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1. Vaccination Recommendations in Solid Organ Transplant Adult Candidates and Recipients

Author

Vigano, M, Beretta, M, Lepore, M, Abete, R, Benatti, S, Grassini, M, Camagni, S, Chiodini, G, Vargiu, S, Vittori, C, Iachini, M, Terzi, A, Neri, F, Pinelli, D, Casotti, V, Di Marco, F, Ruggenenti, P, Rizzi, M, Colledan, M, Fagiuoli, S, Vigano M., Beretta M., Lepore M., Abete R., Benatti S. V., Grassini M. V., Camagni S., Chiodini G., Vargiu S., Vittori C., Iachini M., Terzi A., Neri F., Pinelli D., Casotti V., Di Marco F., Ruggenenti P., Rizzi M., Colledan M., Fagiuoli S., Vigano, M, Beretta, M, Lepore, M, Abete, R, Benatti, S, Grassini, M, Camagni, S, Chiodini, G, Vargiu, S, Vittori, C, Iachini, M, Terzi, A, Neri, F, Pinelli, D, Casotti, V, Di Marco, F, Ruggenenti, P, Rizzi, M, Colledan, M, Fagiuoli, S, Vigano M., Beretta M., Lepore M., Abete R., Benatti S. V., Grassini M. V., Camagni S., Chiodini G., Vargiu S., Vittori C., Iachini M., Terzi A., Neri F., Pinelli D., Casotti V., Di Marco F., Ruggenenti P., Rizzi M., Colledan M., and Fagiuoli S.

Abstract

Prevention of infections is crucial in solid organ transplant (SOT) candidates and recipients. These patients are exposed to an increased infectious risk due to previous organ insufficiency and to pharmacologic immunosuppression. Besides infectious-related morbidity and mortality, this vulnerable group of patients is also exposed to the risk of acute decompensation and organ rejection or failure in the pre- and post-transplant period, respectively, since antimicrobial treatments are less effective than in the immunocompetent patients. Vaccination represents a major preventive measure against specific infectious risks in this population but as responses to vaccines are reduced, especially in the early post-transplant period or after treatment for rejection, an optimal vaccination status should be obtained prior to transplantation whenever possible. This review reports the currently available data on the indications and protocols of vaccination in SOT adult candidates and recipients.

Published
2023

2. Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia

Author

Betalli, P, Cheli, M, Colusso, M, Casotti, V, Alberti, D, Ferrari, A, Starita, G, Lucianetti, A, Pinelli, D, Colledan, M, D'Antiga, L, Betalli P., Cheli M., Colusso M. M., Casotti V., Alberti D., Ferrari A., Starita G., Lucianetti A., Pinelli D., Colledan M., D'Antiga L., Betalli, P, Cheli, M, Colusso, M, Casotti, V, Alberti, D, Ferrari, A, Starita, G, Lucianetti, A, Pinelli, D, Colledan, M, D'Antiga, L, Betalli P., Cheli M., Colusso M. M., Casotti V., Alberti D., Ferrari A., Starita G., Lucianetti A., Pinelli D., Colledan M., and D'Antiga L.

Abstract

Background: Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT). Methods: Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (<5 KPE/year). We analyzed total transplant time (TTT), hepatectomy time, amount of plasma and red blood cell (RBC) transfusions, occurrence of bowel perforations at LT. Results: Among 115 patients, Group A (n 44) and Group B (n 71) were comparable for age, sex, PELD score, TTT. The groups differed for: median hepatectomy time (57 min, IQR = 50–67; vs 65, IQR 55–89, p = 0.045); RBC transfusions (95 ml, IQR 0–250; vs 200 ml, IQR 70–500, p = 0.017); bowel perforations (0/44 vs 15/71, p = 0.001). One-year graft loss in Group A vs Group B was 1/44 vs 7/71 (p = 0.239), whereas deaths were 0/44 vs 5/71 respectively (p = 0.183); 5/15 patients who had a perforation eventually lost the graft. Conclusions: This study found an association between KPE performed in low caseload center and the incidence of complications at LT. These patients tend to have a worse outcome. The centralization of KPE to referral center represents an advantage at the time of LT. Mini abstract: We studied the impact of Kasai portoenterostomy (KPE) caseload on complications at the time of liver transplantation (LT), in 115 patients. We found an association between KPE performed in low caseload center and increased bowel perforations and blood transfusions. We suggest to centralize to experienced center all children requiring KPE.

Published
2022

3. The Conundrum of Cognitive Dysfunction in Children With Portal Hypertension: The Experience of Bergamo, Italy

Author

D'Antiga, L, Casotti, V, Caffi, L, Quadri, S, Colusso, M, Biffi, A, Previtali, P, Spada, M, Amodio, P, D'Antiga L., Casotti V., Caffi L. G., Quadri S., Colusso M., Biffi A., Previtali P., Spada M. S., Amodio P., D'Antiga, L, Casotti, V, Caffi, L, Quadri, S, Colusso, M, Biffi, A, Previtali, P, Spada, M, Amodio, P, D'Antiga L., Casotti V., Caffi L. G., Quadri S., Colusso M., Biffi A., Previtali P., Spada M. S., and Amodio P.

Published
2022

6. Under-vaccination in pediatric liver transplant candidates with acute and chronic liver disease-a retrospective observational study of the European reference network transplantchild

Author

Laue, T, Demir, Z, Debray, D, Cananzi, M, Gaio, P, Casotti, V, D'Antiga, L, Urbonas, V, Baumann, U, Laue T., Demir Z., Debray D., Cananzi M., Gaio P., Casotti V., D'Antiga L., Urbonas V., Baumann U., Laue, T, Demir, Z, Debray, D, Cananzi, M, Gaio, P, Casotti, V, D'Antiga, L, Urbonas, V, Baumann, U, Laue T., Demir Z., Debray D., Cananzi M., Gaio P., Casotti V., D'Antiga L., Urbonas V., and Baumann U.

Abstract

Infection is a serious concern in the short and long term after pediatric liver transplantation. Vaccination represents an easy and cheap opportunity to reduce morbidity and mortality due to vaccine-preventable infection. This retrospective, observational, multi-center study examines the immunization status in pediatric liver transplant candidates at the time of transplantation and compares it to a control group of children with acute liver disease. Findings show only 80% were vaccinated age-appropriately, defined as having received the recommended number of vaccination doses for their age prior to transplantation; for DTP-PV-Hib, less than 75% for Hepatitis B and two-thirds for pneumococcal conjugate vaccine in children with chronic liver disease. Vaccination coverage for live vaccines is better compared to the acute control group with 81% versus 62% for measles, mumps and rubella (p = 0.003) and 65% versus 55% for varicella (p = 0.171). Nevertheless, a country-specific comparison with national reference data suggests a lower vaccination coverage in children with chronic liver disease. Our study reveals an under-vaccination in this high-risk group prior to transplantation and underlines the need to improve vaccination.

