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3. Contributors

Author

A-Kader, H. Hesham, primary, Abdulhai, Sophia, additional, Abu-Elmagd, Kareem, additional, Abu-El-Haija, Maisam, additional, Adler, Douglas G., additional, Albenberg, Lindsey, additional, Alonso, Estella M., additional, Amin, Ruchi, additional, Atay, Orhan, additional, Auricchio, Renata, additional, Baker, Robert D., additional, Baker, Susan S., additional, Baldwin, Katherine, additional, Barry, Jessica, additional, Baron, Todd H., additional, Barth, Bradley, additional, Bass, Dorsey M., additional, Bass, Lee M., additional, Belkind-Gerson, Jaime, additional, Benninga, Marc A., additional, Bhesania, Natalie, additional, Bischoff, Andrea, additional, Bitton, Samuel, additional, Blanchard, Samra S., additional, Bousvaros, Athos, additional, Boyle, Brendan, additional, Brewer, Jennifer, additional, Brownell, Jefferson N., additional, Bruch, Steven W., additional, Campbell, Brendan T., additional, Campbell, Jacob, additional, Caty, Michael Gerard, additional, Cerezo, Carolina S., additional, Chagpar, Ryaz, additional, Chatfield, Beth, additional, Cherry, Rebecca N., additional, Cohen, Gail, additional, Cohen, Mitchell B., additional, Coran, Arnold G., additional, Costa, Guilherme, additional, Cresci, Gail A.M., additional, Crowley, Eileen, additional, Cruise, Michael, additional, Czinn, Steven J., additional, Davidovics, Zev, additional, Torre, Luis De La, additional, DeRoss, Anthony L., additional, Devadason, David, additional, Venkatesh, Rajitha Devadoss, additional, Di Lorenzo, Carlo, additional, Dotson, Jennifer L., additional, Ediger, Tracy R., additional, Eghtesad, Bijan, additional, Eisses, John F., additional, El Yousif, Mounif, additional, McBride Emerick, Karan, additional, Erdman, Steven H., additional, Fawaz, Rima, additional, Feldstein, Ariel E., additional, Fernandes, Melissa, additional, Finn, Laura S., additional, Fiorino, Kristin Nicole, additional, Fishman, Douglas S., additional, Friedlander, Joel A., additional, Fujiki, Masato, additional, Fung, John, additional, Fuss, Ivan, additional, Galloway, David, additional, George, Donald E., additional, Ghishan, Fayez K., additional, Girlanda, Raffaelle, additional, Gitt, Donna, additional, Goldman, Deborah A., additional, Goodine, Sue, additional, Gourley, Glenn R., additional, Green, Nicole, additional, Grisotti, Gabrielle, additional, Gupta, Sandeep K., additional, Hadzic, Nedim, additional, Harpavat, Sanjiv, additional, Hashimoto, Koji, additional, Hassan, Maheen, additional, Heubi, James E., additional, Husain, Sohail Z., additional, Hussey, Séamus, additional, Hyams, Jeffrey S., additional, Hyer, Warren, additional, Hyman, Paul E., additional, Iben, Sabine, additional, Issac, Veronica E., additional, Jonas, Maureen M., additional, Kay, Marsha, additional, Kehar, Mohit, additional, Kelly, Deidre, additional, Kovacic, Karlo, additional, Kunisaki, Shaun