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Zhu J, Chen H, Meng L, Jiang J, Yang Y, Wang J, Ren X, Kong F, Dong R, Chen G, Zheng S. Serum FGF19 combined with GGT and other biomarkers predicted native liver survival following Kasai portoenterostomy in early biliary atresia. J Gastroenterol 2025; 60:783-793. [PMID: 40323393 DOI: 10.1007/s00535-025-02234-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2024] [Accepted: 02/21/2025] [Indexed: 05/22/2025]
Abstract
BACKGROUND Accurately predicting the prognosis of biliary atresia (BA) prior to Kasai portoenterostomy (KPE) remains a challenge. The identification of the specific BA population that may benefit from primary liver transplantation (pLT) instead of KPE remains elusive. METHODS A total of 196 BA patients and 31 age-matched non-BA cholestasis patients were recruited. BA patients were divided into training (February 2018-February 2019) and validation (March 2019-December 2021) cohorts. C-index was applied to evaluate the utility of indicators and models. RESULTS Serum fibroblast growth factor 19 (FGF19) was elevated in BA patients [95.67 (58.97-140.6) vs. 58.73 (43.59-85.35) pg/ml, P = 0.0003]. Constructed with FGF19, GGT, DBIL, and ALB, nomogram A demonstrated optimal C-index in training (0.767 ± 0.039) and validation (0.721 ± 0.062) cohorts with ideal consistency in predicting 1-year NLS after KPE as well as potential clinically utility in BA patients with an age at KPE ≤ 60 days. Leveraging the risk score (RS) developed with nomogram A, our findings revealed a notable decrease in 2-year NLS after KPE among BA patients with a preoperative RS > 1.36, and the patients with a preoperative RS > 2.6 appear to be potential candidates for pLT [2-year NLS after KPE: 0% (training cohort), 21.4% (validation cohort); specificity = 100% and sensitivity = 22.2%]. CONCLUSIONS Nomogram A demonstrated significant efficacy in preoperatively predicting NLS in early BA. BA patients (age at KPE ≤ 60 days) with a RS > 2.6 may potentially benefit from pLT.
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Affiliation(s)
- Jiajie Zhu
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Huifen Chen
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Lingdu Meng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Jingying Jiang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Yifan Yang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Junfeng Wang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Xue Ren
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Fanyang Kong
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Rui Dong
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Gong Chen
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China
| | - Shan Zheng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China.
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Jagadisan B, Dhawan A. Biomarkers in biliary atresia - an elusive promise of predicting outcome. Pediatr Res 2025; 97:1446-1448. [PMID: 39774329 PMCID: PMC12119343 DOI: 10.1038/s41390-024-03772-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/27/2024] [Accepted: 11/02/2024] [Indexed: 01/11/2025]
Abstract
Research on biomarkers in BA promises to predict and change outcomes in BA. This goal is still unrealized at the moment. The study by Kamp et al attempts to understand the drivers of fibrosis and elucidate the role of amyloid-related genes in the pathophysiology of BA. Based on this, they attempt to predict outcomes in BA using biomarkers by analysing plasma samples for amyloid precursor protein. There is a need to evaluate if shifting the focus from pre-KPE biomarkers to post-KPE biomarkers, serial evaluations and the use of composite scores could help in better prognostication.
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Affiliation(s)
- Barath Jagadisan
- Pediatric Liver GI and Nutrition Centre and Mowat Labs, King's College Hospital NHS Foundation Trust, London, UK
| | - Anil Dhawan
- Pediatric Liver GI and Nutrition Centre and Mowat Labs, King's College Hospital NHS Foundation Trust, London, UK.
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Utz Melere M, Sanha V, Farina M, da Silva CS, Nader L, Trein C, Lucchese AM, Ferreira C, Kalil AN, Feier FH. Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort. World J Transplant 2024; 14:88734. [PMID: 38576759 PMCID: PMC10989469 DOI: 10.5500/wjt.v14.i1.88734] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 11/14/2023] [Accepted: 12/18/2023] [Indexed: 03/15/2024] Open
Abstract
BACKGROUND Biliary atresia (BA) is the most common indication for pediatric liver trans plantation, although portoenterostomy is usually performed first. However, due to the high failure rate of portoenterostomy, liver transplantation has been advocated as the primary procedure for patients with BA. It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes. AIM To investigate the effect of prior portoenterostomy in infants un dergoing liver transplantation for BA. METHODS This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil. Patients with BA were divided into two groups: Those under going primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy. Continuous variables were compared using the Student's t-test or the Kruskal-Wallis test, and categorical variables were compared using the χ2 or Fisher's exact test, as appropriate. Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis. Patient and graft survival analyses were conducted with the Kaplan-Meier product-limit estimator, and patient subgroups were compared using the two-sided log-rank test. RESULTS Forty-two patients were included in the study (25 [60%] girls), 23 undergoing liver transplantation without prior portoenterostomy, and 19 undergoing liver transplantation with prior portoenterostomy. Patients with prior portoenterostomy were older (12 vs 8 months; P = 0.02) at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores (13.2 vs 21.4; P = 0.01). The majority of the patients (35/42, 83%) underwent living-donor liver transplantation. The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis (39 vs 11%), but this result did not reach statistical significance. Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation, graft-to-recipient weight ratio, and use of vascular grafts. Finally, the groups did not significantly differ in terms of post-transplant survival. CONCLUSION In our study, prior portoenterostomy did not significantly affect the outcomes of liver transplantation.
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Affiliation(s)
- Melina Utz Melere
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Valberto Sanha
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Marco Farina
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Carolina Soares da Silva
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Luiza Nader
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Cristine Trein
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Angelica Maria Lucchese
- Department of Hepato-biliary-pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
| | - Cristina Ferreira
- Department of Hepatology and Liver Transplantation, Santa Casa de Porto Alegre, Porto Alegre 90050170, Brazil
| | - Antonio Nocchi Kalil
- Department of Surgical Oncology, Santa Rita Hospital/Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90050-170, Rio Grande do Sul, Brazil
| | - Flavia Heinz Feier
- Department of Hepato-biliary-pancreatic Surgery and Liver Transplantation, Irmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre 90020-090, Brazil
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Sanha V, Franzini TA, Junior WF, Kalil AN. Are the Post-transplant Outcomes of Kasai's Early Failure and Late Failure Comparable to the Primary Liver Transplantation? Cureus 2023; 15:e51424. [PMID: 38299129 PMCID: PMC10828627 DOI: 10.7759/cureus.51424] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/31/2023] [Indexed: 02/02/2024] Open
Abstract
It is uncertain whether prior Kasai procedures negatively impact the outcomes of liver transplantation (LT). The prior meta-analysis did not distinguish between Kasai early failure (K-EF) and late failure (K-LF). Numerous studies have been recently published; therefore, we perform a systematic review and meta-analysis. We searched PubMed and Embase databases to identify studies comparing the outcomes of biliary atresia (BA) patients undergoing primary LT versus patients with prior Kasai procedures. Subgroup analysis was done at the time of Kasai failure (early vs. late). Twenty-five studies comprising 6,408 patients receiving LT were included in the analysis. We found a statistically significant increase in one-year graft survival in K-LF versus primary liver transplant (pLT) (P = 0.0003). One-year patient survival was also increased in K-LF, although not statistically significant (P = 0.09). No difference in the one- and five-year graft and patient survival, reoperation rate, infection, and biliary complication was seen in pLT vs overall prior Kasai (K-EF and K-LF). These results suggest that prior kasai procedure does not negatively impact the outcome of LT. In addition, BA patients with prior Kasai undergoing LT later in life tend to perform better than primary liver transplants.
