory, Bernstein, Kimberlee, Bramlage, Kristin, Cecil, Kim, DeVore, Stephanie, Lake, Kathleen, Podberesky, Daniel, Towbin, Alex, Xanthakos, Stavra, Behr, Gerald, Lefkowitch, Jay H, Mencin, Ali, Reynoso, Elena, Alazraki, Adina, Cleeton, Rebecca, Karpen, Saul, Munos, Jessica Cruz, Raviele, Nicholas, Vos, Miriam, Bozic, Molly, Cummings, Oscar W, Klipsch, Ann, Munson, Sarah, Sandrasegaran, Kumar, Subbarao, Girish, Kafka, Kimberly, Scheimann, Ann, Amsden, Katie, Fishbein, Mark H, Kirwan, Elizabeth, Mohammad, Saeed, Rigsby, Cynthia, Sharda, Lisa, Derdoy, Jose, Jain, Ajay, King, Debra, Osmack, Pat, Siegner, Joan, Stewart, Susan, Torretta, Susan, Wriston, Kristina, Baker, Susan S, Zhu, Lixin, Africa, Jonathon, Angeles, Jorge, Arroyo, Sandra, Awai, Hannah, Behling, Cynthia, Bross, Craig, Durelle, Janis, Middleton, Michael, Newton, Kimberly, Paiz, Melissa, Sanford, Jennifer, Sirlin, Claude, Ugalde-Nicalo, Patricia, Villarreal, Mariana Dominguez, Aouizerat, Bradley, Courtier, Jesse, Ferrell, Linda D, Fleck, Shannon, Gill, Ryan, Langlois, Camille, Perito, Emily Rothbaum, Tsai, Patrika, Cooper, Kara, Horslen, Simon, and Hsu, Evelyn

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Clinical Trials and Supportive Activities, Chronic Liver Disease and Cirrhosis, Clinical Research, Hepatitis, Liver Disease, Digestive Diseases, 6.1 Pharmaceuticals, Evaluation of treatments and therapeutic interventions, Oral and gastrointestinal, Adolescent, Alanine Transaminase, Aspartate Aminotransferases, Biopsy, Body Weight, Child, Cysteamine, Cystine Depleting Agents, Delayed-Action Preparations, Double-Blind Method, Female, Humans, Intention to Treat Analysis, Liver, Liver Cirrhosis, Male, Non-alcoholic Fatty Liver Disease, Severity of Illness Index, Pediatrics, ALT, AST, Obesity, NASH CRN, Neurosciences, Paediatrics and Reproductive Medicine, Gastroenterology & Hepatology, Clinical sciences, Nutrition and dietetics
Abstract

Background & aimsNo treatment for nonalcoholic fatty liver disease (NAFLD) has been approved by regulatory agencies. We performed a randomized controlled trial to determine whether 52 weeks of cysteamine bitartrate delayed release (CBDR) reduces the severity of liver disease in children with NAFLD.MethodsWe performed a double-masked trial of 169 children with NAFLD activity scores of 4 or higher at 10 centers. From June 2012 to January 2014, the patients were assigned randomly to receive CBDR or placebo twice daily (300 mg for patients weighing ≤65 kg, 375 mg for patients weighing >65 to 80 kg, and 450 mg for patients weighing >80 kg) for 52 weeks. The primary outcome from the intention-to-treat analysis was improvement in liver histology over 52 weeks, defined as a decrease in the NAFLD activity score of 2 points or more without worsening fibrosis; patients without biopsy specimens from week 52 (17 in the CBDR group and 6 in the placebo group) were considered nonresponders. We calculated the relative risks (RR) of improvement using a stratified Cochran-Mantel-Haenszel analysis.ResultsThere was no significant difference between groups in the primary outcome (28% of children in the CBDR group vs 22% in the placebo group; RR, 1.3; 95% confidence interval [CI], 0.8-2.1; P = .34). However, children receiving CBDR had significant changes in prespecified secondary outcomes: reduced mean levels of alanine aminotransferase (reduction, 53 ± 88 U/L vs 8 ± 77 U/L in the placebo group; P = .02) and aspartate aminotransferase (reduction, 31 ± 52 vs 4 ± 36 U/L in the placebo group; P = .008), and a larger proportion had reduced lobular inflammation (36% in the CBDR group vs 21% in the placebo group; RR, 1.8; 95% CI, 1.1-2.9; P = .03). In a post hoc analysis of children weighing 65 kg or less, those taking CBDR had a 4-fold better chance of histologic improvement (observed in 50% of children in the CBDR group vs 13% in the placebo group; RR, 4.0; 95% CI, 1.3-12.3; P = .005).ConclusionsIn a randomized trial, we found that 1 year of CBDR did not reduce overall histologic markers of NAFLD compared with placebo in children. Children receiving CBDR, however, had significant reductions in serum aminotransferase levels and lobular inflammation. ClinicalTrials.gov no: NCT01529268.