Published
2021

7. Impact of the Severe Acute Respiratory Syndrome Coronavirus 2 Outbreak on Pediatric Liver Transplant Recipients in Lombardy, Northern Italy

Author

Nicastro, E, Di Giorgio, A, Zambelli, M, Ginammi, M, Bravi, M, Stroppa, P, Casotti, V, Palladino, R, Colledan, M, D'Antiga, L, Nicastro E, Di Giorgio A, Zambelli M, Ginammi M, Bravi M, Stroppa P, Casotti V, Palladino R, Colledan M, D'Antiga L, Nicastro, E, Di Giorgio, A, Zambelli, M, Ginammi, M, Bravi, M, Stroppa, P, Casotti, V, Palladino, R, Colledan, M, D'Antiga, L, Nicastro E, Di Giorgio A, Zambelli M, Ginammi M, Bravi M, Stroppa P, Casotti V, Palladino R, Colledan M, and D'Antiga L

Published
2020

8. Health-related quality of life in pre-adolescent liver transplant recipients with biliary atresia: A cross-sectional study

Author

Miserachs, M, Parmar, A, Bakula, A, Hierro, L, D'Antiga, L, Goldschmidt, I, Debray, D, A McLin, V, Casotti, V, Pawlowska, J, Camarena, C, R Otley, A, Baumann, U, L Ng, V, Miserachs M., Parmar A., Bakula A., Hierro L., D'Antiga L., Goldschmidt I., Debray D., A McLin V., Casotti V., Pawlowska J., Camarena C., R Otley A., Baumann U., L Ng V., Miserachs, M, Parmar, A, Bakula, A, Hierro, L, D'Antiga, L, Goldschmidt, I, Debray, D, A McLin, V, Casotti, V, Pawlowska, J, Camarena, C, R Otley, A, Baumann, U, L Ng, V, Miserachs M., Parmar A., Bakula A., Hierro L., D'Antiga L., Goldschmidt I., Debray D., A McLin V., Casotti V., Pawlowska J., Camarena C., R Otley A., Baumann U., and L Ng V.

Abstract

Objective: Pediatric recipients of liver transplantation (LT) often report lower Health-Related Quality of Life (HRQOL) than healthy controls when assessed on generic HRQOL measurement tools. The recent addition of the Pediatric Liver Transplant Quality of Life (PeLTQL), a novel disease-specific HRQOL instrument for pediatric LT recipients, into the clinical armamentarium of tools now routinely available to clinical care teams, provides the unique opportunity to identify disease-related challenges in children who have undergone this life-saving intervention. This study assesses HRQOL in pre-adolescent aged patients with a primary diagnosis of biliary atresia (BA) who underwent LT as an infant, using both generic and disease-specific HRQOL instruments validated for children. We also examined modifiable factors associated with HRQOL after pediatric LT. Methods: HRQOL was the primary outcome of this study assessed using the disease-specific PeLTQL and the generic Pediatric Quality of Life Inventory 4.0 (PedsQL). Exposure variables of interest included medication status (e.g., monotherapy, dual therapy) and participation in sports. Results: A total of 70 (56% female, mean age 9.89 ± 1.25 years) pediatric LT recipients (mean interval since LT was 9.0 ± 1.26 years) comprised the study cohort. LT recipients reported significantly lower PedsQL Scores relative to the general population. Immunosuppression monotherapy was associated with higher patient-reported PeLTQL Scores, and sports participation was associated with higher parent-reported PedsQL Scores. Conclusions: Pre-adolescents who underwent LT as an infant with BA, self-report low HRQOL on both disease-specific and generic HRQOL tools. Further research targeting sports participation and simplifying immunosuppression may further optimize quality of life years restored by life-saving LT.

Published
2019

9. INTRAHEPATIC AND EXTRAHEPATIC CONGENITAL PORTOSYSTEMIC SHUNTS DIFFER IN IN CLINICAL PRESENTATION AND OUTCOMES IN CHILDREN

Author

Mclin, V, Franchi-Abella, S, Debray, D, Korff, S, Casotti, V, Colledan, M, D'Antiga, L, De Goyet, J, Lurz, E, Stephenne, X, Rock, N, Hierro, L, Kanavaki, I, Lipsich, J, Lopez-Santamaria, M, Magnusson, M, Mozer-Glassberg, Y, Ponce, M, Prieto, C, Durmaz, O, Varma, S, Guerin, F, Pop, T, Wildhaber, B, Gonzales, E, McLin VA, Franchi-Abella S, Debray D, Korff S, Casotti V, Colledan M, D'Antiga L, De Goyet JD, Lurz E, Stephenne X, Rock N, Hierro L, Kanavaki I, Lipsich J, Lopez-Santamaria M, Magnusson M, Mozer-Glassberg Y, Ponce MD, Prieto C, Durmaz O, Varma S, Guerin F, Pop T, Wildhaber B, Gonzales EM, Mclin, V, Franchi-Abella, S, Debray, D, Korff, S, Casotti, V, Colledan, M, D'Antiga, L, De Goyet, J, Lurz, E, Stephenne, X, Rock, N, Hierro, L, Kanavaki, I, Lipsich, J, Lopez-Santamaria, M, Magnusson, M, Mozer-Glassberg, Y, Ponce, M, Prieto, C, Durmaz, O, Varma, S, Guerin, F, Pop, T, Wildhaber, B, Gonzales, E, McLin VA, Franchi-Abella S, Debray D, Korff S, Casotti V, Colledan M, D'Antiga L, De Goyet JD, Lurz E, Stephenne X, Rock N, Hierro L, Kanavaki I, Lipsich J, Lopez-Santamaria M, Magnusson M, Mozer-Glassberg Y, Ponce MD, Prieto C, Durmaz O, Varma S, Guerin F, Pop T, Wildhaber B, and Gonzales EM

Published
2019

10. Liver disease in paediatric medicine: An overview

Author

D'antiga, L, Casotti, V, D'Antiga, L, Casotti V., D'antiga L., D'antiga, L, Casotti, V, D'Antiga, L, Casotti V., and D'antiga L.

Abstract

The reports of the World Health Organization (WHO) on global mortality rates by cause suggest that liver disease is an uncommon cause of death in children worldwide, being probably responsible for less than 1% of all paediatric deaths. Nonetheless, viral hepatitis and related liver cancer affect a large number of children, especially in developing countries. In these areas, childhood mortality from liver disease is mainly caused by preventable or treatable conditions, such as HAV, HBV and HCV infections. In Western countries, liver disease is the 20th cause of mortality in childhood, and the most common chronic diseases are biliary atresia and genetic cholestatic disorders. Paediatric liver disease presents most commonly in the first 2 years of life and often progresses to chronicity. Many such conditions end up with cirrhosis and its complications, leading to the need for liver transplantation in a large proportion of cases. A global vision of paediatric liver disease is necessary, since most deaths are caused by preventable or curable conditions.

