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1. Prognosis of Children Undergoing Liver Transplantation: A 30-Year European Study

Author

Baumann, U, Karam, V, Adam, R, Fondevila, C, Dhawan, A, Sokal, E, Jacquemin, E, Kelly, D, Grabhorn, E, Pawlowska, J, D'Antiga, L, Vega, P, Debray, D, Polak, W, de Ville de Goyet, J, Verkade, H, Baumann U., Karam V., Adam R., Fondevila C., Dhawan A., Sokal E., Jacquemin E., Kelly D. A., Grabhorn E., Pawlowska J., D'Antiga L., Vega P. J., Debray D., Polak W. G., de Ville de Goyet J., Verkade H. J., Baumann, U, Karam, V, Adam, R, Fondevila, C, Dhawan, A, Sokal, E, Jacquemin, E, Kelly, D, Grabhorn, E, Pawlowska, J, D'Antiga, L, Vega, P, Debray, D, Polak, W, de Ville de Goyet, J, Verkade, H, Baumann U., Karam V., Adam R., Fondevila C., Dhawan A., Sokal E., Jacquemin E., Kelly D. A., Grabhorn E., Pawlowska J., D'Antiga L., Vega P. J., Debray D., Polak W. G., de Ville de Goyet J., and Verkade H. J.

Abstract

OBJECTIVES: The European Liver Transplant Registry has been collecting data on virtually all pediatric liver transplant (PLT) procedures in Europe since 1968. We analyzed patient outcome over time and identified parameters associated with long-term patient outcome. METHODS: Participating centers and European organ-sharing organizations provided retrospective data to the European Liver Transplant Registry. To identify trends, data were grouped into consecutive time spans: era A: before 2000, era B: 2000 to 2009, and the current era, era C: since 2010. RESULTS: From June 1968 until December 2017, 16 641 PLT were performed on 14 515 children by 133 centers. The children <7 years of age represented 58% in era A, and 66% in the current era (P <.01). The main indications for PLT were congenital biliary diseases (44%) and metabolic diseases (18%). Patient survival at 5 years is currently 86% overall and 97% in children who survive the first year after PLT. The survival rate has improved from 74% in era A to 83% in era B and 85% in era C (P <.0001). Low-volume centers (<5 PLT/year) represented 75% of centers but performed only 19% of PLT and were associated with a decreased survival rate. In the current era, however, survival rates has become irrespective of volume. Infection is the leading cause of death (4.1%), followed by primary nonfunction of the graft (1.4%). CONCLUSIONS: PLT has become a highly successful medical treatment that should be considered for all children with end-stage liver disease. The main challenge for further improving the prognosis remains the early postoperative period.

Published
2022

2. Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group

Author

Ranucci, G, Della Corte, C, Alberti, D, Bondioni, M, Boroni, G, Calvo, P, Cananzi, M, Candusso, M, Clemente, M, D'Antiga, L, Degrassi, I, De Ville De Goyet, J, Di Dato, F, Di Giorgio, A, Vici, C, Ferrari, F, Francalanci, P, Fuoti, M, Fusaro, F, Gaio, P, Grimaldi, C, Iascone, M, Indolfi, G, Iorio, R, Maggiore, G, Mandato, C, Matarazzo, L, Monti, L, Mosca, F, Nebbia, G, Nuti, F, Paolella, G, Pinon, M, Roggero, P, Sciveres, M, Serranti, D, Spada, M, Vajro, P, Nicastro, E, Ranucci G., Della Corte C., Alberti D., Bondioni M. P., Boroni G., Calvo P. L., Cananzi M., Candusso M., Clemente M. G., D'Antiga L., Degrassi I., De Ville De Goyet J., Di Dato F., Di Giorgio A., Vici C. D., Ferrari F., Francalanci P., Fuoti M., Fusaro F., Gaio P., Grimaldi C., Iascone M., Indolfi G., Iorio R., Maggiore G., Mandato C., Matarazzo L., Monti L., Mosca F., Nebbia G., Nuti F., Paolella G., Pinon M., Roggero P., Sciveres M., Serranti D., Spada M., Vajro P., Nicastro E., Ranucci, G, Della Corte, C, Alberti, D, Bondioni, M, Boroni, G, Calvo, P, Cananzi, M, Candusso, M, Clemente, M, D'Antiga, L, Degrassi, I, De Ville De Goyet, J, Di Dato, F, Di Giorgio, A, Vici, C, Ferrari, F, Francalanci, P, Fuoti, M, Fusaro, F, Gaio, P, Grimaldi, C, Iascone, M, Indolfi, G, Iorio, R, Maggiore, G, Mandato, C, Matarazzo, L, Monti, L, Mosca, F, Nebbia, G, Nuti, F, Paolella, G, Pinon, M, Roggero, P, Sciveres, M, Serranti, D, Spada, M, Vajro, P, Nicastro, E, Ranucci G., Della Corte C., Alberti D., Bondioni M. P., Boroni G., Calvo P. L., Cananzi M., Candusso M., Clemente M. G., D'Antiga L., Degrassi I., De Ville De Goyet J., Di Dato F., Di Giorgio A., Vici C. D., Ferrari F., Francalanci P., Fuoti M., Fusaro F., Gaio P., Grimaldi C., Iascone M., Indolfi G., Iorio R., Maggiore G., Mandato C., Matarazzo L., Monti L., Mosca F., Nebbia G., Nuti F., Paolella G., Pinon M., Roggero P., Sciveres M., Serranti D., Spada M., Vajro P., and Nicastro E.