Published
2016

25. Donor Considerations

Author

Hsu, Evelyn, Reyes, Jorge, Ng, Vicky Lee, Section editor, Bucuvalas, John C., Section editor, Doria, Cataldo, Series Editor, Dunn, Stephen P., editor, and Horslen, Simon, editor

Published
2018
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31. Optimizing pediatric liver transplantation: Evaluating the impact of donor age and graft type on patient survival outcome.

Author

Kwon, Yong K., Valentino, Pamela L., Healey, Patrick J., Dick, Andre A. S., Hsu, Evelyn K., Perkins, James D., and Sturdevant, Mark L.

Subjects
OVERALL survival, SURVIVAL rate, LIVER transplantation, PROPORTIONAL hazards models, KIDNEY transplantation, SURVIVAL analysis (Biometry)
Abstract

Background: We examined the combined effects of donor age and graft type on pediatric liver transplantation outcomes with an aim to offer insights into the strategic utilization of these donor and graft options. Methods: A retrospective analysis was conducted using a national database on 0–2‐year‐old (N = 2714) and 3–17‐year‐old (N = 2263) pediatric recipients. These recipients were categorized based on donor age (≥40 vs <40 years) and graft type. Survival outcomes were analyzed using the Kaplan–Meier and Cox proportional hazards models, followed by an intention‐to‐treat (ITT) analysis to examine overall patient survival. Results: Living and younger donors generally resulted in better outcomes compared to deceased and older donors, respectively. This difference was more significant among younger recipients (0–2 years compared to 3–17 years). Despite this finding, ITT survival analysis showed that donor age and graft type did not impact survival with the exception of 0–2‐year‐old recipients who had an improved survival with a younger living donor graft. Conclusions: Timely transplantation has the largest impact on survival in pediatric recipients. Improving waitlist mortality requires uniform surgical expertise at many transplant centers to provide technical variant graft (TVG) options and shed the conservative mindset of seeking only the "best" graft for pediatric recipients. [ABSTRACT FROM AUTHOR]

Published
2024
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32. Caregivers’ and providers’ perspectives of social and medical care after pediatric liver transplant: Results from the multicenter SOCIAL-Tx study

Author

Wadhwani, Sharad I., primary, Alvarado, Alejandra, additional, Shifman, Holly P., additional, Bautista, Bethany, additional, Yalung, Jared, additional, Squires, James E., additional, Campbell, Kathleen, additional, Ebel, Noelle H., additional, Hsu, Evelyn, additional, Vittorio, Jennifer, additional, Zielsdorf, Shannon, additional, Desai, Dev M., additional, Bucuvalas, John C., additional, Gottlieb, Laura, additional, Kotagal, Uma, additional, Lyles, Courtney R., additional, Ackerman, Sara L., additional, and Lai, Jennifer C., additional

Published
2023
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34. Omicron Infections in Vaccinated Pediatric Solid Organ Transplant Recipients