Michael, additional, Kurowski, Jacob A., additional, Langer, Jacob C., additional, Lee, Frances C., additional, Lee, Rose, additional, LeLeiko, Neal S., additional, Liacouras, Chris A., additional, Lin, Henry, additional, Liu, Quin Y., additional, Loomes, Kathleen M., additional, Lu, Peter L., additional, Lusman, Sarah Shrager, additional, Mack, Cara, additional, Maheshwari, Anshu, additional, Mamula, Petar, additional, Manfredi, Michael A., additional, Markowitz, James F., additional, Markowitz, Jonathan E., additional, Mascarenhas, Maria R., additional, Mayer, Ryann, additional, McKiernan, Patrick, additional, Mezoff, Adam G., additional, Mezoff, Ethan A., additional, Mieli-Vergani, Giorgina, additional, Mohr, Franziska, additional, Mokha, Jasmeet, additional, Mousa, Hayat, additional, Moye, Lindsay, additional, Murch, Simon, additional, Murray, Karen F., additional, Naples, Robert, additional, Nathan, Jaimie D., additional, Lee Ng, Vicky, additional, Nguyen, Vi, additional, Nurko, Samuel, additional, Oauhed, Jodie, additional, Ogholikhan, Tina, additional, Oldham, Keith T., additional, Osman, Mohammed, additional, Ovchinsky, Nadia, additional, Panganiban, Jennifer, additional, Pena, Alberto, additional, Petras, Robert E., additional, Pfefferkorn, Marian D., additional, Piccoli, David, additional, Piester, Travis, additional, Pinkos, Beth, additional, Plesec, Thomas, additional, Polites, Stephanie, additional, Ponsky, Todd, additional, Rader, Christine, additional, Radhakrishnan, Kadakkal, additional, Reissis, Yannis, additional, Rodriguez, Leonel, additional, Rodriguez, Ricardo J., additional, Rojas, Isabel, additional, Rome, Ellen S., additional, Rosh, Joel R., additional, Ruiz, Rachel M., additional, Sahn, Benjamin, additional, Saleem, Atif, additional, Samela, Kate A., additional, Santucci, Neha R., additional, Saps, Miguel, additional, Sato, Eleanor H., additional, Sato, Thomas T., additional, Savage, Erica C., additional, Seifarth, Federico G., additional, Conjeevaram Selvakumar, Praveen Kumar, additional, Shapiro, Jason, additional, Siperstein, Allan E., additional, Skelton, Joseph, additional, Snapper, Scott, additional, Soldes, Oliver S., additional, Sood, Manu R., additional, Stahl, Marisa Gallant, additional, Sundaram, Shikha S., additional, Sylvester, Francisco A., additional, Teitelbaum, Jonathan E., additional, Terry, Natalie A., additional, Townsend, Peter, additional, Troncone, Riccardo, additional, Vance, Kate, additional, Vandenplas, Yvan, additional, Venick, Robert S., additional, Vitale, David S., additional, Vockley, Jerry, additional, Vortia, Eugene, additional, Vriesman, Mana H., additional, Wahbeh, Ghassan T., additional, Walsh, R. Matthew, additional, Warner, Suz, additional, Wyllie, Robert, additional, Yasuda, Jessica L., additional, Zeiter, Donna, additional, and Zheng, Hengqi (Betty), additional