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Affiliation(s)
- Valberto Sanha
- General Surgery, Federal University of Health Science of Porto Alegre, Porto Alegre, BRA
| | - Tales A Franzini
- Medicine, Federal University of Health Science of Porto Alegre, Porto Alegre, BRA
| | - Waldemir F Junior
- Surgery, Federal University of Health Science of Porto Alegre, Porto Alegre, BRA
| | - Antonio N Kalil
- General Surgery and Hepato-Pancreato-Biliary (HPB) & Transplant Surgery, Federal University of Health Science of Porto Alegre, Porto Alegre, BRA
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Neto JS, Chapchap P, Feier FH, Pugliese R, Vincenzi R, Benavides MR, Roda K, Kondo M, Fonseca EA. The impact of low recipient weight [≤ 7kg] on long-term outcomes in 1078 pediatric living donor liver transplantations. J Pediatr Surg 2022; 57:955-961. [PMID: 35697543 DOI: 10.1016/j.jpedsurg.2022.05.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2022] [Revised: 05/11/2022] [Accepted: 05/18/2022] [Indexed: 12/19/2022]
Abstract
BACKGROUND infants who require liver transplantation represent a treatment challenge because chronic liver disease at this early age affects the child's growth and development during a critical phase. The aim is to compare demographics, operative data, and long-term outcomes according to recipient weight at the time of LDLT. METHODS This retrospective study included primary LDLT analyzed in 2 groups: BW ≤ 7 kg (n = 322) and BW > 7 kg (n = 756). A historical comparison between periods was also investigated. RESULTS BW ≤ 7 kg had significantly lower height/age and weight/age z-scores, with median PELD score of 19. Transfusion rates were higher in the BW ≤ 7 kg group (30.9 ml/kg versus 15.5 ml/kg, P < 0.001). Higher frequencies of PV complications were seen in the BW ≤ 7 kg cohort. HAT and retransplantation rates were similar. Those with BW ≤ 7 kg required longer ICU and hospital stays. Patient and graft survival were similar. Patient survival in BW≤ 7 kg was significantly better in the most recent period. CONCLUSION Malnutrition and advanced liver disease were more frequent in BW ≤ 7 kg. Despite increased rates of PVT and longer hospital stay, patient and graft long-term survival were similar between groups.
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Affiliation(s)
- João Seda Neto
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil; Hospital Municipal Infantil Menino Jesus, São Paulo, SP, Brazil.
| | - Paulo Chapchap
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil
| | - Flavia H Feier
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil
| | - Renata Pugliese
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil; Hospital Municipal Infantil Menino Jesus, São Paulo, SP, Brazil
| | - Rodrigo Vincenzi
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil; Hospital Municipal Infantil Menino Jesus, São Paulo, SP, Brazil
| | - Marcel R Benavides
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil; Hospital Municipal Infantil Menino Jesus, São Paulo, SP, Brazil
| | - Karina Roda
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil; Hospital Municipal Infantil Menino Jesus, São Paulo, SP, Brazil
| | - Mário Kondo
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil
| | - Eduardo A Fonseca
- Hepatology and Liver Transplantation, Hospital Sírio-Libanês, Rua Barata Ribeiro, 414, cj 65, Bela Vista, SP, Brazil; Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, SP, Brazil; Hospital Municipal Infantil Menino Jesus, São Paulo, SP, Brazil
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Yoeli D, Choudhury RA, Sundaram SS, Mack CL, Roach JP, Karrer FM, Wachs ME, Adams MA. Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study. J Pediatr Surg 2022; 57:407-413. [PMID: 35065808 DOI: 10.1016/j.jpedsurg.2021.12.027] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/31/2021] [Revised: 11/20/2021] [Accepted: 12/30/2021] [Indexed: 12/20/2022]
Abstract
INTRODUCTION Kasai hepatoportoenterostomy is the standard of care for children with biliary atresia, but a majority of patients progress to end-stage liver disease and require a salvage liver transplant. Given the high failure rates of the hepatoportoenterostomy operation, some have advocated for primary liver transplantation as a superior treatment approach. The aim of this study was to compare outcomes of pediatric candidates with biliary atresia listed for primary vs. salvage liver transplantation. METHODS The SRTR/OPTN database was retrospectively reviewed for all children with biliary atresia listed for liver transplant between March 2002 and February 2021. Candidates were categorized as primary liver transplant if they had not undergone previous abdominal surgery prior to listing and salvage liver transplant if they had. Salvage transplants were further categorized as early failure if listed within the first year of life or late failure if listed at an older age. RESULTS 3438 children with biliary atresia were listed for transplant during the study period, with 15% of them listed for a primary transplant, 17% for salvage transplant after early failure, and 67% after late failure. Recipients of salvage liver transplant with late failure had lower bilirubin levels and were less critically ill as demonstrated by MELD/PELD scores and hospitalization status. Correspondingly, these recipients had higher waiting list and graft survival, though this did not remain statistically significant after adjustment in multivariable models. There were no differences in waiting list, recipient, or graft survival with primary vs. salvage liver transplant after early failure. CONCLUSION Kasai hepatoportoenterostomy should remain the standard of care in biliary atresia as it may delay need for transplant beyond the first year of life in a subset of recipients and does not jeopardize subsequent transplant outcomes, even with early failure. LEVELS OF EVIDENCE Retrospective cohort study (Level III).
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Affiliation(s)
- Dor Yoeli
- Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA.
| | - Rashikh A Choudhury
- Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
| | - Shikha S Sundaram
- Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, The Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
| | - Cara L Mack
- Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, The Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
| | - Jonathan P Roach
- Division of Pediatric Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
| | - Frederick M Karrer
- Division of Pediatric Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
| | - Michael E Wachs
- Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
| | - Megan A Adams
- Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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Betalli P, Cheli M, Colusso MM, Casotti V, Alberti D, Ferrari A, Starita G, Lucianetti A, Pinelli D, Colledan M, D'Antiga L. Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia. J Pediatr Surg 2022; 57:223-228. [PMID: 35063251 DOI: 10.1016/j.jpedsurg.2021.12.030] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/16/2021] [Revised: 11/09/2021] [Accepted: 12/29/2021] [Indexed: 12/11/2022]
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.
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Affiliation(s)
- Pietro Betalli
- Department of Paediatric Surgery, "Papa Giovanni XXIII" ASST, Piazza OMS1, Bergamo 24127, Italy.
| | - Maurizio Cheli
- Department of Paediatric Surgery, "Papa Giovanni XXIII" ASST, Piazza OMS1, Bergamo 24127, Italy
| | - Mara Marcella Colusso
- Department of Paediatric Surgery, "Papa Giovanni XXIII" ASST, Piazza OMS1, Bergamo 24127, Italy
| | - Valeria Casotti
- Department of Paediatric Hepatology, Gastroenterology and Transplantation, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Daniele Alberti
- Department of Paediatric Surgery, "Spedali Civili" Children's Hospital, Brescia, Italy
| | - Alberto Ferrari
- From Research Foundation, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Giusy Starita
- Department of Anaesthesia, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | | | - Domenico Pinelli
- Department of Surgery III, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Michele Colledan
- Department of Surgery III, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Lorenzo D'Antiga
- Department of Paediatric Hepatology, Gastroenterology and Transplantation, "Papa Giovanni XXIII" ASST, Bergamo, Italy
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Primary Liver Transplantation vs. Transplant after Kasai Portoenterostomy for Infants with Biliary Atresia. J Clin Med 2022; 11:jcm11113012. [PMID: 35683401 PMCID: PMC9181323 DOI: 10.3390/jcm11113012] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2022] [Revised: 05/04/2022] [Accepted: 05/23/2022] [Indexed: 02/04/2023] Open
Abstract
Introduction: Primary liver transplants (pLT) in patients with biliary atresia (BA) are infrequent, since most babies with BA undergo a prior Kasai portoenterostomy (KPE). This study compared transplant outcomes in children with BA with or without a prior KPE. We hypothesized that pLT have less morbidity and better outcomes compared to those done after a failed KPE. Methods: A retrospective review of patients with BA transplanted at our institution was performed. Patients were included if they received a pLT or if they were transplanted less than 2 years from KPE. Outcomes were compared between those groups. Comparisons were also made based on era (early: 1997−2008 vs. modern: 2009−2020). p < 0.05 was considered significant. Results: Patients who received a pLT were older at diagnosis (141.5 ± 46.0 vs. KPE 67.1 ± 25.5 days, p < 0.001). The time between diagnosis and listing for transplant was shorter in the pLT group (44.5 ± 44.7 vs. KPE 140.8 ± 102.8 days, p < 0.001). In the modern era, the calculated PELD score for the pLT was significantly higher (23 ± 8 vs. KPE 16 ± 8, p = 0.022). Two waitlist deaths occurred in the KPE group (none in pLT, p = 0.14). Both the duration of transplant surgery and transfusion requirements were similar in both groups. There was a significant improvement in graft survival in transplants after KPE between eras (early era 84.3% vs. modern era 97.8%, p = 0.025). The 1-year patient and graft survival after pLT was 100%. Conclusions: Patient and graft survival after pLT are comparable to transplants after a failed KPE but pLT avoids a prior intervention. There was no significant difference in pre- or peri-transplant morbidity between groups other than wait list mortality. A multicenter collaboration with more patients may help demonstrate the potential benefits of pLT in patients predicted to have early failure of KPE.