Author

McAteer, John, primary, Kalluri, Divya D, additional, Abedon, Rivka R, additional, Qin, Caroline X, additional, Auerbach, Scott R, additional, Charnaya, Olga, additional, Danziger-Isakov, Lara A, additional, Ebel, Noelle H, additional, Feldman, Amy G, additional, Hsu, Evelyn K, additional, Mohammad, Saeed, additional, Perito, Emily R, additional, Thomas, Ashley M, additional, Chiang, Teresa P Y, additional, Garonzik-Wang, Jacqueline M, additional, Segev, Dorry L, additional, Werbel, William A, additional, and Mogul, Douglas B, additional

Published
2023
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35. Caregiver Perceptions of Social Risk Screening in Pediatric Liver Transplantation: From the Multicenter SOCIAL-Tx Study

Author

Wadhwani, Sharad I., primary, Kruse, Gina, additional, Squires, James, additional, Ebel, Noelle, additional, Gupta, Nitika, additional, Campbell, Kathleen, additional, Hsu, Evelyn, additional, Zielsdorf, Shannon, additional, Vittorio, Jennifer, additional, Desai, Dev M., additional, Bucuvalas, John C., additional, Gottlieb, Laura M., additional, and Lai, Jennifer C., additional

Published
2023
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36. Clinically Actionable Hypercholesterolemia and Hypertriglyceridemia in Children with Nonalcoholic Fatty Liver Disease

Author

Abrams, Stephanie H., Barlow, Sarah, Himes, Ryan, Krisnamurthy, Rajesh, Maldonado, Leanel, Mahabir, Rory, Carr, April, Bernstein, Kimberlee, Bramlage, Kristin, Cecil, Kim, DeVore, Stephanie, Kohli, Rohit, Lake, Kathleen, Podberesky, Daniel, Towbin, Alex, Behr, Gerald, Lefkowitch, Jay H., Mencin, Ali, Reynoso, Elena, Alazraki, Adina, Cleeton, Rebecca, Cordero, Maria, Hernandez, Albert, Karpen, Saul, Munos, Jessica Cruz, Raviele, Nicholas, Bozic, Molly, Cummings, Oscar W., Klipsch, Ann, Ragozzino, Emily, Sandrasegaran, Kumar, Subbarao, Girish, Walker, Laura, Kafka, Kimberly, Scheimann, Ann, Ito, Joy, Fishbein, Mark H., Mohammad, Saeed, Rigsby, Cynthia, Sharda, Lisa, Whitington, Peter F., Cattoor, Theresa, Derdoy, Jose, Freebersyser, Janet, King, Debra, Lai, Jinping, Osmack, Pat, Siegner, Joan, Stewart, Susan, Torretta, Susan, Wriston, Kristina, Baker, Susan S., Lopez-Graham, Diana, Williams, Sonja, Zhu, Lixin, Awai, Hannah, Bross, Craig, Collins, Jennifer, Durelle, Janis, Middleton, Michael, Paiz, Melissa, Sirlin, Claude, Ugalde-Nicalo, Patricia, Villarreal, Mariana Dominguez, Aouizerat, Bradley, Courtier, Jesse, Ferrell, Linda D., Feier, Natasha, Gill, Ryan, Langlois, Camille, Perito, Emily Rothbaum, Tsai, Patrika, Cooper, Kara, Horslen, Simon, Hsu, Evelyn, Murray, Karen, Otto, Randolph, Yeh, Matthew, Young, Melissa, Brunt, Elizabeth M., Fowler, Kathryn, Kleiner, David E., Brown, Sherry, Doo, Edward C., Hoofnagle, Jay H., Robuck, Patricia R., Sherker, Averell, Torrance, Rebecca, Clark, Jeanne M., Donithan, Michele, Hallinan, Erin, Isaacson, Milana, May, Kevin P., Miriel, Laura, Sternberg, Alice, Tonascia, James, Van Natta, Mark, Wilson, Laura, Yates, Katherine, Harlow, Kathryn E., Africa, Jonathan A., Wells, Alan, Belt, Patricia H., Behling, Cynthia A., Jain, Ajay K., Molleston, Jean P., Newton, Kimberly P., Rosenthal, Philip, Vos, Miriam B., Xanthakos, Stavra A., Lavine, Joel E., and Schwimmer, Jeffrey B.