Published
2019

11. Principles of liver physiology

Author

D'Antiga, L, Casotti, V, Casotti V., D'antiga L., D'Antiga, L, Casotti, V, Casotti V., and D'antiga L.

Abstract

The liver has a pivotal role in the regulation of body homeostasis and hosts the majority of metabolic processes. Among these, the most relevant are glucose, lipid, amino acid and lipoprotein metabolisms, as well as bile formation, contributing to fat digestion and detoxification of xenobiotics. The majority of plasmatic proteins (albumin, acute-phase proteins, coagulation proteins) are synthesised in the hepatocytes, which represent 60–70% of all liver cells. The other 30–40% include endothelial cells, Kupffer cells and stellate cells. The hepatocytes are rich in mitochondria, double-membrane intracellular organelles containing a soluble matrix and their own unique genome, which are the main source of energy through the formation of ATP. The mitochondrial respiratory chain represents the ultimate step of energy production for all the main metabolic pathways. Since these organelles are highly concentrated in the hepatocytes, mitochondrial disorders are generally highly expressed in the liver. The functional development of the liver requires a complicated orchestration of changes in hepatic enzymes and metabolic pathways that result in the mature capacity to undertake metabolism, biotransformation and transport. The liver at birth has an overall immature metabolic function and achieves the adult expression of the drug-metabolizing enzymes CYP450 and UGT only after 1 year of age.

Published
2019

12. Impact of the SARS-CoV-2 outbreak on pediatric liver transplant recipients residing in Lombardy, Northern Italy

Author

Nicastro, E, Di Giorgio, A, Zambelli, M, Ginammi, M, Bravi, M, Stroppa, P, Casotti, V, Palladino, R, Colledan, M, and D'Antiga, L

Subjects
Coronavirus, Transplantation, Science & Technology, Gastroenterology & Hepatology, children, SARS-CoV-2, COVID-19, Surgery, 1103 Clinical Sciences, Life Sciences & Biomedicine, Immunosuppression, Liver Transplantation
Abstract

The novel Severe Acute Respiratory Syndrome Coronavirus (SARS-CoV-2) disease (COVID-19) represents an unprecedented public health issue for the general population and for patients with underlying chronic conditions. Compared to adults, children seem to have a milder course of the disease, with very few requiring medical attention.

Published
2020

13. Effectiveness of Preemptive Therapy for Cytomegalovirus Disease in Pediatric Liver Transplantation

Author

Nicastro, E, Giovannozzi, S, Stroppa, P, Casotti, V, Callegaro, A, Tebaldi, A, Farina, C, Colledan, M, D'Antiga, L, Nicastro E, Giovannozzi S, Stroppa P, Casotti V, Callegaro AP, Tebaldi A, Farina C, Colledan M, D'Antiga L, Nicastro, E, Giovannozzi, S, Stroppa, P, Casotti, V, Callegaro, A, Tebaldi, A, Farina, C, Colledan, M, D'Antiga, L, Nicastro E, Giovannozzi S, Stroppa P, Casotti V, Callegaro AP, Tebaldi A, Farina C, Colledan M, and D'Antiga L

Abstract

Background Most pediatric liver transplantation (LT) centers administer long courses of prophylaxis against cytomegalovirus (CMV) without evidence of benefit and with significant drug exposure and costs. We aimed at evaluating overall outcomes, direct and putative indirect effects of CMV, possible impact of viremia and risk factors for CMV infection in pediatric LT recipients managed with ganciclovir-based preemptive therapy (PET). Methods The records of all the children who underwent LT between 2008 and 2014 were retrospectively analyzed. Results One hundred children were included. Three children had CMV disease; no CMV-related death or graft loss was recorded. The only identified risk factor for CMV infection was the donor/recipient serostatus (odds ratio, 17.23; 95% confidence interval, 1.88-157.87; P = 0.012), while viremia per se did not worsen LT outcomes, such as the incidence of acute rejection, Epstein-Barr virus infection, sepsis, biliary and vascular complications, nor graft dysfunction/loss or death at 3 and 5 years after LT. When compared with a historical cohort of children receiving ganciclovir prophylaxis, PET did not differ from prophylaxis for any of the selected outcomes, but was rather associated with lower antiviral drug exposure (6.4 ± 13 days vs 38.6 ± 14 days, P < 0.0001) and cost per patient (2.2 ± 3.9 k€ vs 6.6 ± 8.2 k€, P = 0.001). Conclusions PET is effective in controlling CMV in children receiving LT, with lower costs and lower exposure to antivirals.

Published
2017

14. 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

15. 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

17. P028 Establishing a SIGENP Italian network for the study of biliary atresia

Author

Ranucci, G., primary, Alberti, D., additional, Bagolan, P., additional, Betalli, P., additional, Calvo, P., additional, Cananzi, M., additional, Candusso, M., additional, Casotti, V., additional, Clemente, M.G., additional, D'Antiga, L., additional, De Ville, J., additional, Di Francesco, F., additional, Fascetti Leon, F., additional, Ferrari, F., additional, Fusaro, F., additional, Gaio, P., additional, Gamba, P., additional, Indolfi, G., additional, Iorio, R., additional, La Pergola, E., additional, Liccardo, D., additional, Lima, M., additional, Maggiore, G., additional, Mandato, C., additional, Matarazzo, L., additional, Nebbia, G., additional, Nobili, V., additional, Nuti, F., additional, Paolella, G., additional, Parolini, F., additional, Romagnoli, R., additional, Sciveres, M., additional, Serranti, D., additional, Spada, M., additional, Tandoi, F., additional, and Vajro, P., additional

Published
2018
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18. Intestinal Transplantation in Children: The First Successful Italian Series

Author

Colledan, M, Stroppa, P, Bravi, M, Casotti, V, Lucianetti, A, Pinelli, D, Zambelli, M, Guizzetti, M, Corna, V, Aluffi, A, Sonzogni, V, Sonzogni, A, D'Antiga, L, Codazzi, D, Colledan M, Stroppa P, Bravi M, Casotti V, Lucianetti A, Pinelli D, Zambelli M, Guizzetti M, Corna V, Aluffi A, Sonzogni V, Sonzogni A, D'Antiga L, Codazzi D, Colledan, M, Stroppa, P, Bravi, M, Casotti, V, Lucianetti, A, Pinelli, D, Zambelli, M, Guizzetti, M, Corna, V, Aluffi, A, Sonzogni, V, Sonzogni, A, D'Antiga, L, Codazzi, D, Colledan M, Stroppa P, Bravi M, Casotti V, Lucianetti A, Pinelli D, Zambelli M, Guizzetti M, Corna V, Aluffi A, Sonzogni V, Sonzogni A, D'Antiga L, and Codazzi D