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Variability of Care and Access to Transplantation for Children with Biliary Atresia Who Need a Liver Replacement. J Clin Med 2022; 11:jcm11082142. [PMID: 35456234 PMCID: PMC9032543 DOI: 10.3390/jcm11082142] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2022] [Revised: 02/24/2022] [Accepted: 03/09/2022] [Indexed: 02/04/2023] Open
Abstract
Background & Aims: Biliary atresia (BA) is the commonest single etiology indication for liver replacement in children. As timely access to liver transplantation (LT) remains challenging for small BA children (with prolonged waiting time being associated with clinical deterioration leading to both preventable pre- and post-transplant morbidity and mortality), the care pathway of BA children in need of LT was analyzed—from diagnosis to LT—with particular attention to referral patterns, timing of referral, waiting list dynamics and need for medical assistance before LT. Methods: International multicentric retrospective study. Intent-to-transplant study analyzing BA children who had indication for LT early in life (aged < 3 years at the time of assessment), over the last 5 years (2016−2020). Clinical and laboratory data of 219 BA children were collected from 8 transplant centers (6 in Europe and 2 in USA). Results: 39 patients underwent primary transplants. Children who underwent Kasai in a specialist -but not transplant- center were older at time of referral and at transplant. At assessment for LT, the vast majority of children already were experiencing complication of cirrhosis, and the majority of children needed medical assistance (nutritional support, hospitalization, transfusion of albumin or blood) while waiting for transplantation. Severe worsening of the clinical condition led to the need for requesting a priority status (i.e., Peld Score exception or similar) for timely graft allocation for 76 children, overall (35%). Conclusions: As LT currently results in BA patient survival exceeding 95% in many expert LT centers, the paradigm for BA management optimization and survival have currently shifted to the pre-LT management. The creation of networks dedicated to the timely referral to a pediatric transplant center and possibly centralization of care should be considered, in combination with implementing all different graft type surgeries in specialist centers (including split and living donor LTs) to achieve timely LT in this vulnerable population.
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10
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Lim WX, Lin AN, Cheng YF, Lee SY, Hsu HW, Chen CL, Chang WC, Yu CY, Tsang LLC, Chuang YH, Ou HY. Portosystemic Shunt in Pediatric Living Donor Liver Transplant. Transplant Proc 2022; 54:403-405. [PMID: 35022136 DOI: 10.1016/j.transproceed.2021.09.072] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2021] [Accepted: 09/28/2021] [Indexed: 02/07/2023]
Abstract
BACKGROUND To evaluate the significance of portosystemic shunts and associated long-term outcomes in living donor liver transplant (LDLT) among pediatric patients. METHODS Retrospective review of 121 pediatric patients who underwent LDLT between May 1994 and December 2015 at Taiwan Kaohsiung Chang Gung Memorial Hospital. Pre- and postoperative computed tomography images of the liver were reviewed, and portal vein complications were assessed. RESULTS Ninety-seven pediatric patients were included in the study, and 70 had portosystemic shunts before transplant. Thirty-three patients have portal systemic shunt (PSS) 6 months after transplant (mean [SD] shunt size, 4.59 [1.98] mm). Thirty-seven patients' portosystemic shunts closed spontaneously (mean [SD] shunt size, 3.14 [1.06] mm). Smaller PSSs tend to close spontaneously with a cutoff point of 3.35 mm by receiver operating characteristic curve (P = .01). Patients with PSS have more portal vein complications than those without PSS (44.3% vs 11.1%, P = .02). Among PSS recipients, patients with portal vein complications tend to have larger PSS size (mean [SD], 4.14 [1.96] mm vs 3.59 [1.48] mm), although the difference is not statistically significant (P = .19). CONCLUSIONS In pediatric patients, preoperative portosystemic shunts are significantly correlated with portal venous complications, some of which require minimal interventions after LDLT with good outcomes. Shunts larger than 3.35 mm tend to persist after transplant with increased portal venous complications.
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Affiliation(s)
- Wei-Xiong Lim
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - An-Ni Lin
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Yu-Fan Cheng
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Sieh-Yang Lee
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Hsien-Wen Hsu
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Chao-Long Chen
- Department of Surgery, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Wan-Ching Chang
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Chun-Yen Yu
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Leo Leung-Chit Tsang
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Yi-Hsuan Chuang
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Hsin-You Ou
- Department of Diagnostic Radiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan.
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11
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Liu L, Wei L, Qu W, Liu Y, Zeng Z, Zhang H, Li M, Liu J, Wang HL, Li K, He Y, Jia J, Sun L, Zhao X, Zhu Z. Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia. Liver Transpl 2022; 28:224-235. [PMID: 34482616 DOI: 10.1002/lt.26287] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2021] [Revised: 08/27/2021] [Accepted: 09/01/2021] [Indexed: 12/31/2022]
Abstract
The aim is to explore the impact of the Kasai procedure (KP) and the length of native liver survival time (NLST) on outcomes of liver transplantation (LT). Patients with biliary atresia (BA), who underwent LT in Beijing Friendship Hospital from January 2017 to December 2019, were enrolled and divided into non-KP (N-KP) and post-KP (P-KP) groups. The patients in the P-KP group were further divided into early failure (KP-EF) defined by NLST <1 year, medium failure (KP-MF, NLST 1-5 years), and late failure (KP-LF, NLST >5 years) subgroups. Clinical data at baseline and during follow-up were collected. The inverse probability of treatment weighting method was used to evaluate the independent effect of KP and the length of NLST on clinical outcomes. Among 197 patients with BA, the N-KP group accounted for 43 (21.8%), KP-EF 71 (46.1%), KP-MF 59 (38.3%), and KP-LF 24 (15.6%) cases, respectively. The N-KP and KP-EF groups had significantly longer hospitalization and intensive care unit stays after LT. Graft and overall survival rates were 93.0% in the N-KP group and 97.4% in P-KP group, respectively. The mortality rate in the P-KP group were significantly lower compared with that of the N-KP group with a hazard ratio (HR) of 0.2 (P = 0.02). The risks of biliary and vascular complications and cytomegalovirus (CMV) infection after LT were significantly higher in KP-EF group than those in the KP-MF and KP-LF groups (HRs = 0.09, 0.2, and 0.3, respectively; all P < 0.001). The KP significantly improved after LT overall survival. Patients with early native liver failure after KP have significantly higher risks for biliary and vascular complications and CMV infection.
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Affiliation(s)
- Liwei Liu
- Liver Research Center, Beijing Key Laboratory of Translational Medicine in Liver Cirrhosis, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Lin Wei
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Wei Qu
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Ying Liu
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Zhigui Zeng
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Haiming Zhang
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Min Li
- Clinical Epidemiology and Evidence Base Medicine Unit, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Jimin Liu
- Department of Pathology and Molecular Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, Canada
| | - Hanlin L Wang
- Department of Pathology and Laboratory Medicine, David Geffen School of Medicine/Ronald Reagan Medical Center, University of California Los Angeles, Los Angeles, CA
| | - Kexin Li
- Liver Research Center, Beijing Key Laboratory of Translational Medicine in Liver Cirrhosis, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Yafei He
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Jidong Jia
- Liver Research Center, Beijing Key Laboratory of Translational Medicine in Liver Cirrhosis, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Liying Sun
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Xinyan Zhao
- Liver Research Center, Beijing Key Laboratory of Translational Medicine in Liver Cirrhosis, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
| | - Zhijun Zhu
- Liver Transplant Center, Clinical Center for Pediatric Liver Transplantation, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China
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12
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Kakos CD, Ziogas IA, Alexopoulos SP, Tsoulfas G. Management of biliary atresia: To transplant or not to transplant. World J Transplant 2021; 11:400-409. [PMID: 34631471 PMCID: PMC8465510 DOI: 10.5500/wjt.v11.i9.400] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/20/2021] [Revised: 06/26/2021] [Accepted: 08/18/2021] [Indexed: 02/06/2023] Open
Abstract
Kasai procedure (KP) and liver transplantation (LT) represent the only therapeutic options for patients with biliary atresia (BA), the most common indication for LT in the pediatric population. However, KP represents by no means a radical option but rather a bridging one, as nearly all patients will finally require a liver graft. More and more experts in the field of transplant surgery propose that maybe it is time for a paradigm change in BA treatment and abandon KP as transplantation seems inevitable. Inadequacy of organs yet makes this option currently not feasible, so it seems useful to find ways to maximize the efficacy of KP. In previous decades, multiple studies tried to identify these factors which opt for better results, but in general, outcomes of KP have not improved to the level that was anticipated. This review provides the framework of conditions which favor native liver survival after KP and the ones which optimize a positive LT outcome. Strategies of transition of care at the right time are also presented, as transplantation plays a key role in the surgical treatment of BA. Future studies and further organization in the transplant field will allow for greater organ availability and better outcomes to be achieved for BA patients.
