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4. Mutations in latent membrane protein 1 of Epstein-Barr virus are associated with increased risk of posttransplant lymphoproliferative disorder in children

Author

Martinez, Olivia M., Krams, Sheri M., Robien, Mark A., Lapasaran, Mary G., Arvedson, Matthew P., Reitsma, Andrea, Balachandran, Yarl, Harris-Arnold, Aleishia, Weinberg, Kenneth, Boyd, Scott D., Armstrong, Brian, Trickey, Amber, Twist, Clare J., Gratzinger, Dita, Tan, Brent, Brown, Merideth, Chin, Clifford, Desai, Dev M., Fishbein, Thomas M., Mazariegos, George V., Tekin, Akin, Venick, Robert S., Bernstein, Daniel, and Esquivel, Carlos O.

Published
2023
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5. 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. Predictors of survival following liver transplantation for pediatric hepatoblastoma and hepatocellular carcinoma: Experience from the Society of Pediatric Liver Transplantation (SPLIT)

Author

Boster, Julia M., Superina, Riccardo, Mazariegos, George V., Tiao, Gregory M., Roach, Jonathan P., Lovell, Mark A., Greffe, Brian S., Yanni, George, Leung, Daniel H., Elisofon, Scott A., McDiarmid, Suzanne V., Gupta, Nitika A., Lobritto, Steven J., Lemoine, Caroline, Stoll, Janis M., Vitola, Bernadette E., Daniel, James F., Sayed, Blayne A., Desai, Dev M., Martin, Abigail E., Amin, Arpit, Anand, Ravinder, Anderson, Sarah G., and Sundaram, Shikha S.

Published
2022
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8. Epstein–Barr virus‐associated post‐transplant lymphoproliferative disorders in pediatric transplantation: A prospective multicenter study in the United States

Author

Tajima, Tetsuya, primary, Martinez, Olivia M., additional, Bernstein, Daniel, additional, Boyd, Scott D., additional, Gratzinger, Dita, additional, Lum, Grant, additional, Sasaki, Kazunari, additional, Tan, Brent, additional, Twist, Clare J., additional, Weinberg, Kenneth, additional, Armstrong, Brian, additional, Desai, Dev M., additional, Mazariegos, George V., additional, Chin, Clifford, additional, Fishbein, Thomas M., additional, Tekin, Akin, additional, Venick, Robert S., additional, Krams, Sheri M., additional, and Esquivel, Carlos O., additional

Published
2024
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9. 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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10. 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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12. The effect of an organ procurement organization surgeon on pediatric organ utilization.

Author

Hwang, Christine S., Shubin, Andrew D., Patel, Madhukar S., Reese, Jeffrey C., Sanchez‐Vivaldi, Jorge A., Desai, Dev M., Vagefi, Parsia A., and MacConmara, Malcolm

Subjects
PEDIATRIC surgeons, LIVER transplantation, SURGEONS, HOMOGRAFTS
Abstract

Introduction: Organ procurement organizations (OPO) have started to employ transplant‐trained surgeons dedicated to organ procurement with the aim to increase allograft utilization and enhance the use of procured organs. We investigated the effects of an OPO‐employed surgeon on the procurement and utilization of organs from pediatric donors within the Southwestern Transplant Alliance OPO. Methods: OPO data were obtained for all procurements that were performed between 2014 and 2019. The analysis was performed to see if the presence of an OPO donor surgeon impacted the utilization of pediatric livers. Donor and recipient demographic data were examined between allografts procured with the presence of an OPO surgeon (OPO‐Present) and those without an OPO surgeon (OPO‐Absent). A p‐value of <.05 was considered significant. Results: Of 149 pediatric procurements, 91 included an OPO‐donor surgeon. In procurements with OPO‐Present, donors were younger (8.2 vs. 11.2, p <.05) and had longer distances to travel to the recipient center (334 vs. 175 miles p <.05), but had comparable cold ischemic times. In terms of organ share type, more OPO‐Present livers were shared nationally and there was no difference in discard rate between OPO‐Present and OPO‐Absent procurements. Finally, OPO‐Present livers were more likely to be transplanted to pediatric recipients compared to OPO‐Absent (47.3% vs. 24.1% p <.05). Conclusion: The presence of an OPO surgeon has impacted organ utilization, leading to increased transplantation of pediatric livers in pediatric recipients, and has expanded the geographical share of pediatric livers. [ABSTRACT FROM AUTHOR]

Published
2024
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14. 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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16. Biliary atresia and liver transplantation in the United States: A contemporary analysis