Abstract

The preliminary experience of the first Italian program of pediatric intestinal transplantation is presented herein. A multidisciplinary group with broad experience in pediatric solid organ transplantation started the program. Nine children with complications of chronic intestinal failure were listed for transplantation. One child died on the waiting list; one received an isolated liver transplantation; three isolated intestinal; three multivisceral; and one, a combined liver/intestine transplantation. There was no in-hospital mortality, and all children were weaned from parenteral nutrition. The recipient of the multivisceral graft died after 14 months for unknown causes. All other recipients are alive after a median follow-up of 13 months. Patient and graft actuarial survivals for recipients of intestinal grafts were 100% at 1 year and 75% at 2 years.

Published
2010

19. Good outcome of hepatocellular carcinoma in chronic liver disease of childhood

Author

Romano, F, Stroppa, P, Bravi, M, Casotti, V, Sonzogni, A, Alberti, D, Colledan, M, D'Antiga, L, Romano F, Stroppa P, Bravi M, Casotti V, Sonzogni A, Alberti D, Colledan M, D'Antiga L, Romano, F, Stroppa, P, Bravi, M, Casotti, V, Sonzogni, A, Alberti, D, Colledan, M, D'Antiga, L, Romano F, Stroppa P, Bravi M, Casotti V, Sonzogni A, Alberti D, Colledan M, and D'Antiga L

Published
2010

20. Management of hepatopulmonary syndrome in children. A single centre report

Author

Bosisio, M, Stroppa, P, Bravi, M, Casotti, V, Codazzi, D, Colledan, M, D'Antiga, L, Bosisio M, Stroppa P, Bravi M, Casotti V, Codazzi D, Colledan M, D'Antiga L, Bosisio, M, Stroppa, P, Bravi, M, Casotti, V, Codazzi, D, Colledan, M, D'Antiga, L, Bosisio M, Stroppa P, Bravi M, Casotti V, Codazzi D, Colledan M, and D'Antiga L

Published
2010

23. Encefalopatia epatica

Author

Salvatore Cucchiara, Giuseppe Maggiore, Annamaria Staiano, Carlo Catassi, Casotti, V, D'Antiga, L, Salvatore Cucchiara, Giuseppe Maggiore, Annamaria Staiano, Carlo Catassi, Casotti, V, and D'Antiga, L

Published
2014

24. Favorable outcome of primary liver transplantation in children with cirrhosis and hepatocellular carcinoma

Author

Romano, F, Stroppa, P, Bravi, M, Casotti, V, Lucianetti, A, Guizzetti, M, Sonzogni, A, Colledan, M, D'Antiga, L, ROMANO, FABRIZIO, D'Antiga, L., Romano, F, Stroppa, P, Bravi, M, Casotti, V, Lucianetti, A, Guizzetti, M, Sonzogni, A, Colledan, M, D'Antiga, L, ROMANO, FABRIZIO, and D'Antiga, L.

Abstract

The outcome of HCC after transplantation (OLT) in children is not well known. Unfavorable features based on adult reports may lead to contraindicate OLT even in children. We reviewed a cohort of children with cirrhosis and HCC to evaluate their outcome after primary transplantation. We considered children with cirrhosis and HCC who had a primary OLT. We retrospectively recorded demographic, medical and surgical features, and MC as predictors of outcome. Among 456 children transplanted in the last 15 yr, 10 (2%), median age at diagnosis 1.8 yr (range 0.5-7.2), had HCC in biliary atresia (3), BSEP deficiency (3), tyrosinemia type 1 (2), complications of choledocal cyst and glycogen storage disease type IV (1 each). At HCC discovery, median AFP was 2322 ng/mL (3-35 000), high or rising in 9/10 patients. Six patients were outside the MC. Median time on the waiting list was 38 days (1-152). Two patients died from early complications of OLT. In the other eight patients, there was no tumor recurrence after a median follow-up of four yr. Children with cirrhosis may develop HCC at a very young age. The outcome appears excellent even outside MC. Primary liver transplantation is advisable for children with cirrhosis, HCC, and no extrahepatic disease.

Published
2011

25. Structural and functional lung disease in primary ciliary dyskinesia

Author

Santamaria, F. (Francesca), Montella, S. (Silvia), Tiddens, H.A.W.M. (Harm), Guidi, G. (Guido), Casotti, V. (Valeria), Maglione, M. (Marco), Jong, P.A. (Pim) de, Santamaria, F. (Francesca), Montella, S. (Silvia), Tiddens, H.A.W.M. (Harm), Guidi, G. (Guido), Casotti, V. (Valeria), Maglione, M. (Marco), and Jong, P.A. (Pim) de

Abstract

Background: High-resolution CT (HRCT) scan data on primary ciliary dyskinesia (PCD) related lung disease are scarce. Study objectives: We evaluated the lung disease in children and adults with PCD by a modified Brody composite HRCT scan score to assess the prevalence of the structural abnormalities; to evaluate the correlation among HRCT scan scores, spirometry findings, and clinical data; and to compare the PCD scores with those of age-matched and sex-matched cystic fibrosis (CF) patients. Patients and methods: Twenty PCD patients (age range, 4.6 to 27.5 years) underwent HRCT scanning, spirometry, and deep throat or sputum culture. A modified Brody score was used to assess bronchiectasis, mucous plugging, peribronchial thickening, parenchyma abnormalities, and mosaic perfusion. Results: The total HRCT scan score was 6% of the maximal score (range, 0.5 to 25.5). Subscores were as follows: bronchiectasis, 5.6%; mucous plugging, 5.6%; peribronchial thickening, 8.3%; parenchyma, 3%; and mosaic perfusion, 0%. The prevalence of lung changes were as follows: bronchiectasis, 80%; peribronchial thickening, 80%; mucous plugging, 75%; parenchyma, 65%; and mosaic perfusion, 45%. Sixteen of 19 PCD patients had positive culture findings, and the most common pathogen found was Haemophilus influenzae (84%). The total HRCT scan score was significantly related to age (p = 0.006), FEV1(p = 0.02), and FVC (p = 0.02). The bronchiectasis subscore was significantly related to FEV1(p = 0.04) and FVC (p = 0.03). In CF patients, the total HRCT scan score was significantly higher than that in PCD patients (p = 0.02). Conclusions: PCD patients show significantly lower pulmonary HRCT scan scores than CF patients. The PCD total and bronchiectasis scores correlate with spirometry findings. The PCD HRCT scan score might be used for longitudinal assessment and/or represent an outcome surrogate in future studies. Copyright