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Affiliation(s)
| | - Ioannis A Ziogas
- Surgery Working Group, Society of Junior Doctors, Athens 15123, Greece
- Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN 37212, United States
| | - Sophoclis P Alexopoulos
- Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN 37212, United States
| | - Georgios Tsoulfas
- Department of Transplant Surgery, Aristotle University School of Medicine, Thessaloniki 54622, Greece
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13
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Capparelli MA, Ayarzabal VH, Halac ET, Questa HA, Minetto MJ, Cervio G, Barrenechea ME. Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia. Pediatr Surg Int 2021; 37:1183-1189. [PMID: 34057595 DOI: 10.1007/s00383-021-04933-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/24/2021] [Indexed: 01/11/2023]
Abstract
PURPOSE Biliary atresia is managed surgically by the Kasai portoenterostomy (KP). It has been reported by some groups that the outcomes of patients who have an early failed KP requiring a liver transplant (LTx) within the first year of life are worse than the outcomes of patients who undergo a primary LTx. The aim of this study was to identify preoperative parameters that could help predict what patients are at risk for the early failure of the procedure. MATERIALS AND METHODS We conducted a retrospective chart review of all patients who underwent a KP between January 2008 and May 2018. The following preoperative parameters were analyzed: age at KP, anatomical variant of the biliary atresia, degree of liver fibrosis, CMV status, and PELD score. The main outcome of the study was the early failure of the KP (EF-K), which was defined as the need for LTx before 1 year of age, or BA-related death before 1 year of age. Second, we analyzed the risk factors associated with death without LTx within the first year of life. RESULTS A total of 58 patients were included in the analysis. The native liver survival (NLS) was 56.5% and 48% at 1 and 5 years post KP, respectively. Overall survival (OS) was 79% and 76% at 1 and 5 years post KP, respectively. Early failure of KP occurred in 23 (39.7%) patients. OS in this group was 47% and 40% at 1 and 5 years, respectively. On the contrary, the OS of the remaining 35 (60.3%) patients was 100% at 1 and 5 years (P < 0.0001). When we compared all preoperative parameters, the only predictor of EF-K was the PELD score. When we analyzed the cases in the EF-K group who died without LTx, we found that the significant predictors were the cystic variant, a degree of liver fibrosis >4, and the PELD score. Nevertheless, on multivariate analysis, only PELD score was found as a statistically significant variable. CONCLUSION Due to bad prognosis found in EF-K patients, we believe that it could be reasonable to offer them a primary LTx. PELD score was found to be the strongest preoperative parameter that allows predicting which patient will likely have an early failed KP. Further prospective and multicenter studies are needed to reinforce these results.
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Affiliation(s)
- Mauro Ariel Capparelli
- Division of General Surgery, J. P. Garrahan Hospital, Combate de los Pozos 1881, C 1245 AAM, Buenos Aires, Argentina.
| | - Victor Hugo Ayarzabal
- Division of General Surgery, J. P. Garrahan Hospital, Combate de los Pozos 1881, C 1245 AAM, Buenos Aires, Argentina
| | - Esteban Tomas Halac
- Division of Liver Transplant, J. P. Garrahan Hospital, Buenos Aires, Argentina
| | - Horacio Alberto Questa
- Division of General Surgery, J. P. Garrahan Hospital, Combate de los Pozos 1881, C 1245 AAM, Buenos Aires, Argentina
| | - Maria Julia Minetto
- Division of Liver Transplant, J. P. Garrahan Hospital, Buenos Aires, Argentina
| | - Guillermo Cervio
- Division of Liver Transplant, J. P. Garrahan Hospital, Buenos Aires, Argentina
| | - Marcelo Eugenio Barrenechea
- Division of General Surgery, J. P. Garrahan Hospital, Combate de los Pozos 1881, C 1245 AAM, Buenos Aires, Argentina
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14
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Khayat A, Alamri AM, Saadah OI. Outcomes of late Kasai portoenterostomy in biliary atresia: a single-center experience. J Int Med Res 2021; 49:3000605211012596. [PMID: 33947263 PMCID: PMC8113946 DOI: 10.1177/03000605211012596] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
OBJECTIVES To describe the clinical, histopathologic, and outcomes data for a cohort of patients with biliary atresia (BA), and to identify the factors affecting survival. METHODS This was a cross-sectional study of all BA patients diagnosed between 1999 and 2017. Clinical, biochemical, imaging, and histopathologic data were analyzed, and Kaplan-Meier survival rates were compared to identify potential prognostic factors. RESULTS We evaluated 23 patients. The median age at the Kasai procedure was 77 ± 34 days, and the median overall survival was 12.5 ± 65 months. Thirteen (56%) patients survived with their native livers, 3 (13%) received a transplant, and 6 died (26%) while awaiting a transplant. Cholangitis and the use of ursodeoxycholic acid were associated with longer survival, while impaired synthetic function was associated with shorter survival. CONCLUSIONS Most patients presented late for the Kasai procedure. The survival rate with the native liver was comparable to other cohorts. Therefore, clinicians are encouraged to refer for the Kasai procedure even with late presentation (between 60 and 90 days), provided there is no hepatic decompensation.
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Affiliation(s)
- Ammar Khayat
- Department of Pediatrics, College of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia.,Pediatric Gastroenterology Unit, Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
| | - Aisha M Alamri
- Department of Pediatrics, East Jeddah General Hospital, Jeddah, Saudi Arabia
| | - Omar I Saadah
- Pediatric Gastroenterology Unit, Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia.,Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
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15
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Tambucci R, de Magnée C, Szabo M, Channaoui A, Pire A, de Meester de Betzenbroeck V, Scheers I, Stephenne X, Smets F, Sokal EM, Reding R. Sequential Treatment of Biliary Atresia With Kasai Hepatoportoenterostomy and Liver Transplantation: Benefits, Risks, and Outcome in 393 Children. Front Pediatr 2021; 9:697581. [PMID: 34307260 PMCID: PMC8292612 DOI: 10.3389/fped.2021.697581] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2021] [Accepted: 05/25/2021] [Indexed: 11/13/2022] Open
Abstract
Introduction: Surgical treatment of biliary atresia (BA) is still based on sequential strategy with Kasai hepatoportoenterostomy (KP) followed by liver transplantation (LT), in case of complicated secondary biliary cirrhosis. Concerns have been expressed regarding the risks of LT related to previous KP, suggesting primary LT as an exclusive treatment of BA. Methods: Single-center retrospective analysis including 393 pediatric patients who underwent LT for BA from 1993 to 2018, categorized into two groups: with (KP) or without (NoKP) previous KP. Pre-LT clinical condition was estimated considering age at LT, time on waiting list, pediatric end-stage liver disease score (PELD), and presence of portal vein hypoplasia. Post-LT outcome was evaluated considering patient and graft survival rates, and need for early reoperation due to abdominal or graft-related complications (<45 days after LT). Results: Two-hundred ninety-six patients (75.3%) were categorized in the KP group, and 97 (24.7%) in the NoKP group. Median age at LT was 1.14 years in the KP group and 0.85 years in the NoKP group (p < 0.0001). PELD score was significantly less severe in KP patients (p < 0.05). One-year patient survival rates were 96.9 and 96.8% in the KP and NoKP groups, respectively (p = 0.43), and the corresponding graft survival was 92.5 and 94.8% (p = 0.97). The need for early reoperation was more frequent in the KP group (29.8%) vs. NoKP group (12.4%, p = 0.01). The rate of bowel perforation was non-significantly higher in the KP group (8.1%) vs. NoKP group (3.1%, p = 0.11). Conclusions: The sequential strategy including KP and LT allowed performing LT in patients with significant older age and better clinical conditions, when compared to those transplanted without previous KP. Patient and graft survivals were not impacted by previous KP. Although previous KP was associated with an increased rate of post-LT surgical complications, bowel perforation and bleeding did not occur significantly more frequently. Such results support the current strategy based on sequential treatment.