Published
2021
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8. Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with Their Native Livers: Results from a North American Multicenter Consortium

Author

Lee Ng, Vicky, Haber, Barbara H., Magee, John C., Miethke, Alexander, Murray, Karen F., Michail, Sonia, Karpen, Saul J., Kerkar, Nanda, Molleston, Jean P., Romero, Rene, Rosenthal, Philip, Schwarz, Kathleen B., Shneider, Benjamin L., Turmelle, Yumirle P., Alonso, Estella M., Sherker, Averell H., and Sokol, Ronald J.

Published
2014
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9. Neurodevelopmental Outcomes in Preschool and School Aged Children With Biliary Atresia and Their Native Liver

Author

Squires, James E., Lee Ng, Vicky, Hawthorne, Kieran, Henn, Lisa L., Sorensen, Lisa G., Fredericks, Emily M., Alonso, Estella M., Murray, Karen F., Loomes, Kathleen M., Karpen, Saul J., Cavallo, Laurel A., Molleston, Jean P., Bezerra, Jorge A., Rosenthal, Philip, Squires, Robert H., Wang, Kasper S., Schwarz, Kathleen B., Arnon, Ronen, Magee, John C., and Sokol, Ronald J.

Abstract

The aim of the study was to assess neurodevelopmental outcomes among children with biliary atresia (BA) surviving with their native liver at ages 3 to 12 years and evaluate variables that associate with neurodevelopment. Participants (ages 3–12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, ages 3–5 years) and Weschler Intelligence Scale for Children, 4th edition (WISC-IV, ages 6–12 years). Continuous scores were analyzed using Kolmogorov-Smironov tests compared with a normal distribution (mean = 100 ± 15). Effect of covariates on Full-Scale Intelligence Quotient (FSIQ) was analyzed using linear regression. Ninety-three participants completed 164 WPPSI-III (mean age 3.9) and 51 WISC-IV (mean age 6.9) tests. WPPSI-III FSIQ (104 ± 14, P< 0.02), Verbal IQ (106 ± 14, P< 0.001), and General Language Composite (107 ± 16, P< 0.001) distributions were shifted higher compared with test norms. WISC-IV FSIQ (105 ± 12, P< 0.01), Perceptual Reasoning Index (107 ± 12, P< 0.01), and Processing Speed Index (105 ± 10, P< 0.02) also shifted upwards. In univariate and multivariable analysis, parent education (P< 0.01) was a significant predictor of FSIQ on WPPSI-III and positively associated with WISC-IV FSIQ. Male sex and higher total bilirubin and gamma glutamyl transferase (GGT) predicted lower WPPSI-III FSIQ. Portal hypertension was predictive of lower WISC-IV FSIQ. This cohort of children with BA and native liver did not demonstrate higher prevalence of neurodevelopmental delays. Markers of advanced liver disease (higher total bilirubin and GGT for age ?5 years; portal hypertension for age =6) correlate with lower FSIQ and may identify a vulnerable subset of patients who would benefit from intervention.

Published
2020
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10. Predictive Equations Are Inaccurate in the Estimation of the Resting Energy Expenditure of Children With End-Stage Liver Disease.

Author

Carpenter, Andrea, Lee Ng, Vicky, Chapman, Karen, Ling, Simon C., Mouzaki, Marialena, and Ng, Vicky Lee

Subjects
PREVENTION of malnutrition, MALNUTRITION, BASAL metabolism, CALORIMETRY, DIET therapy, ENERGY metabolism, LIVER failure, NUTRITIONAL requirements, RETROSPECTIVE studies, DISEASE complications, THERAPEUTICS
Abstract

Background and Objectives: Malnutrition is common in children with end-stage liver disease (ESLD) and is associated with increased morbidity and mortality. The inability to accurately estimate energy needs of these patients may contribute to their poor nutrition status. In clinical practice, predictive equations are used to calculate resting energy expenditure (cREE). The objective of this study is to assess the accuracy of commonly used equations in pediatric patients with ESLD.Methods: Retrospective study performed at the Hospital for Sick Children. Clinical, laboratory, and indirect calorimetry data from children listed for liver transplant between February 2013 and December 2014 were reviewed. Calorimetry results were compared with cREE estimated using the Food and Agriculture Organization/World Health Organization/United Nations University (FAO/WHO/UNU), Schofield [weight], and Schofield [weight and height] equations.Results: Forty-five patients were included in this study. The median age was 9 months, and the most common indication for transplantation was biliary atresia (64%). The Schofield [weight and height], FAO/WHO/UNU, and Schofield [weight] equations were compared with indirect calorimetry and found to have a mean (SD) difference of 48.8 (344.0), 59.3 (229.8), and 206.5 (502.6) kcal/d, respectively. The FAO/WHO/UNU, Schofield [weight], and Schofield [weight and height] equations introduced a mean error of 21%, 38%, and 76%, respectively. The FAO/WHO/UNU equation tended to underestimate, whereas the Schofield equations overestimated the REE.Conclusions: Commonly used predictive equations perform poorly in infants and young children with ESLD. Indirect calorimetry should be used when available to guide energy provision, particularly in children who are already malnourished. [ABSTRACT FROM AUTHOR]

Published
2017
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12. Health-related quality of life in long-term survivors of paediatric liver transplantation.