Published
2008
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32. Antimitochondrial antibodies: Two paediatric case report

Author

Torre, G., primary, Casotti, V., additional, Candusso, M., additional, Melzi, M.L., additional, Stroppa, P., additional, Bravi, M., additional, Cavalleri, L., additional, Bosisio, M., additional, Alessio, M.G., additional, Sonzogni, A., additional, and Invernizzi, P., additional

Published
2007
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34. Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia

Author

Pietro Betalli, Maurizio Cheli, Mara Marcella Colusso, Valeria Casotti, Daniele Alberti, Alberto Ferrari, Giusy Starita, Alessandro Lucianetti, Domenico Pinelli, Michele Colledan, Lorenzo D'Antiga, Betalli, P, Cheli, M, Colusso, M, Casotti, V, Alberti, D, Ferrari, A, Starita, G, Lucianetti, A, Pinelli, D, Colledan, M, and D'Antiga, L

Subjects
Liver transplantation, Survival, Infant, Portoenterostomy, Hepatic, General Medicine, Biliary atresia, Children, Intestinal perforation, Kasai portoenterostomy, Treatment Outcome, Pediatrics, Perinatology and Child Health, Humans, Surgery, Child, Retrospective Studies
Abstract

Background: Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT). Methods: Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (

Published
2022

35. Effectiveness of Preemptive Therapy for Cytomegalovirus Disease in Pediatric Liver Transplantation

Author

Anna Paola Callegaro, Claudio Farina, Emanuele Nicastro, Paola Stroppa, Michele Colledan, Sara Giovannozzi, Valeria Casotti, Alessandra Tebaldi, Lorenzo DʼAntiga, Nicastro, E, Giovannozzi, S, Stroppa, P, Casotti, V, Callegaro, A, Tebaldi, A, Farina, C, Colledan, M, and D'Antiga, L

Subjects
Cytomegalovirus Infection, Male, medicine.medical_treatment, Cytomegalovirus, Kaplan-Meier Estimate, 030230 surgery, Liver transplantation, 0302 clinical medicine, Retrospective Studie, Odds Ratio, Medicine, Age Factor, Child, media_common, Drug Cost, virus diseases, Treatment Outcome, Italy, Child, Preschool, Cytomegalovirus Infections, Female, 030211 gastroenterology & hepatology, Human, medicine.drug, Ganciclovir, Drug, medicine.medical_specialty, Adolescent, Time Factor, media_common.quotation_subject, Congenital cytomegalovirus infection, MEDLINE, Antiviral Agents, Drug Administration Schedule, 03 medical and health sciences, Internal medicine, Humans, Cost-Benefit Analysi, Antiviral Agent, Transplantation, Chi-Square Distribution, Cost Saving, business.industry, Risk Factor, Infant, Retrospective cohort study, Odds ratio, Original Clinical Science—Liver, medicine.disease, Transplant Recipients, Liver Transplantation, business, Chi-squared distribution
Abstract

BACKGROUND: Most pediatric liver transplantation (LT) centers administer long courses of prophylaxis against cytomegalovirus (CMV) without evidence of benefit and with significant drug exposure and costs. We aimed at evaluating overall outcomes, direct and putative indirect effects of CMV, possible impact of viremia and risk factors for CMV infection in pediatric LT recipients managed with ganciclovir-based preemptive therapy (PET). METHODS: The records of all the children who underwent LT between 2008 and 2014 were retrospectively analyzed. RESULTS: One hundred children were included. Three children had CMV disease; no CMV-related death or graft loss was recorded. The only identified risk factor for CMV infection was the donor/recipient serostatus (odds ratio, 17.23; 95% confidence interval, 1.88-157.87; P = 0.012), while viremia per se did not worsen LT outcomes, such as the incidence of acute rejection, Epstein-Barr virus infection, sepsis, biliary and vascular complications, nor graft dysfunction/loss or death at 3 and 5 years after LT. When compared with a historical cohort of children receiving ganciclovir prophylaxis, PET did not differ from prophylaxis for any of the selected outcomes, but was rather associated with lower antiviral drug exposure (6.4 ± 13 days vs 38.6 ± 14 days, P < 0.0001) and cost per patient (2.2 ± 3.9 k€ vs 6.6 ± 8.2 k€, P = 0.001). CONCLUSIONS: PET is effective in controlling CMV in children receiving LT, with lower costs and lower exposure to antivirals.

Published
2017

36. Favorable outcome of primary liver transplantation in children with cirrhosis and hepatocellular carcinoma

Author

Fabrizio, Romano, Paola, Stroppa, Michela, Bravi, Valeria, Casotti, Alessandro, Lucianetti, Michela, Guizzetti, Aurelio, Sonzogni, Michele, Colledan, Lorenzo, D'Antiga, Romano, F, Stroppa, P, Bravi, M, Casotti, V, Lucianetti, A, Guizzetti, M, Sonzogni, A, Colledan, M, and D'Antiga, L

Subjects
Liver Cirrhosis, Male, Carcinoma, Hepatocellular, Liver Neoplasms, Infant, Liver Transplantation, Cohort Studies, Treatment Outcome, Child, Preschool, MED/18 - CHIRURGIA GENERALE, Humans, Female, Child, Immunosuppressive Agents, Retrospective Studies, Liver transplantation, HCC, Children
Abstract

The outcome of HCC after transplantation (OLT) in children is not well known. Unfavorable features based on adult reports may lead to contraindicate OLT even in children. We reviewed a cohort of children with cirrhosis and HCC to evaluate their outcome after primary transplantation. We considered children with cirrhosis and HCC who had a primary OLT. We retrospectively recorded demographic, medical and surgical features, and MC as predictors of outcome. Among 456 children transplanted in the last 15 yr, 10 (2%), median age at diagnosis 1.8 yr (range 0.5-7.2), had HCC in biliary atresia (3), BSEP deficiency (3), tyrosinemia type 1 (2), complications of choledocal cyst and glycogen storage disease type IV (1 each). At HCC discovery, median AFP was 2322 ng/mL (3-35,000), high or rising in 9/10 patients. Six patients were outside the MC. Median time on the waiting list was 38 days (1-152). Two patients died from early complications of OLT. In the other eight patients, there was no tumor recurrence after a median follow-up of four yr. Children with cirrhosis may develop HCC at a very young age. The outcome appears excellent even outside MC. Primary liver transplantation is advisable for children with cirrhosis, HCC, and no extrahepatic disease.