Abstract

Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.

Published
2022

3. Preliminary Analysis of the Impact of COVID-19 Outbreak on Italian Liver Transplant Programs

Author

Agnes, S, Andorno, E, Avolio, A, Baccarani, U, Carraro, A, Cescon, M, Cillo, U, Colledan, M, De Carlis, L, De Simone, P, De Ville De Goyet, J, Di Benedetto, F, Ettorre, G, Gringeri, E, Gruttadauria, S, Lupo, L, Mazzaferro, V, Regalia, E, Romagnoli, R, Rossi, G, Rossi, M, Spada, M, Tisone, G, Vennarecci, G, Vivarelli, M, Zamboni, F, Boggi, U, Agnes S., Andorno E., Avolio A. W., Baccarani U., Carraro A., Cescon M., Cillo U., Colledan M., De Carlis L., De Simone P., De Ville De Goyet J., Di Benedetto F., Ettorre G. M., Gringeri E., Gruttadauria S., Lupo L. G., Mazzaferro V., Regalia E., Romagnoli R., Rossi G. E., Rossi M., Spada M., Tisone G., Vennarecci G., Vivarelli M., Zamboni F., Boggi U., Agnes, S, Andorno, E, Avolio, A, Baccarani, U, Carraro, A, Cescon, M, Cillo, U, Colledan, M, De Carlis, L, De Simone, P, De Ville De Goyet, J, Di Benedetto, F, Ettorre, G, Gringeri, E, Gruttadauria, S, Lupo, L, Mazzaferro, V, Regalia, E, Romagnoli, R, Rossi, G, Rossi, M, Spada, M, Tisone, G, Vennarecci, G, Vivarelli, M, Zamboni, F, Boggi, U, Agnes S., Andorno E., Avolio A. W., Baccarani U., Carraro A., Cescon M., Cillo U., Colledan M., De Carlis L., De Simone P., De Ville De Goyet J., Di Benedetto F., Ettorre G. M., Gringeri E., Gruttadauria S., Lupo L. G., Mazzaferro V., Regalia E., Romagnoli R., Rossi G. E., Rossi M., Spada M., Tisone G., Vennarecci G., Vivarelli M., Zamboni F., and Boggi U.

Abstract

Liver Transplant Programs in Italy have faced a sequela of management and clinical decision-making problems due to the high incidence in some regions of the country of severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2). The Italian Society for Organ Transplantation (SITO) and the Board of Liver Transplant Program Directors issued a survey to assess the initial impact of this pandemic event on the routine activity of 22 Italian Liver Transplant Programs. One hundred percent of participants completed the survey within a few days. The analysis is presented dividing the centers in two macro-areas: north-central Italy and south-central Italy. The reason for this is that the two areas had a different incidence of the infection and because they have distinctive rates of cadaveric donation. Overall, all centers remained open although a reduction in the activity was noted. Transplant Programs reduced their outpatient activity both in terms of pre-transplant evaluation (68% of the centers) and transplant recipient follow-up (100%); a reduction in transplant activity was observed in the first two weeks of March only in the north-central macro area (23 LTs vs 39 in 2018 and 60 in 2019); overall, SARS-CoV-2 infection was registered for 24 liver transplant recipients and 37 health care providers in liver transplant units. In the perspective of the increasing magnitude of the epidemic, more data will be required to define appropriate strategies for the increasingly complex management of liver transplant patients.