Published
2018
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37. Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study

Author

Hertel, Paula M., Harpavat, Sanjiv, Brandt, Mary L., Leung, Daniel H., Karnsakul, Wikrom, Torrance, Rebecca, Hall, Sherry, Doo, Edward, Hoofnagle, Jay H., Whitington, Peter, Bass, Lee, Miethke, Alexander G., Heubi, James E., Setchell, Kenneth, Bove, Kevin E., Tiao, Greg, Mack, Cara L., Narkewicz, Michael R., Feldman, Amy G., Sundaram, Shikha S., Suchy, Frederick J., Karrer, Frederick M., Lovell, Mark, Van Hove, Johan L., Rand, Elizabeth B., Squires, James E., Venkat, Veena L., Sindhi, Rakesh, Ranganathan, Sarangarajan, Bull, Laura, Teckman, Jeffrey, Bozic, Molly, Subbarao, Girish, Horslen, Simon, Hsu, Evelyn, Finn, Laura, Healey, Patrick, Kohli, Rohit, Thomas, Danny, Soufi, Nisreen, Michail, Sonia, Clifton, Matt, Gupta, Nitika, Romero, Rene, Vos, Miriam, Caltharp, Shelley, Kamath, Binita M., Ling, Simon C., Gold, Anna, Fecteau, Annie, Guthery, Stephen L., Jensen, Kyle, Meyers, Rebecka, Lowichik, Amy, Book, Linda, Merion, Robert M., Spino, Cathie, Jones, Karen, Ng, Vicky L., Sorensen, Lisa G., Alonso, Estella M., Fredericks, Emily M., Ye, Wen, Moore, Jeff, Karpen, Saul J., Shneider, Benjamin L., Molleston, Jean P., Bezerra, Jorge A., Murray, Karen F., Loomes, Kathleen M., Rosenthal, Philip, Squires, Robert H., Wang, Kasper, Arnon, Ronen, Schwarz, Kathleen B., Turmelle, Yumirle P., Haber, Barbara H., Sherker, Averell H., Magee, John C., and Sokol, Ronald J.

Published
2018
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38. In Children With Nonalcoholic Fatty Liver Disease, Zone 1 Steatosis Is Associated With Advanced Fibrosis

Author

Abrams, Stephanie H., Barlow, Sarah, Himes, Ryan, Krisnamurthy, Rajesh, Maldonado, Leanel, Mahabir, Rory, Carr, April, Bernstein, Kimberlee, Bramlage, Kristin, Cecil, Kim, DeVore, Stephanie, Kohli, Rohit, Lake, Kathleen, Podberesky, Daniel, Towbin, Alex, Xanthakos, Stavra, Behr, Gerald, Lavine, Joel E., Lefkowitch, Jay H., Mencin, Ali, Reynoso, Elena, Alazraki, Adina, Cleeton, Rebecca, Cordero, Maria, Hernandez, Albert, Karpen, Saul, Munos, Jessica Cruz, Raviele, Nicholas, Vos, Miriam, Bozic, Molly, Cummings, Oscar W., Klipsch, Ann, Molleston, Jean P., Ragozzino, Emily, Sandrasegaran, Kumar, Subbarao, Girish, Walker, Laura, Kafka, Kimberly, Scheimann, Ann, Ito, Joy, Fishbein, Mark H., Mohammad, Saeed, Rigsby, Cynthia, Sharda, Lisa, Whitington, Peter F., Cattoor, Theresa, Derdoy, Jose, Freebersyser, Janet, Jain, Ajay, King, Debra, Lai, Jinping, Osmack, Pat, Siegner, Joan, Stewart, Susan, Torretta, Susan, Wriston, Kristina, Baker, Susan S., Lopez–Graham, Diana, Williams, Sonja, Zhu, Lixin, Africa, Jonathan, Awai, Hannah, Behling, Cynthia, Bross, Craig, Collins, Jennifer, Durelle, Janis, Harlow, Kathryn, Middleton, Michael, Newton, Kimberly, Paiz, Melissa, Schwimmer, Jeffrey B., Sirlin, Claude, Ugalde-Nicalo, Patricia, Villarreal, Mariana Dominguez, Aouizerat, Bradley, Courtier, Jesse, Ferrell, Linda D., Feier, Natasha, Gill, Ryan, Langlois, Camille, Perito, Emily Rothbaum, Rosenthal, Philip, Tsai, Patrika, Cooper, Kara, Horslen, Simon, Hsu, Evelyn, Murray, Karen, Otto, Randolph, Yeh, Matthew, Young, Melissa, Brunt, Elizabeth M., Fowler, Kathryn, Africa, Jonathan A., Behling, Cynthia A., Zhang, Nan, Luo, Yunjun, Wells, Alan, Hou, Jiayi, Belt, Patricia H., Kohil, Rohit, and Newton, Kimberly P.