Published
2011

37. Intestinal transplantation in children: the first successful Italian series

Author

V. Sonzogni, Domenico Pinelli, M. Zambelli, A. Aluffi, Lorenzo D'Antiga, M Guizzetti, Alessandro Lucianetti, Aurelio Sonzogni, Paola Stroppa, D. Codazzi, Valeria Casotti, Michele Colledan, V. Corno, M. Bravi, Colledan, M, Stroppa, P, Bravi, M, Casotti, V, Lucianetti, A, Pinelli, D, Zambelli, M, Guizzetti, M, Corna, V, Aluffi, A, Sonzogni, V, Sonzogni, A, D'Antiga, L, and Codazzi, D

Subjects
Intestinal pseudo-obstruction, Cytomegalovirus Infection, Short Bowel Syndrome, medicine.medical_specialty, medicine.medical_treatment, Intestinal Atresia, Liver transplantation, Medicine, Humans, Survivors, Child, Transplantation, Intestine transplantation, business.industry, Intestinal atresia, Graft Survival, Intestinal Pseudo-Obstruction, Infant, Intestinal Volvulu, medicine.disease, Short bowel syndrome, Chronic intestinal failure, Surgery, Intestine, Liver Transplantation, Intestines, Survival Rate, Viscera, surgical procedures, operative, Parenteral nutrition, Italy, Child, Preschool, Cytomegalovirus Infections, Survivor, business, Human, Intestinal Volvulus
Abstract

The preliminary experience of the first Italian program of pediatric intestinal transplantation is presented herein. A multidisciplinary group with broad experience in pediatric solid organ transplantation started the program. Nine children with complications of chronic intestinal failure were listed for transplantation. One child died on the waiting list; one received an isolated liver transplantation; three isolated intestinal; three multivisceral; and one, a combined liver/intestine transplantation. There was no in-hospital mortality, and all children were weaned from parenteral nutrition. The recipient of the multivisceral graft died after 14 months for unknown causes. All other recipients are alive after a median follow-up of 13 months. Patient and graft actuarial survivals for recipients of intestinal grafts were 100% at 1 year and 75% at 2 years.

Published
2010

38. Vaccinations in Paediatric Solid Organ Transplant Candidates and Recipients.

Author

Casotti V, Stroppa P, Bravi M, Tebaldi A, Loglio A, Viganò M, Fagiuoli S, and D'Antiga L

Abstract

Solid organ transplant (SOT) candidates and recipients are a fragile population, in which the presence of a pre-transplant disease leading to organ insufficiency and the post-transplant immunosuppressive treatment expose them to an increased risk of infectious diseases. The best intervention to guarantee efficient prevention of infections, with optimal cost-benefit ratio, is represented by vaccination programs; however, the response to vaccines needs that the immune system maintains a good function. This is even more relevant at paediatric age, when specific immunological conditions make transplant candidates and recipients particularly vulnerable. Paediatric patients may be naïve to most infections and may have incomplete immunization status at the time of transplant listing due to their age. Moreover, the unaccomplished development of a mature immune system and the immunosuppressive regimen adopted after transplant might affect the efficacy of post-transplant vaccinations. Therefore, every effort should be made to obtain the widest vaccination coverage before the transplantation, whenever possible. This review reports the most relevant literature, providing information on the current approach to the vaccinations in paediatric SOT candidates and recipients.

Published
2024
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39. Corrigendum to "Expert management of congenital portosystemic shunts and their complications" [JHEP Reports 6 (2024)].

Author

McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier DA, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling-Soonawala N, Debray D, and Wildhaber BE

Abstract

[This corrects the article DOI: 10.1016/j.jhepr.2023.100933.]., (© 2024 The Author(s).)

Published
2024
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40. Expert management of congenital portosystemic shunts and their complications.

Author

McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier A, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, van der Doef HPJ, Vilgrain V, Wacker J, Zwaveling N, Debray D, and Wildhaber BE

Abstract

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up., Competing Interests: The authors declare no conflicts of interest that pertain to this work. Please refer to the accompanying ICMJE disclosure forms for further details., (© 2023 The Author(s).)

Published
2023
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41. Vaccination Recommendations in Solid Organ Transplant Adult Candidates and Recipients.

Author

Viganò M, Beretta M, Lepore M, Abete R, Benatti SV, Grassini MV, Camagni S, Chiodini G, Vargiu S, Vittori C, Iachini M, Terzi A, Neri F, Pinelli D, Casotti V, Di Marco F, Ruggenenti P, Rizzi M, Colledan M, and Fagiuoli S

Abstract

Prevention of infections is crucial in solid organ transplant (SOT) candidates and recipients. These patients are exposed to an increased infectious risk due to previous organ insufficiency and to pharmacologic immunosuppression. Besides infectious-related morbidity and mortality, this vulnerable group of patients is also exposed to the risk of acute decompensation and organ rejection or failure in the pre- and post-transplant period, respectively, since antimicrobial treatments are less effective than in the immunocompetent patients. Vaccination represents a major preventive measure against specific infectious risks in this population but as responses to vaccines are reduced, especially in the early post-transplant period or after treatment for rejection, an optimal vaccination status should be obtained prior to transplantation whenever possible. This review reports the currently available data on the indications and protocols of vaccination in SOT adult candidates and recipients.

Published
2023
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42. Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia.

Author

Betalli P, Cheli M, Colusso MM, Casotti V, Alberti D, Ferrari A, Starita G, Lucianetti A, Pinelli D, Colledan M, and D'Antiga L

Subjects
Child, Humans, Infant, Portoenterostomy, Hepatic adverse effects, Retrospective Studies, Treatment Outcome, Biliary Atresia, Intestinal Perforation epidemiology, Intestinal Perforation etiology, Liver Transplantation adverse effects
Abstract

Background: Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT)., Methods: Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (<5 KPE/year). We analyzed total transplant time (TTT), hepatectomy time, amount of plasma and red blood cell (RBC) transfusions, occurrence of bowel perforations at LT., Results: Among 115 patients, Group A (n 44) and Group B (n 71) were comparable for age, sex, PELD score, TTT. The groups differed for: median hepatectomy time (57 min, IQR = 50-67; vs 65, IQR 55-89, p = 0.045); RBC transfusions (95 ml, IQR 0-250; vs 200 ml, IQR 70-500, p = 0.017); bowel perforations (0/44 vs 15/71, p = 0.001). One-year graft loss in Group A vs Group B was 1/44 vs 7/71 (p = 0.239), whereas deaths were 0/44 vs 5/71 respectively (p = 0.183); 5/15 patients who had a perforation eventually lost the graft., Conclusions: This study found an association between KPE performed in low caseload center and the incidence of complications at LT. These patients tend to have a worse outcome. The centralization of KPE to referral center represents an advantage at the time of LT., Mini Abstract: We studied the impact of Kasai portoenterostomy (KPE) caseload on complications at the time of liver transplantation (LT), in 115 patients. We found an association between KPE performed in low caseload center and increased bowel perforations and blood transfusions. We suggest to centralize to experienced center all children requiring KPE., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Published
2022
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44. Under-Vaccination in Pediatric Liver Transplant Candidates with Acute and Chronic Liver Disease-A Retrospective Observational Study of the European Reference Network TransplantChild .