Published
2020

5. European Liver Transplant Registry: Donor and transplant surgery aspects of 16,641 liver transplantations in children

Author

de Ville de Goyet, J, Baumann, U, Karam, V, Adam, R, Nadalin, S, Heaton, N, Reding, R, Branchereau, S, Mirza, D, Klempnauer, J, Fischer, L, Kalicinski, P, Colledan, M, Lopez Santamaria, M, de Kleine, R, Chardot, C, Yilmaz, S, Kilic, M, Boillot, O, di Francesco, F, Polak, W, Verkade, H, de Ville de Goyet, Jean, Baumann, Ulrich, Karam, Vincent, Adam, René, Nadalin, Silvio, Heaton, Nigel, Reding, Raymond, Branchereau, Sophie, Mirza, Darius, Klempnauer, Jürgen L, Fischer, Lutz, Kalicinski, Piotr, Colledan, Michele, Lopez Santamaria, Manuel, de Kleine, Ruben H, Chardot, Christophe, Yilmaz, Sezai, Kilic, Murat, Boillot, Olivier, di Francesco, Fabrizio, Polak, Wojciech G, Verkade, Henkjan J, de Ville de Goyet, J, Baumann, U, Karam, V, Adam, R, Nadalin, S, Heaton, N, Reding, R, Branchereau, S, Mirza, D, Klempnauer, J, Fischer, L, Kalicinski, P, Colledan, M, Lopez Santamaria, M, de Kleine, R, Chardot, C, Yilmaz, S, Kilic, M, Boillot, O, di Francesco, F, Polak, W, Verkade, H, de Ville de Goyet, Jean, Baumann, Ulrich, Karam, Vincent, Adam, René, Nadalin, Silvio, Heaton, Nigel, Reding, Raymond, Branchereau, Sophie, Mirza, Darius, Klempnauer, Jürgen L, Fischer, Lutz, Kalicinski, Piotr, Colledan, Michele, Lopez Santamaria, Manuel, de Kleine, Ruben H, Chardot, Christophe, Yilmaz, Sezai, Kilic, Murat, Boillot, Olivier, di Francesco, Fabrizio, Polak, Wojciech G, and Verkade, Henkjan J

Abstract

Background and Aims: The European Liver Transplant Registry (ELTR) has collected data on liver transplant procedures performed in Europe since 1968. Approach and Results: Over a 50-year period (1968–2017), clinical and laboratory data were collected from 133 transplant centers and analyzed retrospectively (16,641 liver transplants in 14,515 children). Data were analyzed according to three successive periods (A, before 2000; B, 2000–2009; and C, since 2010), studying donor and graft characteristics and graft outcome. The use of living donors steadily increased from A to C (A, n = 296 [7%]; B, n = 1131 [23%]; and C, n = 1985 [39%]; p = 0.0001). Overall, the 5-year graft survival rate has improved from 65% in group A to 75% in group B (p < 0.0001) and to 79% in group C (B versus C, p < 0.0001). Graft half-life was 31 years, overall; it was 41 years for children who survived the first year after transplant. The late annual graft loss rate in teenagers is higher than that in children aged <12 years and similar to that of young adults. No evidence for accelerated graft loss after age 18 years was found. Conclusions: Pediatric liver transplantation has reached a high efficacy as a cure or treatment for severe liver disease in infants and children. Grafts that survived the first year had a half-life similar to standard human half-life. Transplantation before or after puberty may be the pivot-point for lower long-term outcome in children. Further studies are necessary to revisit some old concepts regarding transplant benefit (survival time) for small children, the role of recipient pathophysiology versus graft aging, and risk at transition to adult age.

Published
2022

6. A national mandatory-split liver policy: A report from the Italian experience

Author

Angelico, R, Trapani, S, Spada, M, Colledan, M, de Ville de Goyet, J, Salizzoni, M, De Carlis, L, Andorno, E, Gruttadauria, S, Ettorre, G, Cescon, M, Rossi, G, Risaliti, A, Tisone, G, Tedeschi, U, Vivarelli, M, Agnes, S, De Simone, P, Lupo, L, Di Benedetto, F, Santaniello, W, Zamboni, F, Mazzaferro, V, Rossi, M, Puoti, F, Camagni, S, Grimaldi, C, Gringeri, E, Rizzato, L, Nanni Costa, A, Cillo, U, Angelico R., Trapani S., Spada M., Colledan M., de Ville de Goyet J., Salizzoni M., De Carlis L., Andorno E., Gruttadauria S., Ettorre G. M., Cescon M., Rossi G., Risaliti A., Tisone G., Tedeschi U., Vivarelli M., Agnes S., De Simone P., Lupo L. G., Di Benedetto F., Santaniello W., Zamboni F., Mazzaferro V., Rossi M., Puoti F., Camagni S., Grimaldi C., Gringeri E., Rizzato L., Nanni Costa A., Cillo U., Angelico, R, Trapani, S, Spada, M, Colledan, M, de Ville de Goyet, J, Salizzoni, M, De Carlis, L, Andorno, E, Gruttadauria, S, Ettorre, G, Cescon, M, Rossi, G, Risaliti, A, Tisone, G, Tedeschi, U, Vivarelli, M, Agnes, S, De Simone, P, Lupo, L, Di Benedetto, F, Santaniello, W, Zamboni, F, Mazzaferro, V, Rossi, M, Puoti, F, Camagni, S, Grimaldi, C, Gringeri, E, Rizzato, L, Nanni Costa, A, Cillo, U, Angelico R., Trapani S., Spada M., Colledan M., de Ville de Goyet J., Salizzoni M., De Carlis L., Andorno E., Gruttadauria S., Ettorre G. M., Cescon M., Rossi G., Risaliti A., Tisone G., Tedeschi U., Vivarelli M., Agnes S., De Simone P., Lupo L. G., Di Benedetto F., Santaniello W., Zamboni F., Mazzaferro V., Rossi M., Puoti F., Camagni S., Grimaldi C., Gringeri E., Rizzato L., Nanni Costa A., and Cillo U.