Author

Konidis, Stacey V., Hrycko, Alexander, Nightingale, Scott, Renner, Eberhard, Lilly, Leslie, Therapondos, George, Fu, Ann, Avitzur, Yaron, and Lee Ng, Vicky

Subjects
STATISTICAL correlation, HEALTH surveys, LIVER transplantation, LONGITUDINAL method, QUALITY of life, QUESTIONNAIRES, RESEARCH funding, T-test (Statistics), WELL-being, DATA analysis software, DESCRIPTIVE statistics
Abstract

Copyright of Paediatrics & Child Health (1205-7088) is the property of Oxford University Press / USA and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2015
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14. Experiences and barriers to Health-Related Quality of Life following liver transplantation: a qualitative analysis of the perspectives ofpediatric patients and their parents.

Author

Nicholas, David B., Otley, Anthony R., Taylor, Rachel, Dhawan, Anil, Gilmour, Susan, and Lee Ng, Vicky

Subjects
PUBLIC health, HUMAN services, BIOSECURITY, HEALTH education, MEDICINE, PHYSIOLOGY
Abstract

This paper examines health-related quality of life (HRQOL) experiences and barriers facing young people who have received a liver transplant (LT). Semi-structured qualitative interviews were conducted with children and adolescents who have undergone LT and their parents. Findings indicate that LT fosters substantially improved child and adolescent HRQOL; however, young people also experience challenges such as difficulties with medication compliance, self-management of care routines, physical activity restrictions, and undesirable medical procedures. Implications and recommendations for clinical practice and research are discussed. [ABSTRACT FROM AUTHOR]

Published
2010
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15. Sirolimus for pediatric liver transplant recipients with post-transplant lymphoproliferative disease and hepatoblastoma.

Author

Jiménez-Rivera, Carolina, Avitzur, Yaron, Fecteau, Annie H., Jones, Nicola, Grant, David, and Lee Ng, Vicky

Subjects
RAPAMYCIN, MACROLIDE antibiotics, IMMUNOSUPPRESSIVE agents, LIVER transplantation, LYMPHOPROLIFERATIVE disorders, CELL proliferation, LYMPHATIC diseases
Abstract

Jiménez-Rivera C, Avitzur Y, Fecteau AH, Jones N, Grant D, Ng VL. Sirolimus for pediatric liver transplant recipients with post-transplant lymphoproliferative disease and hepatoblastoma. Pediatr Transplantation 2004: 8: 243–248. © 2004 Blackwell Munksgaard Sirolimus is a promising immune suppressive agent, with the potential to reduce calcineurin inhibitor associated nephrotoxicity, halt progression of chronic rejection and prevent tumor proliferation. The aim of this study was to review the experience using sirolimus in pediatric liver transplant recipients at a single center. Database and medical charts of all pediatric liver transplant recipients receiving sirolimus at the Hospital for Sick Children in Toronto were reviewed. Eight patients received sirolimus between October, 2000 and September, 2002. Indications for using sirolimus were post-transplant lymphoproliferative disease (PTLD) (n = 6) and hepatoblastoma (n = 2). Two patients with PTLD concurrently had renal impairment and chronic rejection. Sirolimus dosages ranged between 1.5 and 5 mg once daily. Median duration of follow-up was 17 months. Persistently elevated liver transaminase levels in the two children with chronic rejection decreased during sirolimus therapy. Recurrence of PTLD occurred in one patient. Two patients were diagnosed with acute cellular rejection after transition to maintenance sirolimus monotherapy. Resolution of adverse effects including mouth sores (n = 3), leg swelling (n = 2) and hyperlipidemia (n = 3) occurred either spontaneously or with dose reduction. Sirolimus was discontinued in four patients because of persisting bone marrow suppression, interstitial pneumonitis, life-threatening sepsis and refractory diarrhea. Children with PTLD or hepatoblastoma may benefit from immune suppression with sirolimus after liver transplantation. Further multi-center, prospective, randomized controlled trials will be instrumental to further the knowledge of long-term efficacy, safety and tolerability of sirolimus for selected children following liver transplantation. [ABSTRACT FROM AUTHOR]

Published
2004
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16. Health-Related Quality of Life after Pediatric Liver Transplantation: A Qualitative Analysis of the Perspectives of Health Care Providers