Author

Laue T, Demir Z, Debray D, Cananzi M, Gaio P, Casotti V, D'Antiga L, Urbonas V, and Baumann U

Abstract

Infection is a serious concern in the short and long term after pediatric liver transplantation. Vaccination represents an easy and cheap opportunity to reduce morbidity and mortality due to vaccine-preventable infection. This retrospective, observational, multi-center study examines the immunization status in pediatric liver transplant candidates at the time of transplantation and compares it to a control group of children with acute liver disease. Findings show only 80% were vaccinated age-appropriately, defined as having received the recommended number of vaccination doses for their age prior to transplantation; for DTP-PV-Hib, less than 75% for Hepatitis B and two-thirds for pneumococcal conjugate vaccine in children with chronic liver disease. Vaccination coverage for live vaccines is better compared to the acute control group with 81% versus 62% for measles, mumps and rubella ( p = 0.003) and 65% versus 55% for varicella ( p = 0.171). Nevertheless, a country-specific comparison with national reference data suggests a lower vaccination coverage in children with chronic liver disease. Our study reveals an under-vaccination in this high-risk group prior to transplantation and underlines the need to improve vaccination.

Published
2021
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45. Impact of the Severe Acute Respiratory Syndrome Coronavirus 2 Outbreak on Pediatric Liver Transplant Recipients in Lombardy, Northern Italy.

Author

Nicastro E, Di Giorgio A, Zambelli M, Ginammi M, Bravi M, Stroppa P, Casotti V, Palladino R, Colledan M, and D'Antiga L

Subjects
Adolescent, Adult, COVID-19, Child, Child, Preschool, Comorbidity, Female, Humans, Infant, Infant, Newborn, Italy epidemiology, Liver Diseases epidemiology, Male, Pandemics, Prognosis, SARS-CoV-2, Young Adult, Betacoronavirus, Coronavirus Infections epidemiology, Liver Diseases surgery, Liver Transplantation, Pneumonia, Viral epidemiology, Transplant Recipients
Published
2020
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46. Health-related quality of life in pre-adolescent liver transplant recipients with biliary atresia: A cross-sectional study.

Author

Miserachs M, Parmar A, Bakula A, Hierro L, D'Antiga L, Goldschmidt I, Debray D, A McLin V, Casotti V, Pawłowska J, Camarena C, R Otley A, Baumann U, and L Ng V

Subjects
Canada, Child, Cross-Sectional Studies, Europe, Female, Humans, Infant, Liver, Male, Patient Reported Outcome Measures, Sports psychology, Survivorship, Biliary Atresia surgery, Health Surveys, Immunosuppression Therapy psychology, Liver Transplantation psychology, Quality of Life legislation & jurisprudence, Transplant Recipients psychology
Abstract

Objective: Pediatric recipients of liver transplantation (LT) often report lower Health-Related Quality of Life (HRQOL) than healthy controls when assessed on generic HRQOL measurement tools. The recent addition of the Pediatric Liver Transplant Quality of Life (PeLTQL), a novel disease-specific HRQOL instrument for pediatric LT recipients, into the clinical armamentarium of tools now routinely available to clinical care teams, provides the unique opportunity to identify disease-related challenges in children who have undergone this life-saving intervention. This study assesses HRQOL in pre-adolescent aged patients with a primary diagnosis of biliary atresia (BA) who underwent LT as an infant, using both generic and disease-specific HRQOL instruments validated for children. We also examined modifiable factors associated with HRQOL after pediatric LT., Methods: HRQOL was the primary outcome of this study assessed using the disease-specific PeLTQL and the generic Pediatric Quality of Life Inventory 4.0 (PedsQL). Exposure variables of interest included medication status (e.g., monotherapy, dual therapy) and participation in sports., Results: A total of 70 (56% female, mean age 9.89 ± 1.25 years) pediatric LT recipients (mean interval since LT was 9.0 ± 1.26 years) comprised the study cohort. LT recipients reported significantly lower PedsQL Scores relative to the general population. Immunosuppression monotherapy was associated with higher patient-reported PeLTQL Scores, and sports participation was associated with higher parent-reported PedsQL Scores., Conclusions: Pre-adolescents who underwent LT as an infant with BA, self-report low HRQOL on both disease-specific and generic HRQOL tools. Further research targeting sports participation and simplifying immunosuppression may further optimize quality of life years restored by life-saving LT., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Published
2019
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47. Effectiveness of Preemptive Therapy for Cytomegalovirus Disease in Pediatric Liver Transplantation.

Author

Nicastro E, Giovannozzi S, Stroppa P, Casotti V, Callegaro AP, Tebaldi A, Farina C, Colledan M, and DʼAntiga L

Subjects
Adolescent, Age Factors, Antiviral Agents adverse effects, Antiviral Agents economics, Chi-Square Distribution, Child, Child, Preschool, Cost Savings, Cost-Benefit Analysis, Cytomegalovirus Infections diagnosis, Cytomegalovirus Infections economics, Cytomegalovirus Infections virology, Drug Administration Schedule, Drug Costs, Female, Ganciclovir adverse effects, Ganciclovir economics, Humans, Infant, Italy, Kaplan-Meier Estimate, Liver Transplantation economics, Male, Odds Ratio, Retrospective Studies, Risk Factors, Time Factors, Treatment Outcome, Antiviral Agents administration & dosage, Cytomegalovirus Infections prevention & control, Ganciclovir administration & dosage, Liver Transplantation adverse effects
Abstract

Background: Most pediatric liver transplantation (LT) centers administer long courses of prophylaxis against cytomegalovirus (CMV) without evidence of benefit and with significant drug exposure and costs. We aimed at evaluating overall outcomes, direct and putative indirect effects of CMV, possible impact of viremia and risk factors for CMV infection in pediatric LT recipients managed with ganciclovir-based preemptive therapy (PET)., Methods: The records of all the children who underwent LT between 2008 and 2014 were retrospectively analyzed., Results: One hundred children were included. Three children had CMV disease; no CMV-related death or graft loss was recorded. The only identified risk factor for CMV infection was the donor/recipient serostatus (odds ratio, 17.23; 95% confidence interval, 1.88-157.87; P = 0.012), while viremia per se did not worsen LT outcomes, such as the incidence of acute rejection, Epstein-Barr virus infection, sepsis, biliary and vascular complications, nor graft dysfunction/loss or death at 3 and 5 years after LT. When compared with a historical cohort of children receiving ganciclovir prophylaxis, PET did not differ from prophylaxis for any of the selected outcomes, but was rather associated with lower antiviral drug exposure (6.4 ± 13 days vs 38.6 ± 14 days, P < 0.0001) and cost per patient (2.2 ± 3.9 k&OV0556; vs 6.6 ± 8.2 k&OV0556;, P = 0.001)., Conclusions: PET is effective in controlling CMV in children receiving LT, with lower costs and lower exposure to antivirals.