Published
2018
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39. Frailty in Children with Liver Disease: A Prospective Multicenter Study

Author

Lurz, Eberhard, Quammie, Claudia, Englesbe, Michael, Alonso, Estella M., Lin, Henry C., Hsu, Evelyn K., Furuya, Katryn N., Gupta, Nitika A., Venkat, Veena L., Daniel, James F., Leonis, Mike A., Miloh, Tamir, Telega, Grzegorz W., Yap, Jason, Menendez, Jerome, Book, Linda S., Himes, Ryan W., Sundaram, Shikha S., Parekh, Rulan, Sonnenday, Chris, Bucuvalas, John, Ng, Vicky L., and Kamath, Binita M.

Published
2018
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45. Low and High Birth Weights Are Risk Factors for Nonalcoholic Fatty Liver Disease in Children

Author

Abrams, Stephanie H., Barlow, Sarah, Himes, Ryan, Krisnamurthy, Rajesh, Maldonado, Leanel, Mahabir, Rory, Carr, April, Bernstein, Kimberlee, Bramlage, Kristin, Cecil, Kim, DeVore, Stephanie, Kohli, Rohit, Lake, Kathleen, Podberesky, Daniel, Towbin, Alex, Xanthakos, Stavra, Allende, Daniela, Dasarathy, Srinivasan, McCullough, Arthur J., Pagadala, Mangesh, Pai, Rish, Winston, Cha'Ron, Behr, Gerald, Lavine, Joel E., Lefkowitch, Jay H., Mencin, Ali, Reynoso, Elena, Abdelmalek, Manal F., Bashir, Mustafa, Buie, Stephanie, Diehl, Anna Mae, Guy, Cynthia, Kigongo, Christopher, Malik, David, Pan, Yi-Ping, Piercy, Dawn, Kopping, Mariko, Thrasher, Tyler, Alazraki, Adina, Cleeton, Rebecca, Cordero, Maria, Hernandez, Albert, Karpen, Saul, Munos, Jessica Cruz, Raviele, Nicholas, Vos, Miriam, Bozic, Molly, Chalasani, Naga, Cummings, Oscar W., Gawrieh, Samer, Klipsch, Ann, Molleston, Jean P., Ragozzino, Emily, Ragozzino, Linda, Sandrasegaran, Kumar, Subbarao, Girish, Vuppalanchi, Raj, Walker, Laura, Kafka, Kimberly, Scheimann, Ann, Ito, Joy, Fishbein, Mark H., Mohammad, Saeed, Rigsby, Cynthia, Sharda, Lisa, Whitington, Peter F., Cattoor, Theresa, Derdoy, Jose, Freebersyser, Janet, Jain, Ajay, King, Debra, Lai, Jinping, Osmack, Pat, Siegner, Joan, Stewart, Susan, Neuschwander-Tetri, Brent A., Torretta, Susan, Wriston, Kristina, Assadian, Fereshteh, Barone, Vanessa, Gonzalez, Maria Cardona, Davila, Jodie, Fix, Oren, Hennessey, Kelly Anne, Kowdley, Kris V., Lopez, Kacie, Ness, Erik, Poitevin, Michelle, Procaccini, Nicholas, Quist, Brook, Saddic, Alana, Wiseman, Cara, Yeh, Matthew, Baker, Susan S., Lopez-Graham, Diana, Williams, Sonja, Zhu, Lixin, Africa, Jonathan, Ang, Brandon, Awai, Hannah, Behling, Cynthia, Bhatt, Archana, Bross, Craig, Collins, Jennifer, Durelle, Janis, Harlow, Kathryn, Loomba, Rohit, Middleton, Michael, Newton, Kimberly, Paiz, Melissa, Schwimmer, Jeffrey B., Sirlin, Claude, Ugalde-Nicalo, Patricia, Villarreal, Mariana Dominguez, Aouizerat, Bradley, Bass, Nathan M., Brandman, Danielle, Courtier, Jesse, Ferrell, Linda D., Feier, Natasha, Gill, Ryan, Hameed, Bilal, Langlois, Camille, Maher, Jacqueline, Perito, Emily Rothbaum, Ramos, Claudia, Rosenthal, Philip, Terrault, Norah, Tsai, Patrika, Ungermann, Ashley, Atla, Pradeep, Croft, Brandon, Garcia, Rebekah, Garcia, Sonia, Sheikh, Muhammad, Singh, Mandeep, Cooper, Kara, Horslen, Simon, Hsu, Evelyn, Murray, Karen, Otto, Randolph, Young, Melissa, Boyett, Sherry, Carucci, Laura, Contos, Melissa J., Kirwin, Sherri, Kraft, Kenneth, Luketic, Velimir A.C., Puri, Puneet, Sanyal, Arun J., Schlosser, Jolene, Siddiqui, Mohammad S., Brunt, Elizabeth M., Fowler, Kathryn, Kleiner, David E., Brown, Sherry, Doo, Edward C., Hoofnagle, Jay H., Robuck, Patricia R., Sherker, Averell, Torrance, Rebecca, Belt, Patricia, Clark, Jeanne M., Donithan, Michele, Hallinan, Erin, Isaacson, Milana, May, Kevin P., Miriel, Laura, Sternberg, Alice, Tonascia, James, Van Natta, Mark, Wilson, Laura, Yates, Katherine, Newton, Kimberly P., Feldman, Haruna S., and Chambers, Christina D.