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Affiliation(s)
- Roberto Tambucci
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Catherine de Magnée
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Margot Szabo
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Aniss Channaoui
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Aurore Pire
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Vanessa de Meester de Betzenbroeck
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Isabelle Scheers
- Pediatric Gastroenterology and Hepatology Division, Department of Pediatrics, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Xavier Stephenne
- Pediatric Gastroenterology and Hepatology Division, Department of Pediatrics, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Françoise Smets
- Pediatric Gastroenterology and Hepatology Division, Department of Pediatrics, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Etienne M Sokal
- Pediatric Gastroenterology and Hepatology Division, Department of Pediatrics, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
| | - Raymond Reding
- Pediatric Surgery and Transplantation Unit, Department of Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
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16
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LeeVan E, Matsuoka L, Cao S, Groshen S, Alexopoulos S. Biliary-Enteric Drainage vs Primary Liver Transplant as Initial Treatment for Children With Biliary Atresia. JAMA Surg 2019; 154:26-32. [PMID: 30208381 DOI: 10.1001/jamasurg.2018.3180] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
Importance Some infants with biliary atresia are treated with primary liver transplant (pLT), but most are initially treated with biliary-enteric drainage (BED) with a subsequent salvage liver transplant. Given the improvements in liver transplant outcomes, it is important to determine whether BED treatment remains the optimal surgical algorithm for patients with biliary atresia. Objective To compare the survival of patients with biliary atresia initially treated with BED with patients who underwent pLT. Design, Setting, and Participants This cohort study used deidentified records from the California Office of Statewide Health Planning and Development database to identify patients with biliary atresia (n = 1252) between January 1, 1990, through December 31, 2015. Patients were categorized into 1 of 2 cohorts: those who received BED treatment and those who underwent pLT. Excluded from the study were those born before January 1, 1995, and those without any documented operative intervention by age 5 years. Data analysis was performed from January 1, 1990, to December 31, 2015. Main Outcomes and Measures Overall survival was compared between the BED and pLT cohorts using the Kaplan-Meier method. The treatment's association with treatment era was examined by comparing survival before 2002 and on or after January 1, 2002. Results In total, 1252 patients with biliary atresia were identified. After exclusions, 626 remained; of these patients, 351 (56.1%) were female and 275 (43.9%) were male with a median (interquartile range) age at intervention for initial BED treatment of 65 (48-81) days. Among the 626 patients studied, initial BED treatment was performed in 313 patients (50.0%), and pLT was performed in 313 patients (50.0%). Although patients who underwent pLT had a higher mortality rate within the first 3 months after the procedure, they had a reduced risk of long-term mortality compared with patients initially managed with BED treatment (hazard ratio [HR] ≥6 months after the initial procedure, 0.19; 95% CI, 0.08-0.42; P = .01). Patients requiring salvage liver transplant had a substantially higher risk of mortality than patients who received pLT (HR, 0.43; 95% CI 0.25-0.76; P = .003). Those who underwent pLT had superior survival compared with BED treatment recipients on or after 2002 (HR, 0.16; 95% CI, 0.05-0.54; P < .001), and that persisted when censoring patients who underwent salvage liver transplant (HR, 0.23; 95% CI, 0.07-0.82; P = .01). Conclusions and Relevance Patients who underwent pLT experienced superior long-term survival compared with patients who underwent BED treatment. Multi-institutional trials are needed to determine which initial treatment is most advantageous to patients with biliary atresia.
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Affiliation(s)
- Elyse LeeVan
- Department of Surgery, Huntington Memorial Hospital, Pasadena, California
| | - Lea Matsuoka
- Division of Hepatobiliary and Liver Transplantation, Vanderbilt University Medical Center, Nashville, Tennessee
| | - Shu Cao
- Department of Preventive Medicine, Keck Hospital, University of Southern California, Los Angeles
| | - Susan Groshen
- Department of Preventive Medicine, Keck Hospital, University of Southern California, Los Angeles
| | - Sophoclis Alexopoulos
- Division of Hepatobiliary and Liver Transplantation, Vanderbilt University Medical Center, Nashville, Tennessee
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17
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Li S, Ma N, Meng X, Zhang W, Sun C, Dong C, Wang K, Wu B, Gao W. The effects of Kasai procedure on living donor liver transplantation for children with biliary atresia. J Pediatr Surg 2019; 54:1436-1439. [PMID: 30243738 DOI: 10.1016/j.jpedsurg.2018.07.022] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2018] [Revised: 06/19/2018] [Accepted: 07/31/2018] [Indexed: 02/07/2023]
Abstract
OBJECTIVE To evaluate the effects of Kasai procedure (hepatic portoenterostomy) on living donor liver transplantation (LDLT) for children with biliary atresia (BA). METHODS From January 2006 to January 2014, 150 children with BA were treated with LDLT in China. The children were categorized into pre-Kasai and non-Kasai groups, based on whether they had previously undergone Kasai procedure. Clinical data were retrospectively analyzed, and the difference in postoperative survival was compared between the groups. Preoperative data, including height, weight, serum bilirubin, and pediatric end-stage liver disease score, and perioperative blood loss, operation duration, incidence of postoperative surgical complications including vascular complications, bile duct complications, lymphatic fluid leakage, and digestive tract fistula were compared between the groups. RESULTS In total, 89 and 61 children were categorized in the pre-Kasai and non-Kasai groups, respectively. The 1-, 6-, and 12-month survival was 97.8%, 95.4%, and 95.4% for the Kasai group, and 98.4%, 96.7%, and 96.7% for the non-Kasai group, respectively (P > 0.05). The differences in mean operation duration and mean blood loss, and the incidences of outflow tract obstruction, portal vein stenosis, hepatic artery thrombosis, bile duct complications, lymphatic fluid leakage, and digestive tract fistula were not statistically significant between the groups (P > 0.05). CONCLUSION Kasai procedure could effectively delay the requirement of liver transplantation. In light of previous findings that Kasai procedure could significantly improve the liver transplantation-free survival of children with BA, we suggest that Kasai procedure should be used as a first-line treatment method for this condition. TYPE OF STUDY Treatment Study. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Shanni Li
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Nan Ma
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Xingchu Meng
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Wei Zhang
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Chao Sun
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Chong Dong
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Kai Wang
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Bin Wu
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China
| | - Wei Gao
- Tianjin First Center Hospital, Key Laboratory of Organ Transplant of Tianjin, China.
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18
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Hsiao CY, Ho CM, Wu YM, Ho MC, Hu RH, Lee PH. Biliary Complication in Pediatric Liver Transplantation: a Single-Center 15-Year Experience. J Gastrointest Surg 2019; 23:751-759. [PMID: 30632007 DOI: 10.1007/s11605-018-04076-x] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2018] [Accepted: 11/28/2018] [Indexed: 02/07/2023]
Abstract
BACKGROUND The incidence and several risk factors of biliary complication (BC) following pediatric liver transplantation (LT) are widely known, but data on long-term outcomes and management is limited. This retrospective study aimed to investigate the incidence, associated risk factors, management, and outcomes of early and late BC in pediatric LT. METHODS This study enrolled 134 pediatric patients (< 18 years old) who underwent LT at a tertiary care center in Taiwan between January 2001 and December 2015. Diagnosis of BC was based on clinical, biochemical, and radiologic examinations. Clinical data and chart records were reviewed and compared between the groups. RESULTS Among the 134 children, 21 children (15.7%) had BC after LT. Nine children had early complications, including leakage plus stricture (n = 2), stricture only (n = 2), and leakage only (n = 5). Twelve children had late BC; all of whom had anastomotic stricture. Of the 21 patients with BC, 11 patients (52.4%) were treated without surgery. The median time of first treatment for BC was 6.5 months (range, 11 days to 6.2 years). Five of the 9 patients with early complications and two of the 12 patients with late complications died of biliary tract infection. The major risk factors of BC in pediatric LT were (1) recipient age > 2 years, (2) Kasai portoenterostomy revision, and (3) hepatic artery thrombosis. CONCLUSIONS Several risk factors of BC in pediatric LT were identified. Children with early BC appeared to have relatively unfavorable outcomes. However, late BC treated by either radiological or surgical methods appeared to have a relatively good long-term prognosis.
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Affiliation(s)
- Chih-Yang Hsiao
- Department of Surgery, National Taiwan University Hospital Yun-Lin Branch, Douliou City of Yun-Lin County, Taiwan.,Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Cheng-Maw Ho
- Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan
| | - Yao-Ming Wu
- Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.,College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Ming-Chih Ho
- Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan. .,College of Medicine, National Taiwan University, Taipei, Taiwan. .,Department of Surgery, National Taiwan University Hospital, No. 7, Chung-Shan South Road, Taipei, Taiwan.