Author

Miserachs, Mar, B. Nicholas, David, R. Otley, Anthony, and Lee Ng, Vicky

Abstract

With improved survival outcomes after pediatric liver transplantation (LT), health-related quality of life (HRQoL) is an important outcome metric. Understanding the elements contributing to HRQoL after LT in children would enable more targeted strategies towards optimizing best outcomes. This qualitative study aimed to explore health care providers (HCP) perceptions about HRQoL after pediatric LT. Thirteen experienced HCP participated in two focus group discussions. Data analysis via a thematic analysis approach revealed 4 major themes: “LT as a facilitator of better HRQoL,” “coping and adapting to LT,” “living with a transplanted liver,” and “the family context.” HCP identified elements that both enhance (improved physical health, peer relationship, and activities of daily living) and challenge (need for immunosuppression, transplant follow-up, and restrictions) the multidimensional domains of HRQoL. HCP perceived LT to be a stressful life-changing event for children and their families. Patients and their parents’ ability to cope and adjust positively to LT was perceived as a key contributor to better HRQoL. HCP perspective highlights the importance of promoting psychosocial support and a family-centered care delivery model towards the overarching goal of optimizing durable outcomes.

Published
2017
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18. Assessment of School Readiness in Chronic Cholestatic Liver Disease: A Pilot Study Examining Children with and without Liver Transplantation

Author

Gold, Anna, Rogers, Alaine, Cruchley, Elizabeth, Rankin, Stephanie, Parmar, Arpita, M. Kamath, Binita, Avitzur, Yaron, and Lee Ng, Vicky

Abstract

Background. Assessment of school readiness evaluates physical, social-emotional, and neuropsychological domains essential for educational success. Cognitive testing of preschool aged children with chronic liver disease may guide more timely interventions and focused efforts by health care providers. Patients and Methods. Children with chronic cholestatic liver disease diagnosed as an infant and still with their native liver (NL) and children who received a liver transplant (LT) before age of 2 years underwent testing with a battery of well-validated pediatric psychometric measures. Results. Eighteen (13 LT, 5 NL) patients (median age of 4.45 and 4.05 years, resp.) were tested. Median Full-Scale IQ was 98 (range 102–116) for LT and 116 [(range 90–106), p=0.35, NS] for NL subjects. LT recipients had significantly greater visual based difficulties, poorer caregiver rated daily living skills (p=0.04), and higher levels of executive function based difficulties (e.g., inattention, inhibition). Conclusion. This pilot study highlights the risk of neuropsychological difficulties in early school age children who were under 2 years of age at time of LT. Comprehensive early school age assessment should integrate psychometric measures to identify children at greatest risk, thus allowing for proactive educational intervention.

Published
2017
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19. Outcomes of 5-Year Survivors of Pediatric Liver Transplantation: Report on 461 Children From a North American Multicenter Registry.

Author

Lee Ng, Vicky, Fecteau, Annie, Shepherd, Ross, Magee, John, Bucuvalas, John, Alonso, Estella, McDiarmid, Suzanne, Cohen, Geoff, and Anand, Ravinder

Subjects
*LIVER transplantation
Abstract

An abstract of the article "Outcomes of 5-Year Survivors of Pediatric Liver Transplantation: Report on 461 Children From a North American Multicenter Registry," by Vicky Lee, Annie Fecteau, Ross Shepherd, John Magee, John Bucuvalas, Estella Alonso, Suzanne McDiarmid, Geoff Cohen, Ravinder Anand, and the Studies of Pediatric Liver Transplant Research Group is presented.

Published
2008

20. Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis

Author

E Natsheh, Susan, A Roberts, Eve, Ngan, Bo, Chait, Peter, and Lee Ng, Vicky

Abstract

Hemophagocytic lymphohistiocytosis (HLH) may manifest as neonatal liver failure characterized by hepatosplenomegaly, profound coagulopathy, ascites and hyperbilirubinemia. Marked hyperferritinemia may be present in these patients, mimicking perinatal hemochromatosis. Tissue specimens are critical in distinguishing these two diseases and in directing management. Clinical recognition and diagnosis of HLH can be difficult but are crucial for appropriate therapy and genetic counselling. Liver transplantation is absolutely contraindicated for patients with HLH but may be the only life-saving treatment modality for patients with perinatal hemochromatosis.

Published
2001
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