Abstract

To implement split liver transplantation (SLT) a mandatory-split policy has been adopted in Italy since August 2015: donors aged 18-50 years at standard risk are offered for SLT, resulting in a left-lateral segment (LLS) graft for children and an extended-right graft (ERG) for adults. We aim to analyze the impact of the new mandatory-split policy on liver transplantation (LT)-waiting list and SLT outcomes, compared to old allocation policy. Between August 2015 and December 2016 out of 413 potentially “splittable” donors, 252 (61%) were proposed for SLT, of whom 53 (21%) donors were accepted for SLT whereas 101 (40.1%) were excluded because of donor characteristics and 98 (38.9%) for absence of suitable pediatric recipients. The SLT rate augmented from 6% to 8.4%. Children undergoing SLT increased from 49.3% to 65.8% (P =.009) and the pediatric LT-waiting list time dropped (229 [10-2121] vs 80 [12-2503] days [P =.045]). The pediatric (4.5% vs 2.5% [P =.398]) and adult (9.7% to 5.2% [P <.001]) LT-waiting list mortality reduced; SLT outcomes remained stable. Retransplantation (HR = 2.641, P =.035) and recipient weight >20 kg (HR = 5.113, P =.048) in LLS, and ischemic time >8 hours (HR = 2.475, P =.048) in ERG were identified as predictors of graft failure. A national mandatory-split policy maximizes the SLT donor resources, whose selection criteria can be safely expanded, providing favorable impact on the pediatric LT-waiting list and priority for adult sick LT candidates.

Published
2019

12. Preliminary Analysis of the Impact of COVID-19 Outbreak on Italian Liver Transplant Programs.

Author

Agnes, Salvatore, Andorno, E, Avolio, Alfonso Wolfango, Baccarani, U, Carraro, A, Cescon, M, Cillo, U, Colledan, M, De Carlis, L, De Simone, P, De Ville De Goyet, J, Di Benedetto, F, Ettorre, Gm, Gringeri, E, Gruttadauria, S, Lupo, Lg, Mazzaferro, V, Regalia, E, Romagnoli, R, Rossi, Ge, Rossi, M, Spada, M, Tisone, G, Vennarecci, G, Vivarelli, M, Zamboni, F, Boggi, U., Agnes S (ORCID:0000-0002-3341-4221), Avolio AW (ORCID:0000-0003-2491-7625), Agnes, Salvatore, Andorno, E, Avolio, Alfonso Wolfango, Baccarani, U, Carraro, A, Cescon, M, Cillo, U, Colledan, M, De Carlis, L, De Simone, P, De Ville De Goyet, J, Di Benedetto, F, Ettorre, Gm, Gringeri, E, Gruttadauria, S, Lupo, Lg, Mazzaferro, V, Regalia, E, Romagnoli, R, Rossi, Ge, Rossi, M, Spada, M, Tisone, G, Vennarecci, G, Vivarelli, M, Zamboni, F, Boggi, U., Agnes S (ORCID:0000-0002-3341-4221), and Avolio AW (ORCID:0000-0003-2491-7625)

Abstract

Liver Transplant Programs in Italy have faced a sequela of management and clinical decisionmaking problems due to the high incidence in some regions of the country of severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2). The Italian Society for Organ Transplantation (SITO) and the Board of Liver Transplant Program Directors issued a survey to assess the initial impact of this pandemic event on the routine activity of 22 Italian Liver Transplant Programs. One hundred percent of participants completed the survey within a few days. The analysis is presented dividing the centers in two macro-areas: north-central Italy and south-central Italy. The reason for this is that the two areas had a different incidence of the infection and because they have distinctive rates of cadaveric donation. Overall, all centers remained open although a reduction in the activity was noted. Transplant Programs reduced their outpatient activity both in terms of pre-transplant evaluation (68% of the centers) and transplant recipient follow-up (100%); a reduction in transplant activity was observed in the first two weeks of March only in the north-central macro area (23 LTs vs 39 in 2018 and 60 in 2019); overall, SARS-CoV-2 infection was registered for 24 liver transplant recipients and 37 health care providers in liver transplant units. In the perspective of the increasing magnitude of the epidemic, more data will be required to define appropriate strategies for the increasingly complex management of liver transplant patients.