Author

Anouti, Ahmad, primary, Patel, Madhukar S., additional, VanWagner, Lisa B., additional, Lee, William M., additional, Fung, John J., additional, Cholankeril, George, additional, Hwang, Christine S., additional, Mufti, Arjmand R., additional, Tujios, Shannan, additional, Kerr, Thomas, additional, Rich, Nicole E., additional, Louissaint, Jeremy, additional, Desai, Dev M., additional, Vagefi, Parsia A., additional, Hanish, Steven, additional, Shah, Jigesh, additional, Singal, Amit G., additional, and Cotter, Thomas G., additional

Published
2023
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19. Normothermic machine perfusion for older transplant recipients

Author

Shubin, Andrew D., primary, Feizpour, Cyrus A., additional, Hwang, Christine S., additional, Hanish, Steven I., additional, Raschzok, Nathanael, additional, Wang, Benjamin K., additional, Desai, Dev M., additional, Shah, Jigesh A., additional, Vagefi, Parsia A., additional, MacConmara, Malcolm P., additional, and Patel, Madhukar S., additional

Published
2023
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20. Natural History of Liver Disease in a Large International Cohort of Children with Alagille syndrome: Results from The <scp>GALA</scp> Study

Author

UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, Vandriel, Shannon M., Li, Li‐Ting, She, Huiyu, Wang, Jian‐She, Gilbert, Melissa A., Jankowska, Irena, Czubkowski, Piotr, Gliwicz‐Miedzińska, Dorota, Gonzales, Emmanuel M., Jacquemin, Emmanuel, Bouligand, Jérôme, Spinner, Nancy B., Loomes, Kathleen M., Piccoli, David A., D'Antiga, Lorenzo, Nicastro, Emanuele, Sokal, Etienne, Demaret,Tanguy, Ebel, Noelle H., Feinstein, Jeffrey A., Fawaz, Rima, Nastasio, Silvia, Lacaille, Florence, Debray, Dominique, Arnell, Henrik, Fischler, Björn, Siew, Susan, Stormon, Michael, Karpen, Saul J., Romero, Rene, Kim, Kyung Mo, Baek, Woo Yim, Hardikar, Winita, Shankar, Sahana, Roberts, Amin J., Evans, Helen M., Jensen, M. Kyle, Kavan, Marianne, Sundaram, Shikha S., Chaidez, Alexander, Karthikeyan, Palaniswamy, Sanchez, Maria Camila, Cavalieri, Maria Lorena, Verkade, Henkjan J., Lee, Way Seah, Squires, James E., Hajinicolaou, Christina, Lertudomphonwanit, Chatmanee, Fischer, Ryan T., Larson‐Nath, Catherine, Mozer‐Glassberg, Yael, Arikan, Cigdem, Lin, Henry C., Quintero Bernabeu, Jesus, Alam, Seema, Kelly, Deirdre, Carvalho, Elisa, Ferreira, Cristina Targa, Indolfi, Giuseppe, Quiros‐Tejeira, Ruben E., Bulut, Pinar, Calvo, Pier Luigi, Önal, Zerrin, Valentino, Pamela L., Desai, Dev M., Eshun, John, Rogalidou, Maria, Dezsőfi, Antal, Wiecek, Sabina, Nebbia, Gabriella, Borges Pinto, Raquel, Wolters, Victorien M., Tamara, María Legarda, Zizzo, Andréanne N., Garcia, Jennifer, Schwarz, Kathleen, Beretta, Marisa, Sandahl, Thomas Damgaard, Jimenez‐Rivera, Carolina, Kerkar, Nanda, Brecelj, Jernej, Mujawar, Quais, Rock, Nathalie, Busoms, Cristina Molera, Karnsakul, Wikrom, Lurz, Eberhard, Santos‐Silva, Ermelinda, Blondet, Niviann, Bujanda, Luis, Shah, Uzma, Thompson, Richard J., Hansen, Bettina E., Kamath, Binita M., UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, Vandriel, Shannon M., Li, Li‐Ting, She, Huiyu, Wang, Jian‐She, Gilbert, Melissa A., Jankowska, Irena, Czubkowski, Piotr, Gliwicz‐Miedzińska, Dorota, Gonzales, Emmanuel M., Jacquemin, Emmanuel, Bouligand, Jérôme, Spinner, Nancy B., Loomes, Kathleen M., Piccoli, David A., D'Antiga, Lorenzo, Nicastro, Emanuele, Sokal, Etienne, Demaret,Tanguy, Ebel, Noelle H., Feinstein, Jeffrey A., Fawaz, Rima, Nastasio, Silvia, Lacaille, Florence, Debray, Dominique, Arnell, Henrik, Fischler, Björn, Siew, Susan, Stormon, Michael, Karpen, Saul J., Romero, Rene, Kim, Kyung Mo, Baek, Woo Yim, Hardikar, Winita, Shankar, Sahana, Roberts, Amin J., Evans, Helen M., Jensen, M. Kyle, Kavan, Marianne, Sundaram, Shikha S., Chaidez, Alexander, Karthikeyan, Palaniswamy, Sanchez, Maria Camila, Cavalieri, Maria Lorena, Verkade, Henkjan J., Lee, Way Seah, Squires, James E., Hajinicolaou, Christina, Lertudomphonwanit, Chatmanee, Fischer, Ryan T., Larson‐Nath, Catherine, Mozer‐Glassberg, Yael, Arikan, Cigdem, Lin, Henry C., Quintero Bernabeu, Jesus, Alam, Seema, Kelly, Deirdre, Carvalho, Elisa, Ferreira, Cristina Targa, Indolfi, Giuseppe, Quiros‐Tejeira, Ruben E., Bulut, Pinar, Calvo, Pier Luigi, Önal, Zerrin, Valentino, Pamela L., Desai, Dev M., Eshun, John, Rogalidou, Maria, Dezsőfi, Antal, Wiecek, Sabina, Nebbia, Gabriella, Borges Pinto, Raquel, Wolters, Victorien M., Tamara, María Legarda, Zizzo, Andréanne N., Garcia, Jennifer, Schwarz, Kathleen, Beretta, Marisa, Sandahl, Thomas Damgaard, Jimenez‐Rivera, Carolina, Kerkar, Nanda, Brecelj, Jernej, Mujawar, Quais, Rock, Nathalie, Busoms, Cristina Molera, Karnsakul, Wikrom, Lurz, Eberhard, Santos‐Silva, Ermelinda, Blondet, Niviann, Bujanda, Luis, Shah, Uzma, Thompson, Richard J., Hansen, Bettina E., and Kamath, Binita M.