| | - Rey-Heng Hu
- Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.,College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Po-Huang Lee
- Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.,College of Medicine, National Taiwan University, Taipei, Taiwan
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19
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Gorgis NM, Kennedy C, Lam F, Thompson K, Coss-Bu J, Akcan Arikan A, Nguyen T, Hosek K, Miloh T, Karpen SJ, Penny DJ, Goss J, Desai MS. Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation. Hepatology 2019; 69:1206-1218. [PMID: 30076624 DOI: 10.1002/hep.30204] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2018] [Accepted: 07/28/2018] [Indexed: 12/13/2022]
Abstract
Cirrhotic cardiomyopathy (CCM), a comorbidity of end-stage cirrhotic liver disease, remains uncharacterized in children, largely because of a lack of an established pediatric definition. The aim of this retrospective cohort analysis is to derive objective two-dimensional echocardiographic (2DE) criteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence of BA-CCM with liver transplant (LT) outcomes in this population. Using receiver operating characteristic (ROC) curve analysis, optimal cut-off values for left ventricular (LV) geometrical parameters that were highly sensitive and specific for the primary outcomes: A composite of serious adverse events (CSAE) and peritransplant death were determined. These results were used to propose a working definition for BA-CCM: (1) LV mass index (LVMI) ≥95 g/m2.7 or (2) relative wall thickness of LV ≥0.42. Applying these criteria, BA-CCM was found in 34 of 69 (49%) patients with BA listed for LT and was associated with increased multiorgan dysfunction, mechanical and vasopressor support, and longer intensive care unit (ICU) and hospital stays. BA-CCM was present in all 4 waitlist deaths, 7 posttransplant deaths, and 20 patients with a CSAE (P < 0.01). On multivariable regression analysis, BA-CCM remained independently associated with both death and a CSAE (P < 0.01). Utilizing ROC analysis, LVMI was found to be a stronger predictor for adverse outcomes compared with current well-established markers, including Pediatric End-Stage Liver Disease (PELD) score. Conclusion: BA-CCM is highly sensitive and specific for morbidity and mortality in children with BA listed for LT. 2DE screening for BA-CCM may provide pertinent clinical information for prioritization and optimal peritransplant management of these children.
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Affiliation(s)
- Noelle M Gorgis
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
| | - Curtis Kennedy
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
| | - Fong Lam
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
| | - Kathleen Thompson
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
| | - Jorge Coss-Bu
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
| | - Ayse Akcan Arikan
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX.,Section of Pediatric Nephrology, Baylor College of Medicine, Houston, TX
| | - Trung Nguyen
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
| | - Kathleen Hosek
- Section of Pediatric Gastroenterology and Hepatology, Baylor College of Medicine, Houston, TX
| | - Tamir Miloh
- Section of Pediatric Gastroenterology and Hepatology, Baylor College of Medicine, Houston, TX
| | - Saul J Karpen
- Department of Pediatrics, Emory University School of Medicine, Atlanta, GA
| | - Daniel J Penny
- Section of Pediatric Cardiology, Baylor College of Medicine, Houston, TX
| | - John Goss
- Department of Surgery, Division of Abdominal Transplantation and Hepatobiliary Surgery, Baylor College of Medicine, Houston, TX
| | - Moreshwar S Desai
- Section of Pediatric Critical Care, Baylor College of Medicine, Houston, TX
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20
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D'Souza R, Grammatikopoulos T, Pradhan A, Sutton H, Douiri A, Davenport M, Verma A, Dhawan A. Acute-on-chronic liver failure in children with biliary atresia awaiting liver transplantation. Pediatr Transplant 2019; 23:e13339. [PMID: 30597734 DOI: 10.1111/petr.13339] [Citation(s) in RCA: 27] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/02/2018] [Revised: 11/05/2018] [Accepted: 11/25/2018] [Indexed: 12/19/2022]
Abstract
OBJECTIVES Acute-on-chronic liver failure (ACLF) is an acute decompensation of cirrhosis complicated by other organ failure and is associated with increased mortality and morbidity. ACLF has not been studied in children with biliary atresia (BA), which is the commonest indication for pediatric liver transplantation (LT) worldwide. This study aims to evaluate ACLF and outcomes in children with BA while awaiting deceased donor LT. METHODS This was a subanalysis of the dataset from a prospective cohort study of patients aged 0-18 years who underwent portoenterostomy for BA and were listed for LT at King's College Hospital, London, between 1999 and 2003. Outcomes included the development of ACLF, mortality, and complications. RESULTS Ninety-nine (41 male) children were included, and follow-up was 10 [6.0-15.0] years. A total of 20/99 children developed ACLF. ACLF was associated with increased mortality while awaiting LT (20% vs 4%; P = 0.03). There were no associations between biochemical parameters at listing and death. Increased bilirubin levels 3 months post-portoenterostomy was predictive of development of ACLF (AUROC = 0.72, P < 0.01). Age at LT and time on the waiting list in the ACLF subgroup were both lower compared to the non-ACLF group (P > 0.05). Sepsis and gastrointestinal bleeding were the commonest precipitants of ACLF. Complications included ascites, hepatic encephalopathy, and hepatorenal syndrome; the ACLF subgroup required multisystem support and longer intensive care unit stay. CONCLUSIONS ACLF in children with BA awaiting deceased donor LT carries increased mortality and morbidity. This warrants stratification of patients for earlier wait-listing and prioritization for LT.
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Affiliation(s)
- Rashmi D'Souza
- Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK
| | - Tassos Grammatikopoulos
- Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK
| | | | - Harry Sutton
- Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK
| | - Abdel Douiri
- Department of Primary Care and Public Health Sciences, King's College London, London, UK
| | - Mark Davenport
- Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK
| | - Anita Verma
- Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK.,Department of Medical Microbiology, King's College Hospital, London, UK
| | - Anil Dhawan
- Paediatric Liver, Gastroenterology and Nutrition Centre, King's College Hospital, London, UK
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21
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Contemporary Pediatric Liver Transplantation: Complex Challenges, Evolving Solutions. Int Anesthesiol Clin 2017; 55:148-161. [PMID: 28221170 DOI: 10.1097/aia.0000000000000138] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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22
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Sundaram SS, Mack CL, Feldman AG, Sokol RJ. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl 2017; 23:96-109. [PMID: 27650268 PMCID: PMC5177506 DOI: 10.1002/lt.24640] [Citation(s) in RCA: 160] [Impact Index Per Article: 20.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2016] [Accepted: 09/10/2016] [Indexed: 12/12/2022]
Abstract
Biliary atresia (BA) is a progressive, fibro-obliterative disorder of the intrahepatic and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation (LT). Indications for LT in BA include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extrahepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for LT. Optimal pretransplant management of these potentially life-threatening complications and maximizing nutrition and growth require the expertise of a multidisciplinary team with experience caring for BA. The timing of transplant for BA requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with BA often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical. Liver Transplantation 23:96-109 2017 AASLD.
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Affiliation(s)
- Shikha S. Sundaram
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
| | - Cara L. Mack
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
| | - Amy G. Feldman
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
| | - Ronald J. Sokol
- Digestive Health Institute and Pediatric Liver Center, Children’s Hospital Colorado, Section of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO
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23
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Abstract
Liver transplantation (LT) is an important component in the therapeutic armamentarium of managing end-stage liver disease. In North American children, biliary atresia remains the most common indication for LT compared to hepatitis C in adults, while hepatoblastoma is the most common liver tumor requiring LT, versus Hepatocellular carcinoma in adults. Rejection, lymphoproliferative disease, renal insufficiency, metabolic syndrome, recurrent disease, 'de novo' autoimmune hepatitis and malignancy require careful surveillance and prompt action in adults and children after LT. In children, specific attention to EBV viremia, growth, development, adherence and transition to the adult services is also required. Antibody mediated rejection and screening for donor specific antibodies is becoming important in managing liver graft dysfunction. Biomarkers to identify and predict tolerance are being developed. Machine perfusion and stem cells (iPS) to synthesize organs are generating interest and are a focus for research.
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Affiliation(s)
- Nanda Kerkar
- a Keck School of Medicine, Medical Director Liver/Intestinal and Hepatology Transplant Program, Children's Hospital of Los Angeles , University of Southern California , Los Angeles , CA , USA
| | - Arathi Lakhole
- b Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Los Angeles , University of Southern California , Los Angeles , CA , USA
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24
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Wang P, Xun P, He K, Cai W. Comparison of liver transplantation outcomes in biliary atresia patients with and without prior portoenterostomy: A meta-analysis. Dig Liver Dis 2016; 48:347-52. [PMID: 26748427 DOI: 10.1016/j.dld.2015.11.021] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/19/2015] [Revised: 11/15/2015] [Accepted: 11/19/2015] [Indexed: 12/11/2022]
Abstract
BACKGROUND Portoenterostomy is currently the standard first procedure for biliary atresia, and liver transplantation is reserved as a complementary therapy for those with late diagnosis, rapid hepatic decompensation, or failed portoenterostomy. Many previous publications have analysed the impact of prior portoenterostomy on the clinical outcomes of liver transplantation and the conclusions are discordant. METHODS PubMed and EMBASE were systematically searched for relevant articles, and studies published in Chinese were searched in the Wanfang China Medical Collections. The references of the retrieved studies were also reviewed. In addition, Google scholar was used to further confirm the literature search. RESULTS Fourteen studies were included comprising 1560 patients, of which 1190 (76.3%) received portoenterostomy. Meta-analysis did not reveal significant differences in either patient survival rate (odds ratio, 0.82) or graft survival rate (odds ratio, 1.11) over a 5-year follow-up between biliary atresia patients with and without a portoenterostomy procedure prior to liver transplantation; patients who received a prior portoenterostomy procedure had a higher risk of postoperative infection (odds ratio, 2.02). CONCLUSION Accumulated literature suggested that a prior portoenterostomy did not adversely affect outcomes of liver transplantation in children with biliary atresia.