Published
2020

21. Pediatric chronic intestinal failure in italy: Report from the 2016 survey on behalf of Italian society for gastroenterology, hepatology and nutrition (SIGENP)

Author

Diamanti, A, Capriati, T, Gandullia, P, Di Leo, G, Lezo, A, Lacitignola, L, Spagnuolo, M, Gatti, S, D'Antiga, L, Verlato, G, Roggero, P, Amarri, S, Baldassarre, M, Cirillo, F, Elia, D, Boldrini, R, Campanozzi, A, Catassi, C, Aloi, M, Romano, C, Candusso, M, Cecchi, N, Bellini, T, Tyndall, E, Fusaro, F, Caldaro, T, Alberti, D, Gamba, P, Lima, M, Bagolan, P, De Ville de Goyet, J, Dall'Oglio, L, Spada, M, Grandi, F, Diamanti A, Capriati T, Gandullia P, Di Leo G, Lezo A, Lacitignola L, Spagnuolo MI, Gatti S, D'Antiga L, Verlato G, Roggero P, Amarri S, Baldassarre ME, Cirillo F, Elia D, Boldrini R, Campanozzi A, Catassi C, Aloi M, Romano C, Candusso M, Cecchi N, Bellini T, Tyndall E, Fusaro F, Caldaro T, Alberti D, Gamba P, Lima M, Bagolan P, De Ville de Goyet J, Dall'Oglio L, Spada M, Grandi F., Diamanti, A, Capriati, T, Gandullia, P, Di Leo, G, Lezo, A, Lacitignola, L, Spagnuolo, M, Gatti, S, D'Antiga, L, Verlato, G, Roggero, P, Amarri, S, Baldassarre, M, Cirillo, F, Elia, D, Boldrini, R, Campanozzi, A, Catassi, C, Aloi, M, Romano, C, Candusso, M, Cecchi, N, Bellini, T, Tyndall, E, Fusaro, F, Caldaro, T, Alberti, D, Gamba, P, Lima, M, Bagolan, P, De Ville de Goyet, J, Dall'Oglio, L, Spada, M, Grandi, F, Diamanti A, Capriati T, Gandullia P, Di Leo G, Lezo A, Lacitignola L, Spagnuolo MI, Gatti S, D'Antiga L, Verlato G, Roggero P, Amarri S, Baldassarre ME, Cirillo F, Elia D, Boldrini R, Campanozzi A, Catassi C, Aloi M, Romano C, Candusso M, Cecchi N, Bellini T, Tyndall E, Fusaro F, Caldaro T, Alberti D, Gamba P, Lima M, Bagolan P, De Ville de Goyet J, Dall'Oglio L, Spada M, and Grandi F.

Abstract

Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.

Published
2017

23. Congenital porto-systemic shunts in children: preliminary results from the IRCPSS

Author

McLin, Valérie, Franchi-Abella, S, Debray, D., Korff, S, Casotti V, Colledan, M, d'Antiga, L, de Ville de Goyet, J, Lurz, E., Stéphenne, Xavier, ROCK, Nathalie, 52nd Annual Meeting ESPGHAN (European Society for Pediatric Gastroenterology, Hepatology and Nutrition), UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, and UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique

Abstract

Objectives and Study: Our aim was to use data from patients followed by centers participating in the first International Registry for Congenital Porto-Systemic Shunts (IRCPSS) to identify trends that might inform therapeutic strategies according to shunt anatomy. Methods: Data were collected retrospectively (descriptive patient data, mode of presentation, complications, management). Intrahepatic (IH) was defined as porto-hepatic and extrahepatic (EH) as a porto-systemic communication occurring either upstream of the portal vein or originating at the portal vein. Persistent ductus venosus were considered EH. Results: 246 children were identified with either intrahepatic (IH) or extrahepatic shunts (EH): 122 IH and 120 EH. 3 patients had both IH and EH. 24% of all CPSS were identified pre-natally. Among patients diagnosed pre-natally, a majority had IH shunts (75%). 189 (76%) patients were diagnosed post-natally at a mean age of 39.1 mo (0-200) for IH and 61.9mo (0-192) for EH. IH and EH shunts were equally frequent when diagnosed after birth. IH shunts were more often an incidental finding. Among patients diagnosed post-natally, symptoms were equally frequent among patients with IH (57%) or EH (61%) CPSS and are summarized in Table 1. In addition, patients with EH CPSS were more likely to have several symptoms than patients with IH CPSS. They were also more likely to have liver nodules on imaging (40.7% vs 26%). Closure: 184 children with CPSS were closed including 11 patients with 2 steps closure. Among these 184 patients 54 % of IH CPSS and 5% of EH CPSS closed spontaneously. 46% of IH CPSS required medical or surgical closure of which nearly 40% for a preventive indication. 94% of patients with EH CPSS were closed through a procedure, of which 41% were preventive. Conclusion: IH and EH shunts were equally frequent in this multicenter retrospective cohort of CPSS in children. CPSS are a cause of severe symptoms in children and should be sought in infants with hypoglycemia or cholestasis. In older children, they should be considered in the differential diagnosis of liver nodules, cardiopulmonary symptoms or neurocognitive deficits. Given the potential severity of complications, preventative closure was often performed, although timing and approach need further study, something which the IRCPSS aims to address.