Abstract

Background: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers and real-world data are lacking. This study aimed to elucidate the natural history of liver disease in a contemporary, international, cohort of children with ALGS. Methods: Multicenter retrospective study of children with a clinically and/or genetically confirmed ALGS diagnosis, born Jan-1997 - Aug-2019. Native liver survival (NLS) and event-free survival rates were assessed. Cox models were constructed to identify early biochemical predictors of clinically evident portal hypertension (CEPH) and NLS. Results: 1433 children (57% male) from 67 centers in 29 countries were included. 10 and 18-years NLS rates were 54.4% and 40.3%. By 10 and 18-years, 51.5% and 66.0% of ALGS children experienced ≥1 adverse liver-related event (CEPH, transplant or death). Children (>6 and ≤12 months) with median total bilirubin (TB) levels between ≥5.0 and <10.0 mg/dL had a 4.1-fold (95% CI 1.6 - 10.8) and those ≥10.0 mg/dL had an 8.0-fold (95% CI 3.4 - 18.4) increased risk of developing CEPH compared with those <5.0 mg/dL. Median TB levels between ≥5.0 and <10.0 mg/dL and >10.0 mg/dL were associated with a 4.8 (95% CI 2.4 - 9.7) and 15.6 (95% CI 8.7 - 28.2) increased risk of transplantation relative to <5.0 mg/dL. Median TB <5.0 mg/dL were associated with higher NLS rates relative to ≥5.0 mg/dL, with 79% reaching adulthood with native liver (p<0.001). Conclusions: In this large international cohort of ALGS, only 40.3% of children reach adulthood with their native liver. A TB <5.0 mg/dL between 6-and-12-months of age is associated with better hepatic outcomes. These thresholds provide clinicians with an objective tool to assist with clinical decision-making and in the evaluation of novel therapies.

Published
2023

22. Intraoperative fluid administration volumes during pediatric liver transplantation and postoperative outcomes: A multicenter analysis.

Author

Efune, Proshad N., Hoyt, Matthew J., Saynhalath, Rita, Ahn, Chul, Pearsall, Matthew F., Khan, Umar H., Feehan, Thomas, Desai, Dev M., and Szmuk, Peter

Subjects
ARTIFICIAL respiration, LIVER transplantation, TREATMENT effectiveness, LENGTH of stay in hospitals, INTENSIVE care units, CHILDREN'S health
Abstract