Published
2017
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48. Favorable outcome of primary liver transplantation in children with cirrhosis and hepatocellular carcinoma.

Author

Romano F, Stroppa P, Bravi M, Casotti V, Lucianetti A, Guizzetti M, Sonzogni A, Colledan M, and D'Antiga L

Subjects
Child, Child, Preschool, Cohort Studies, Female, Humans, Immunosuppressive Agents therapeutic use, Infant, Liver Transplantation adverse effects, Male, Retrospective Studies, Treatment Outcome, Carcinoma, Hepatocellular therapy, Liver Cirrhosis therapy, Liver Neoplasms therapy, Liver Transplantation methods
Abstract

The outcome of HCC after transplantation (OLT) in children is not well known. Unfavorable features based on adult reports may lead to contraindicate OLT even in children. We reviewed a cohort of children with cirrhosis and HCC to evaluate their outcome after primary transplantation. We considered children with cirrhosis and HCC who had a primary OLT. We retrospectively recorded demographic, medical and surgical features, and MC as predictors of outcome. Among 456 children transplanted in the last 15 yr, 10 (2%), median age at diagnosis 1.8 yr (range 0.5-7.2), had HCC in biliary atresia (3), BSEP deficiency (3), tyrosinemia type 1 (2), complications of choledocal cyst and glycogen storage disease type IV (1 each). At HCC discovery, median AFP was 2322 ng/mL (3-35,000), high or rising in 9/10 patients. Six patients were outside the MC. Median time on the waiting list was 38 days (1-152). Two patients died from early complications of OLT. In the other eight patients, there was no tumor recurrence after a median follow-up of four yr. Children with cirrhosis may develop HCC at a very young age. The outcome appears excellent even outside MC. Primary liver transplantation is advisable for children with cirrhosis, HCC, and no extrahepatic disease., (© 2011 John Wiley & Sons A/S.)

Published
2011
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49. Airway dimensions in bronchopulmonary dysplasia: implications for airflow obstruction.

Author

Tiddens HA, Hofhuis W, Casotti V, Hop WC, Hulsmann AR, and de Jongste JC

Subjects
Airway Obstruction, Female, Humans, Infant, Newborn, Male, Pulmonary Ventilation, Bronchopulmonary Dysplasia pathology, Bronchopulmonary Dysplasia physiopathology
Abstract

The cause of lung function abnormalities in bronchopulmonary dysplasia (BPD) is incompletely understood, even in the "new era" of this disease. Altered airway wall dimensions are important in the pathogenesis of airflow obstruction in diseases such as asthma and chronic obstructive pulmonary disease. Whether airway wall dimensions contribute to lung function abnormalities in BPD is unknown. The purpose of this study was to investigate airway wall dimensions in relation to airway size in BPD. Lung tissue of patients with BPD was obtained at autopsy, and lung tissue from children who died from sudden infant death syndrome (SIDS) served as control. Airway wall dimensions and epithelial loss were measured in 75 airways from 5 BPD patients and 176 airways from 11 SIDS patients. Repeated measures analysis of variance was used to assess the relationships between airway wall dimensions and airway size for BPD and SIDS patients. Little epithelial loss was present in the BPD patients while extensive loss was observed in some of the SIDS patients. The inner wall area, outer wall area, epithelium area and smooth muscle area were all substantially larger (all P < 0.001) in BPD than in SIDS patients. It is likely that the increased thickness of the airway wall components contributes to airflow obstruction in BPD patients., ((c) 2008 Wiley-Liss, Inc.)

Published
2008
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50. Structural and functional lung disease in primary ciliary dyskinesia.

Author

Santamaria F, Montella S, Tiddens HAWM, Guidi G, Casotti V, Maglione M, and de Jong PA

Subjects
Adolescent, Adult, Case-Control Studies, Child, Child, Preschool, Cohort Studies, Female, Forced Expiratory Volume, Humans, Kartagener Syndrome pathology, Lung pathology, Male, Severity of Illness Index, Spirometry, Tomography, X-Ray Computed, Kartagener Syndrome diagnostic imaging, Kartagener Syndrome physiopathology, Lung diagnostic imaging, Lung physiopathology
Abstract

Background: High-resolution CT (HRCT) scan data on primary ciliary dyskinesia (PCD) related lung disease are scarce., Study Objectives: We evaluated the lung disease in children and adults with PCD by a modified Brody composite HRCT scan score to assess the prevalence of the structural abnormalities; to evaluate the correlation among HRCT scan scores, spirometry findings, and clinical data; and to compare the PCD scores with those of age-matched and sex-matched cystic fibrosis (CF) patients., Patients and Methods: Twenty PCD patients (age range, 4.6 to 27.5 years) underwent HRCT scanning, spirometry, and deep throat or sputum culture. A modified Brody score was used to assess bronchiectasis, mucous plugging, peribronchial thickening, parenchyma abnormalities, and mosaic perfusion., Results: The total HRCT scan score was 6% of the maximal score (range, 0.5 to 25.5). Subscores were as follows: bronchiectasis, 5.6%; mucous plugging, 5.6%; peribronchial thickening, 8.3%; parenchyma, 3%; and mosaic perfusion, 0%. The prevalence of lung changes were as follows: bronchiectasis, 80%; peribronchial thickening, 80%; mucous plugging, 75%; parenchyma, 65%; and mosaic perfusion, 45%. Sixteen of 19 PCD patients had positive culture findings, and the most common pathogen found was Haemophilus influenzae (84%). The total HRCT scan score was significantly related to age (p = 0.006), FEV(1) (p = 0.02), and FVC (p = 0.02). The bronchiectasis subscore was significantly related to FEV(1) (p = 0.04) and FVC (p = 0.03). In CF patients, the total HRCT scan score was significantly higher than that in PCD patients (p = 0.02)., Conclusions: PCD patients show significantly lower pulmonary HRCT scan scores than CF patients. The PCD total and bronchiectasis scores correlate with spirometry findings. The PCD HRCT scan score might be used for longitudinal assessment and/or represent an outcome surrogate in future studies.

Published
2008
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