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Affiliation(s)
- Panliang Wang
- Department of Pediatric Surgery, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Pengcheng Xun
- Department of Epidemiology and Biostatistics, School of Public Health-Bloomington, Indiana University, Bloomington, IN, USA
| | - Ka He
- Department of Epidemiology and Biostatistics, School of Public Health-Bloomington, Indiana University, Bloomington, IN, USA.
| | - Wei Cai
- Department of Pediatric Surgery, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China; Shanghai Key Laboratory of Pediatric Gastroenterology and Nutrition, Shanghai, China; Shanghai Institute of Pediatric Research, Shanghai, China.
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25
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Tomita H, Fuchimoto Y, Fujino A, Hoshino K, Yamada Y, Masugi Y, Sakamoto M, Kasahara M, Kanamori Y, Nakazawa A, Yoshida F, Akatsuka S, Nakano M, Kuroda T. Development and Validation of a Novel Fibrosis Marker in Biliary Atresia during Infancy. Clin Transl Gastroenterol 2015; 6:e127. [PMID: 26583502 PMCID: PMC4816091 DOI: 10.1038/ctg.2015.55] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2015] [Accepted: 10/12/2015] [Indexed: 12/29/2022] Open
Abstract
OBJECTIVES Most biliary atresia (BA) patients suffer from liver fibrosis and often require liver transplantation. The aim of this study was to develop and validate a novel fibrosis marker for BA patients aged <1 year-the infant BA liver fibrosis (iBALF) score-subsequent to the previously reported fibrosis marker for BA patients aged ≥1 year. METHODS From three institutions for pediatric surgery, BA patients and their native liver histology examinations performed at the age of <1 year were retrospectively identified and assigned to a development cohort (58 patients and 73 examinations) or validation cohort (92 patients and 117 examinations) according to their institutions. Histological fibrosis stages (F0-F4), blood test results, and clinical information at the time of liver histology examination were reviewed. The iBALF score was determined using multivariate ordered logistic regression analysis and was assessed for its associations with histological fibrosis stages. RESULTS The iBALF score equation was composed of natural logarithms, including serum total bilirubin level, blood platelet counts, and days of age. The score revealed a strong correlation with fibrosis stage (r=0.80 and 0.73 in the development and validation cohorts, respectively; P<0.001). The areas under the receiver-operating characteristic curves for diagnosing each fibrosis stage were 0.86-0.94 in the development cohort and 0.86-0.90 in the validation cohort (P<0.001), indicating good diagnostic power. In addition, no patient with an iBALF score >6 (equivalent to F4) at the initial surgery survived with their native liver at 1 year of age (n=9). CONCLUSIONS The iBALF score that was developed was a good noninvasive marker of native liver fibrosis for BA patients aged <1 year.
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Affiliation(s)
- Hirofumi Tomita
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.,Department of Pediatric Surgery, Saitama City Hospital, Midori-ku, Saitama-shi, Japan
| | - Yasushi Fuchimoto
- Department of Surgery, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan
| | - Akihiro Fujino
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Ken Hoshino
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Yohei Yamada
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Yohei Masugi
- Department of Pathology, Keio University School of Medicine, Shinjuku-ku, Japan
| | - Michiie Sakamoto
- Department of Pathology, Keio University School of Medicine, Shinjuku-ku, Japan
| | - Mureo Kasahara
- Department of Transplant Surgery, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan
| | - Yutaka Kanamori
- Department of Surgery, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan
| | - Atsuko Nakazawa
- Department of Pathology, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan
| | - Fumiko Yoshida
- Department of Pediatric Surgery, Saitama City Hospital, Midori-ku, Saitama-shi, Japan
| | - Seiya Akatsuka
- Department of Pathology, Saitama City Hospital, Midori-ku, Saitama-shi, Japan
| | - Miwako Nakano
- Department of Pediatric Surgery, Saitama City Hospital, Midori-ku, Saitama-shi, Japan
| | - Tatsuo Kuroda
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
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26
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Living Related Liver Transplantation for Biliary Atresia in the Last 5 years: Experience from the First Liver Transplant Program in India. Indian J Pediatr 2015; 82:884-9. [PMID: 25708058 DOI: 10.1007/s12098-014-1687-x] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/22/2014] [Accepted: 12/31/2014] [Indexed: 02/01/2023]
Abstract
OBJECTIVE To study the clinical, biochemical profile and outcome of patients with biliary atresia (BA) who underwent living related liver transplantation (LRLT) at authors' institute in the last 5 y (2008-2013). METHODS Case records of the 20 patients diagnosed with biliary atresia who had undergone living related liver transplantation at authors' centre in the last 5 y were analysed. RESULTS Eighteen patients with BA with a failed Kasai procedure and 2 without a prior Kasai's portoenterostomy received a liver transplant. At a median follow up of 2 y and 6 mo, both the patient and graft survival rates were 90 %. The median age of the recipients at the time of LRLT was 8 mo and 12 (60 %) of the transplanted children were less than or equal to 1 y of age. The male-female ratio was 1.8:1. The median weight was 7.3 kg (5.8-48 kg); two thirds were less than 10 kg. The median pre-transplant total serum bilirubin (TSB) and international normalized ratio (INR) were 12.98 (0.5-48.3) mg/dl and 1.3 (1.0-3.9) respectively. All patients received a living related graft and there was no donor mortality. The median duration of postoperative ventilation was 14 h. The post-operative complications were infection (30 %), vascular complications (20 %) and acute rejection (20 %). The median duration of postoperative hospital stay was 21 d (17-42). Two patients died of combined hepatic and portal vein thrombosis in the early postoperative period. Late rejection was encountered in 1 patient and another developed chronic kidney disease necessitating a renal transplant. There were no late vascular occlusions or development of post transplant lymphoproliferative disease. CONCLUSIONS Thus, LRLT for BA with or without a prior portoenterostomy, is a feasible and successful treatment modality with good outcomes attained despite the challenges of age and size. This treatment modality is now well established in India.
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27
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Li QG, Wan P, Zhang JJ, Chen QM, Chen XS, Han LZ, Xia Q. Liver transplantation for biliary atresia: A single-center study from mainland China. World J Gastroenterol 2015; 21:9638-9647. [PMID: 26327772 PMCID: PMC4548125 DOI: 10.3748/wjg.v21.i32.9638] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/15/2015] [Revised: 05/10/2015] [Accepted: 06/10/2015] [Indexed: 02/06/2023] Open
Abstract
AIM: To summarize our single-center experience with liver transplantation (LT) for biliary atresia (BA).
METHODS: From October 2006 to December 2012, 188 children with BA were analyzed retrospectively. The stage I group (from October 2006 to December 2010) comprised the first 74 patients, and the stage II group (from January 2011 to December 2012) comprised the remaining 114 patients. Finally, 123 liver transplants were performed in 122 (64.9%) patients, whereas 66 patients did not undergo LT due to denial by their parents or lack of suitable liver grafts. The selection of graft types depended on the patients’ clinical status and whether a suitable living donor was available. The characteristics of patients in stages I and II were described, and the surgical outcomes of LT recipients were compared between the two stages. The Kaplan-Meier method was used to estimate the cumulative patient and graft survival rates, and the equality of survival distributions was evaluated using the log-rank test.
RESULTS: The 188 children consisted of 102 boys and 86 girls. Their ages ranged from 3 to 144 mo with a median of 8 mo. One hundred and fifteen (61.2%) patients were born in rural areas. Comparing stage I and stage II patients, the proportion of patients referred by pediatricians (43.2% vs 71.1%, respectively; P < 0.001) and the proportion of patients who previously received a Kasai procedure (KP) (32.4% vs 44.7%, respectively; P = 0.092) obviously increased, and significantly more parents were willing to treat their children with LT (73% vs 86%, respectively; P = 0.027). Grafts from living donors (102/122, 83.6%) were the most commonly used graft type. Surgical complications (16/25, 64.0%) were the main reason for posttransplant mortality. Among the living donor liver transplantation recipients (n = 102), the incidence of surgical complications was significantly reduced (34.1% vs 15.5%, respectively; P = 0.029) and survival rates of patients and grafts were greatly improved (81.8% vs 89.7%, respectively, at 1 year; 75.0% vs 87.8%, respectively, at 3 years; P = 0.107) from stage I to stage II.