Published
2019

30. Congenital porto-systemic shunts in children: preliminary results from the IRCPSS

Author

UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, McLin, V., Franchi-Abella, S, Debray, D., Korff, S., Casotti, V., Colledan, M., d'Antiga, L., de Ville de Goyet, J., Lurz, E., Stéphenne, Xavier, ROCK, Nathalie, Hierro, L., Kanavaki, A., Lipsich, J., Lopez-Santamaria, M., Magnusson, M., Mozer-Glassberg, Y., Ponce, M.D., Prieto, C., Ugurcan, O.D., Varma, S., Guerin, F., Pop,T., Wildhaber, B., Gonzales, E., The International Liver Congress (EASL), UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, McLin, V., Franchi-Abella, S, Debray, D., Korff, S., Casotti, V., Colledan, M., d'Antiga, L., de Ville de Goyet, J., Lurz, E., Stéphenne, Xavier, ROCK, Nathalie, Hierro, L., Kanavaki, A., Lipsich, J., Lopez-Santamaria, M., Magnusson, M., Mozer-Glassberg, Y., Ponce, M.D., Prieto, C., Ugurcan, O.D., Varma, S., Guerin, F., Pop,T., Wildhaber, B., Gonzales, E., and The International Liver Congress (EASL)

Abstract

Background: • Congenital portosystemic shunts (CPSS) are increasingly diagnosed in children and adults owing to high resolution imaging. • Their natural history is incompletely understood. • CPSS may be associated with life-threatening complications. • There are no established predictors of spontaneous closure. •Among patients in whom CPSS does not close spontaneously, is unclear which patients will develop complications, consequently which shunts require closure. Aim: To use preliminary data from 15/28 centers participating in the International Registry for Congenital Porto-Systemic Shunts (IRCPSS) to identify trends that might inform management decisions

Published
2019

34. COMMENTARY

Author

Kelly, D A and de Ville de Goyet, J

Published
2003

45. CPAP by helmet for treatment of acute respiratory failure after pediatric liver transplantation

Author

Chiusolo, F., Fanelli, V., Ciofi degli Atti, M. L., Conti, Giorgio, Tortora, F., Pariante, R., Ravà, L., Grimaldi, C., de Ville de Goyet, J., Picardo, S., Conti, G. (ORCID:0000-0002-8566-9365), Chiusolo, F., Fanelli, V., Ciofi degli Atti, M. L., Conti, Giorgio, Tortora, F., Pariante, R., Ravà, L., Grimaldi, C., de Ville de Goyet, J., Picardo, S., and Conti, G. (ORCID:0000-0002-8566-9365)

Abstract

ARF after pediatric liver transplantation accounts for high rate of morbidity and mortality associated with this procedure. The role of CPAP in postoperative period is still unknown. The aim of the study was to describe current practice and risk factors associated with the application of helmet CPAP. In this retrospective observational cohort study, 119 recipients were divided into two groups based on indication to CPAP after extubation. Perioperative variables were studied, and determinants of CPAP application were analyzed in a multivariate logistic model. Sixty patients (60/114) developed ARF and were included in the CPAP group. No differences were found between the two groups for primary disease, graft type, and blood product transfused. At multivariate analysis, weight <11 kg (OR = 2.9; 95% CI = 1.1-7.3; P =.026), PaO2/FiO2<380 before extubation (OR = 5.4; 95% CI = 2.1-13.6; P <.001), need of vasopressors (OR = 2.6; 95% CI = 1.1-6.4; P =.038), and positive fluid balance >148 mL/kg (OR = 4.0; 95% CI = 1.6-10.1; P =.004) were the main determinants of CPAP application. In the CPAP group, five patients (8.4%) needed reintubation. Pediatric liver recipients with lower weight, higher need of inotropes/vasopressors, higher positive fluid balance after surgery, and lower PaO2/FiO2before extubation were at higher odds of developing ARF needing CPAP application.

Published
2018

46. Liver transplantation in children less than 1 year of age

Author

Sokal, E.M., Veyckemans, F., de Ville de Goyet, J., Moulin, D., Van Hoorebeeck, N., Alberti, D., Buts, J.P., Rahier, J., Van Obbergh, L., Clapuyt, P., Carlier, M., Claus, D., Latinne, D., de Hemptinne, B., and Otte, J.B.