Introduction: Fluid administration is an important aspect of the management of children undergoing liver transplantation and may impact postoperative outcomes. Our aim was to evaluate the association between volume of intraoperative fluid administration and our primary outcome, the duration of postoperative mechanical ventilation following pediatric liver transplantation. Secondary outcomes included intensive care unit length of stay and hospital length of stay. Methods: We conducted a multicenter, retrospective cohort study using electronic data from three major pediatric liver transplant centers. Intraoperative fluid administration was indexed to weight and duration of anesthesia. Univariate and stepwise linear regression analyses were conducted. Results: Among 286 successful pediatric liver transplants, the median duration of postoperative mechanical ventilation was 10.8 h (IQR 0.0, 35.4), the median intensive care unit length of stay was 4.3 days (IQR 2.7, 6.8), and the median hospital length of stay was 13.6 days (9.8, 21.1). Univariate linear regression showed a weak correlation between intraoperative fluids and duration of ventilation (r2 =.037, p =.001). Following stepwise linear regression, intraoperative fluid administration remained weakly correlated (r2 =.161, p =.04) with duration of postoperative ventilation. The following variables were also independently correlated with duration of ventilation: center (Riley Children's Health versus Children's Health Dallas, p =.001), and open abdominal incision after transplant (p =.001). Discussion: The amount of intraoperative fluid administration is correlated with duration of postoperative mechanical ventilation in children undergoing liver transplantation, however, it does not seem to be a strong factor. Conclusions: Other modifiable factors should be sought which may lead to improved postoperative outcomes in this highly vulnerable patient population. [ABSTRACT FROM AUTHOR]

Published
2023
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24. No Stone Left Unturned: Utilization of an Organ Procurement Organization Donor Surgeon at Procurement Reduces Discards of Marginal Liver Allografts

Author

Shubin, Andrew D., primary, MacConmara, Malcolm P., additional, Patel, Madhukar S., additional, Wang, Benjamin K., additional, Feizpour, Cyrus A., additional, Reese, Jeffrey, additional, Niles, Patricia A., additional, Shah, Jigesh A., additional, Desai, Dev M., additional, De Gregorio, Lucia, additional, Hanish, Steven I., additional, Vagefi, Parsia A., additional, and Hwang, Christine S., additional

Published
2022
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26. Delayed graft function in pediatric living donor kidney transplantation.

Author

Hwang, Christine S., Kadakia, Yash, Sanchez‐Vivaldi, Jorge A., Patel, Madhukar S., Shah, Jigesh A., DeGregorio, Lucia, Desai, Dev M., Vagefi, Parsia A., and MacConmara, Malcolm

Subjects
KIDNEY transplantation, CHILD patients, GRAFT survival, SURVIVAL rate, FOCAL segmental glomerulosclerosis
Abstract

Background: Pediatric recipients of living donor kidneys have a low rate of delayed graft function (DGF). We examined the incidence, risk factors and outcomes of DGF in pediatric patients who received a living donor allograft. Methods: The STARfile was queried to examine all pediatric patients transplanted with a living donor kidney between 2000 and 2020. Donor and recipient demographic data were examined, as were survival and outcomes. Recipients were stratified into DGF and no DGF groups. DGF was defined as the need for dialysis within the first week after transplant. Results: 6480 pediatric patients received a living donor (LD) kidney transplant during the study period. 269 (4.2%) developed DGF post‐transplant. Donors were similar in age, creatinine, and cold ischemia time. Recipients of kidneys with DGF were similar in age, sensitization status and HLA mismatch. Focal segmental glomerulosclerosis (FSGS) was the most common diagnosis in recipients with DGF, and allograft thrombosis was the most common cause of graft loss in this group. Small recipients (weight < 15 kg) were found to have a significantly higher rate of DGF. Length of stay doubled in recipients with DGF, and rejection rates were higher post‐transplant. Recipients of LD kidneys who developed DGF had significantly worse 1 year allograft survival (67% vs. 98%, p <.0001). Conclusions: Pediatric living donor kidney transplant recipients who experience DGF have significantly poorer allograft survival. Optimizing the donor and recipient matching to avoid compounding risks may allow for better outcomes. [ABSTRACT FROM AUTHOR]

Published
2023
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45. Ligand-mediated negative regulation of a chimeric transmembrane receptor tyrosine phosphatase

Author

Desai, Dev M., Sap, Jan, Schlessinger, Joseph, and Weiss, Arthur

Subjects
Phosphatases -- Research, Cellular signal transduction -- Research, T cells -- Receptors, Mosaicism -- Research, Biological sciences
Abstract

A chimeric protein in which the extracellular and transmembrane domains of CD45 were replaced with those of the epidermal growth factor receptor was constructed to determine whether the extracellular domain of CD45 influenced its function. CD45 may influence T cell surface signaling molecules. The ability of this chimera to restore T cell receptor signal transduction function in a CD45-deficient T cell line was examined. The findings suggest that ligand-mediated regulation of receptor-protein tyrosine phosphatases may have mechanistic similarities with receptor tyrosine kinases.

Published
1993

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