Published
2017
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46. Considerations for liver transplantation in deoxyguanosine kinase deficiency: A case series and review of the literature.

Author

Duong, Jennifer T., Pacheco, M. Cristina, Hsu, Evelyn, and Blondet, Niviann

Subjects
LITERATURE reviews, LIVER transplantation, DEOXYGUANOSINE, LIVER failure, MITOCHONDRIAL pathology
Abstract

Background: Deoxyguanosine kinase (DGUOK) deficiency is a rare mitochondrial disorder characterized by early onset liver failure and varying degrees of neurologic dysfunction. Patients typically present during infancy with progressive hepatic dysfunction leading to liver failure, which can precede neurologic deterioration. Outcomes posttransplantation are historically worse than average and the role of liver transplantation remains controversial. These factors, in combination with the increasing number of patients being diagnosed via molecular genetic testing, may impede waitlist access. Methods: We report our single‐center experience with three patients with DGUOK deficiency, all of whom were considered for transplant. We review the current literature regarding management and discuss the role of liver transplantation in DGUOK deficiency‐associated liver failure. Results: Two patients presented with hypoglycemia, conjugated hyperbilirubinemia, and lactic acidosis within the first week of life, were diagnosed with DGUOK deficiency prior to 2 months of age and had severe neurologic involvement. The third patient presented in later infancy was diagnosed with DGUOK deficiency at 18 months of age and had minimal neurologic involvement. All three patients were considered for transplant, though only two patients were listed. All three died from complications of end‐stage liver failure prior to liver transplantation between the ages of 5–20 months. Conclusion: Selection for liver transplantation in DGUOK deficiency is complex, requiring a multidisciplinary team approach. Recent data suggest that liver transplantation can be successful in select patients with absent or mild neurologic manifestations. National databases reporting long‐term outcomes posttransplantation are needed. [ABSTRACT FROM AUTHOR]

Published
2024
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