Subjects
Transplantation of organs, tissues, etc. -- Demographic aspects, Transplantation of organs, tissues, etc. -- Patient outcomes, Liver -- Transplantation, Health
Abstract

Liver transplantation has improved the survival of children with end-stage liver disease, and is frequently the only curative treatment for these patients. More infants are being given liver transplants because of success with older children, but many transplant centers do not accept infants, even if they have life-threatening liver disease. The outcomes of liver transplantation in 17 infants under one year of age are reported. Of the 17 patients, 5 received more than one graft and a total of 26 liver transplantations were performed. Eleven patients survived to be discharged from the hospital, and 10 survived a year or more after hospital discharge. Four of the six patients who died following transplantation had required hospitalization prior to surgery. Surgical complications included blood clots in the hepatic artery (of the liver). Bacterial infections were common; two patients died as a result of complications associated with viral infections. Eleven patients experienced 18 episodes of graft rejection. One patient with rejection required a second transplant, but nine responded successfully to medical treatment. Although several patients had impaired kidney function following surgery, it was similar to their pretransplant status. Liver function is normal in all, but two of the surviving patients. Three of the older survivors have entered primary school and have no known deficits, while a fourth is developmentally delayed. The findings suggest that liver transplantation is a feasible treatment for children under one year of age, but should only be performed in medical facilities which are capable of managing these patients. The 64.7 percent survival rate of these infants, while not out of line, is less than the 75.8 percent survival of all 139 children who received liver transplants at this facility during a 5-year period. Careful selection of candidates and preoperative nutritional boosting are highly desirable. Further studies are needed to determine the risk factors of liver transplantation, which are specific to infants. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1990

47. Size reduction of the donor liver is a safe way to alleviate the shortage of size-matched organs in pediatric liver transplantation

Author

Otte, J.B., de Ville de Goyet, J., Sokal, E., Alberti, D., Moulin, D., de Hemptinne, B., Veyckemans, F., Van Obbergh, L., Carlier, M., Clapuyt, Ph., Claus, D., and Jamart, J.

Subjects
Transplantation of organs, tissues, etc. -- Demographic aspects, Liver -- Transplantation, Health
Abstract

A major drawback in pediatric liver transplantation is the severe shortage of appropriately sized livers. Between 25 and 30 percent of children waiting for liver transplants die before an appropriate donor organ can be found. When liver transplantation possible, it offers the hope of long-term survival. A report is presented of 141 transplantations performed on 117 children using 54 reduced-size grafts. Ten children were under one year of age; 63 were between l and 3 years old; 21 were between 3 and 6; and, 23 were between 6 and 14 years old. The most common indication for transplant was biliary atresia, a congenital absence of one or more of the biliary structures, which affected 86 patients. Reduced-size livers were used in both urgent and elective cases. There were 83 full-size livers and 54 reduced-size livers transplanted; 4 were considered other technical variants. The one-year survival rate for the entire group was 77 percent; for the full-size liver recipients it was 82 percent, and for the reduced-size liver recipients it was 68 percent. Graft loss occurred with 26 (31 percent) of the full-size livers, and 24 (44 percent) of the reduced-size livers. Of the 74 children who had functioning livers six months after transplant, 67 percent had completely normal liver function tests, and 14 percent had only slightly abnormal liver tests. Use of this technique has reduced the mortality of children on the waiting list to 14 percent. It is concluded that the technique of reduced-size liver transplantation is safe and reliable and is recommended for treatment in urgent and elective cases. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1990

50. Balancing donor and recipient risk factors in liver transplantation: the value of D-MELD with particular reference to HCV recipients