CONCLUSION: The status of surgical treatments for BA has been changing in mainland China. Favorable midterm outcomes after LT were achieved as centers gained greater technical experience.
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28
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Qiao G, Li L, Cheng W, Zhang Z, Ge J, Wang C. Conditional probability of survival in patients with biliary atresia after Kasai portoenterostomy: a Chinese population-based study. J Pediatr Surg 2015; 50:1310-5. [PMID: 25917622 DOI: 10.1016/j.jpedsurg.2015.03.062] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/29/2014] [Revised: 03/17/2015] [Accepted: 03/29/2015] [Indexed: 01/23/2023]
Abstract
Survival estimates are commonly reported for the outcomes of patients. Conditional probability, which offers more relevant information regarding the prognosis for patients once they have survived for some time, are lacking for patients with biliary atresia. The aim of this study is to calculate the conditional probability of survival in patients with biliary atresia after Kasai portoenterostomy. Clinical data from 244 consecutive patients who underwent Kasai portoenterostomy were reviewed and the actuarial survival estimated. Conditional survival probabilities and 95% CIs were calculated. Of the 244 patients, the overall median NLS was 41.2 months (95% CI:30.8-51.6 months), and the 1-year, 3-year, and 5-year NLS rates were 85.4%, 61.1%, and 43.3%, respectively. The probabilities of surviving at the time point of 5 years given survival to 1, 2, 3, and 4 years were 50%, 56%, 73%, and 93%, respectively. For conditional survival for 1 additional year, patients with age >90 days and cholangitis had significantly predictive values(both P<0.05). Cox proportional hazards model showed that age > 90 days(HR: 2.519, P=0.001), and postoperative cholangitis(HR:2.568, P<0.001) were associated with an adverse NLS in the multivariable adjusted analysis. our findings showed that conditional survival of patients with BA is not linear over time after Kasai operation. Information derived from conditional survival can be used to better manage patients with BA, including the potential subsequent treatment decisions, the chance of listing for transplantation, or even the end-of-life planning.
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Affiliation(s)
- Guoliang Qiao
- Graduate School of Peking Union Medical College, Beijing, People's Republic of China; Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China
| | - Long Li
- Graduate School of Peking Union Medical College, Beijing, People's Republic of China; Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China.
| | - Wei Cheng
- Department of Surgery, United Family Hospital, Beijing, People's Republic of China; Department of Paediatrics and Department of Surgery, Faculty of Medicine, Nursing and Health Sciences, Monash University, Australia
| | - Zhen Zhang
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China
| | - Juntao Ge
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China
| | - Chen Wang
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China
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29
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Neto JS, Feier FH, Bierrenbach AL, Toscano CM, Fonseca EA, Pugliese R, Candido HL, Benavides MR, Porta G, Chapchap P. Impact of Kasai portoenterostomy on liver transplantation outcomes: A retrospective cohort study of 347 children with biliary atresia. Liver Transpl 2015; 21:922-7. [PMID: 25832004 DOI: 10.1002/lt.24132] [Citation(s) in RCA: 40] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2015] [Revised: 03/02/2015] [Accepted: 03/07/2015] [Indexed: 02/07/2023]
Abstract
Biliary atresia (BA) is the main diagnosis leading to liver transplantation (LT) in children. When diagnosed early in life, a Kasai portoenterostomy (Kasai-PE) can prevent or postpone LT. Instances of previous operations can result in difficulties during the LT. We hypothesized that a previous Kasai-PE could affect LT outcomes. A retrospective cohort study of 347 BA patients submitted to LT between 1995 and 2013 at Hospital Sírio-Libanês and A. C. Camargo Cancer Center was conducted. Patients were divided into those with a previous Kasai portoenterostomy early failure (K-EF), Kasai portoenterostomy late failure (K-LF), and those with no Kasai portoenterostomy (No-K). Primary outcomes were patient and graft survival. A total of 94 (27.1%) patients had a K-EF, 115 (33.1%) had a K-LF, and 138 (39.8%) had No-K before LT. Children in the K-LF group were older and had lower Pediatric End-Stage Liver Disease (PELD) scores. Patients in both K-EF and K-LF groups had more post-LT biliary complications. After Cox-multivariate analysis adjusting for confounding factors to determine the influence of Kasai-PE on patient and graft survival, the K-LF group had an 84% less probability of dying and a 55% less chance to undergo retransplantation. The K-LF group had a protective effect on posttransplant patient and graft survival. When properly performed, the Kasai procedure can postpone LT and positively affect outcomes. Having a K-EF and having not performed a Kasai-PE had the same effect in patient and graft survival; however, a previous Kasai-PE can increase post-LT complications as biliary complications and bowel perforations.
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Affiliation(s)
- Joao Seda Neto
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Flávia H Feier
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Ana Luiza Bierrenbach
- Teaching and Research Institute, Hospital Sirio-Libanes, São Paulo, São Paulo, Brazil
| | - Cristiana M Toscano
- Department of Health, Instituto de Patologia Tropical e Saúde Pública, Universidade Federal de Goiás, Goiás, Brazil
| | - Eduardo A Fonseca
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Renata Pugliese
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Helry L Candido
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Marcel R Benavides
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Gilda Porta
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil.,Hepatology and Liver Transplantation, A. C. Camargo Cancer Center, São Paulo, São Paulo, Brazil
| | - Paulo Chapchap
- Hepatology and Liver Transplantation, Sao Paulo, Sao Paulo, Brazil
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Julka KD, Lin TS, Chen CL, Wang CC, Komorowski AL. Reconstructing single hepatic artery with two arterial stumps: biliary complications in pediatric living donor liver transplantation. Pediatr Surg Int 2014; 30:39-46. [PMID: 24292409 DOI: 10.1007/s00383-013-3436-z] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/14/2013] [Indexed: 01/03/2023]
Abstract
INTRODUCTION Liver grafts can at times have two hepatic arterial stumps. This can result in a dilemma whether to reconstruct single or both the arteries. Hepatic artery (HA) thrombosis is the most dreaded complication in pediatric living donor liver transplantation (LDLT) as it can result in biliary complications and subsequent graft loss. We herein report the feasibility of reconstructing single hepatic artery in pediatric living donor liver transplantation having two arterial stumps in the liver graft. MATERIALS AND METHODS From 2008 to 2010, 87 pediatric patients undergoing LDLT were divided into three groups. Group 1 (n = 20): two HA stumps with two HA reconstruction, Group 2 (n = 22): two HA stumps with one HA reconstruction and Group 3 (n = 45): one HA stump with one HA reconstruction. The decision regarding the reconstruction of single or multiple HAs was made depending on the pre-operative radiological and intraoperative assessments. RESULTS The incidence of HA thrombosis (p = 0.126) and biliary complications (p = 0.617), was similar in the three groups. CONCLUSION Single HA reconstruction does not increase the risk of biliary strictures in pediatric LDLT recipients having dual hepatic arterial stumps in the liver graft.
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Affiliation(s)
- Karan D Julka
- Liver Transplantation Program and Department of Surgery, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 123 Ta-Pei Road, Niao-Song, Kaohsiung, 833, Taiwan,
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Sun LY, Yang YS, Zhu ZJ, Gao W, Wei L, Sun XY, Qu W, Rao W, Zeng ZG, Dong C, Tu JP, Wang J, Liu YH, Liu Y, Yu LX, Wang Y, Li J, Shen ZY. Outcomes in children with biliary atresia following liver transplantation. Hepatobiliary Pancreat Dis Int 2013; 12:143-8. [PMID: 23558067 DOI: 10.1016/s1499-3872(13)60023-5] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
BACKGROUND Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.
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Affiliation(s)
- Li-Ying Sun
- Nankai University School of Medicine, Tianjin 300071, China
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Gallo A, Esquivel CO. Current options for management of biliary atresia. Pediatr Transplant 2013; 17:95-8. [PMID: 23347466 DOI: 10.1111/petr.12040] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/19/2012] [Indexed: 11/28/2022]
Abstract
It is encouraging that we are improving the technical aspects of treatment modalities for biliary atresia. However, it is clear that more needs to be done to best develop new treatment plans while applying the modalities we have (porto-enterostomy or liver transplantation or both) in a way that will afford the best survival and quality-of-life. This review article will discuss a number of points that are vital to improving care and illustrates the need to further scrutinize treatment decisions.
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Affiliation(s)
- Amy Gallo
- Division of Abdominal Transplantation, Stanford University School of Medicine, Lucile Packard Children's Hospital at Stanford, Stanford, CA 94305, USA
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