Author

Avolio, A, Cillo, U, Salizzoni, M, DE CARLIS, LUCIANO GREGORIO, Colledan, M, Gerunda, G, Mazzaferro, V, Tisone, G, Romagnoli, R, Caccamo, L, Rossi, M, Vitale, A, Cucchetti, A, Lupo, L, Gruttadauria, S, Nicolotti, N, Burra, P, Gasbarrini, A, Agnes, S, Lirosi, M, Miele, L, Pompili, M, Siciliano, M, Perilli, V, Gaspari, R, Castagneto, M, Lupo, F, Tandoi, F, De Carlis, L, Mangoni, I, Belli, L, Pinna, A, Cescon, M, Gridelli, B, Li Petri, S, Volpes, R, Pinelli, D, Fagiuoli, S, Montalti, R, Regalia, E, Rossi, G, Antonelli, B, Berloco, P, Lai, Q, Risaliti, A, Nicolini, D, Valente, U, Gelli, M, Morelli, N, Zamboni, F, Tondolo, V, Ettorre, G, Vennarecci, G, Bresadola, F, Baccarani, U, Toniutto, P, Manzia, T, Anselmo, A, Angelico, M, Calise, F, Scuderi, V, Romano, M, Rendina, M, Barone, M, Cuomo, O, Perrella, A, Santaniello, W, Donataccio, M, Dalle Ore, G, De Ville De Goyet, J, Monti, L, De Waure, C., Avolio A.W., Cillo U., Salizzoni M., De Carlis L., Colledan M., Gerunda G.E., Mazzaferro V., Tisone G., Romagnoli R., Caccamo L., Rossi M., Vitale A., Cucchetti A., Lupo L., Gruttadauria S., Nicolotti N., Burra P., Gasbarrini A., Agnes S., Lirosi M.C., Miele L., Pompili M., Siciliano M., Perilli V., Gaspari R., Castagneto M., Tandoi F., Mangoni I., Belli L., Pinna A.D., Cescon M., Gridelli B., Li Petri S., Volpes R., Pinelli D., Fagiuoli S., Montalti R., Regalia E., Rossi G., Antonelli B., Berloco P., Lai Q., Risaliti A., Nicolini D., Valente U., Gelli M., Morelli N., Zamboni F., Tondolo V., Brotzu G., Ettorre G.M., Vennarecci G., Bresadola F., Baccarani U., Toniutto P.L., Manzia T., Anselmo A., Angelico M., Calise F., Scuderi V., Romano M., Rendina M., Barone M., Cuomo O., Perrella A., Santaniello W., Donataccio M., Dalle Ore G., de Ville de J., Monti L., Avolio, A W, Cillo, U, Salizzoni, M, De Carlis, L, Colledan, M, Gerunda, G E, Mazzaferro, Mariapia, Tisone, G, Romagnoli, R, Caccamo, L, Rossi, M, Vitale, A, Cucchetti, A, Lupo, L, Gruttadauria, S, Nicolotti, N, Burra, P, Gasbarrini, A, Agnes, S, Montalti, Roberto, Avolio, A, DE CARLIS, L, Gerunda, G, Mazzaferro, V, Lirosi, M, Miele, L, Pompili, M, Siciliano, M, Perilli, V, Gaspari, R, Castagneto, M, Lupo, F, Tandoi, F, Mangoni, I, Belli, L, Pinna, A, Cescon, M, Gridelli, B, Li Petri, S, Volpes, R, Pinelli, D, Fagiuoli, S, Montalti, R, Regalia, E, Rossi, G, Antonelli, B, Berloco, P, Lai, Q, Risaliti, A, Nicolini, D, Valente, U, Gelli, M, Morelli, N, Zamboni, F, Tondolo, V, Ettorre, G, Vennarecci, G, Bresadola, F, Baccarani, U, Toniutto, P, Manzia, T, Anselmo, A, Angelico, M, Calise, F, Scuderi, V, Romano, M, Rendina, M, Barone, M, Cuomo, O, Perrella, A, Santaniello, W, Donataccio, M, Dalle Ore, G, De Ville De Goyet, J, Monti, L, and De Waure, C

Subjects
Graft Rejection, Male, medicine.medical_treatment, Settore MED/18 - CHIRURGIA GENERALE, Hepacivirus, Liver transplantation, Gastroenterology, Liver disease, Postoperative Complications, Retrospective Studie, UNSUSTAINABLE MATCH, Models, Risk Factors, Immunology and Allergy, Health Status Indicators, Age Factor, PROGNOSTIC INDICES, Pharmacology (medical), Health Status Indicator, D-MELD, Liver Transplantation, liver transplantation, Hazard ratio, Graft Survival, donor-recipient match, outcome, prognosis, risk factors, Age Factors, Trapianto epatico, Hepatitis C, Hepatitis B, Statistical, Middle Aged, Prognosis, Tissue Donors, Survival Rate, Italy, HCV, Female, Human, Adult, medicine.medical_specialty, Prognosi, Donor Selection, End Stage Liver Disease, Young Adult, Internal medicine, medicine, Humans, fattori di rischio, Aged, Retrospective Studies, Transplantation, Hepaciviru, Models, Statistical, business.industry, Donor selection, Risk Factor, Settore MED/09 - MEDICINA INTERNA, Odds ratio, medicine.disease, Settore MED/18, Surgery, body regions, Postoperative Complication, business
Abstract

Donor-recipient match is a matter of debate in liver transplantation. D-MELD (donor age × recipient biochemical model for end-stage liver disease [MELD]) and other factors were analyzed on a national Italian database recording 5946 liver transplants. Primary endpoint was to determine factors predictive of 3-year patient survival. D-MELD cutoff predictive of 5-year patient survival 1628). At 3 years, the odds ratio (OR) for death was 2.03 (95% confidence interval [CI], 1.44-2.85) in D-MELD class C versus B. The OR was 0.40 (95% CI, 0.24-0.66) in class A versus class B. Other predictors were hepatitis C virus (HCV; OR = 1.42; 95% CI, 1.11-1.81), hepatitis B virus (HBV; OR = 0.69; 95% CI, 0.51-0.93), retransplant (OR = 1.82; 95% CI, 1.16-2.87) and low-volume center (OR = 1.48; 95% CI, 1.11-1.99). Cox regressions up to 90 months confirmed results. The hazard ratio was 1.97 (95% CI, 1.59-2.43) for D-MELD class C versus class B and 0.42 (95% CI, 0.29-0.60) for D-MELD class A versus class B. Recipient age, HCV, HBV and retransplant were also significant. The 5yrsPS

Published
2011

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