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Samanta A, Sarma MS. "Post-Kasai Portoenterostomy Cholangitis: What Have We Learnt So Far?". J Clin Exp Hepatol 2025; 15:102471. [PMID: 39816454 PMCID: PMC11730534 DOI: 10.1016/j.jceh.2024.102471] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/09/2024] [Accepted: 11/30/2024] [Indexed: 01/12/2025] Open
Abstract
Post-Kasai portoenterostomy (KPE) cholangitis is one of the most common complications that has a negative impact on liver function and native liver survival. Early diagnosis and judicious empiric antimicrobial management are, therefore, important to prevent further liver damage and decompensation. However, there is no consensus regarding the standard definition of post-KPE cholangitis, and established guidelines on evaluation and management are also lacking. Metagenomic next-generation sequencing, a new molecular diagnostic technique, has the potential for detecting broader spectrum of pathogens, especially in blood culture-negative patients. Antibiotic prophylaxis to prevent cholangitis has been widely used, but questions over the choice of antibiotics, route of administration, and optimal duration remain unsettled. The available evidence on the efficacy of antibiotic prophylaxis in preventing cholangitis has shown conflicting results. This review offers a summary of the current research on advances in diagnostic approaches, including molecular techniques, and therapeutic challenges in managing intractable cholangitis.
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Affiliation(s)
- Arghya Samanta
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India
| | - Moinak S. Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India
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2
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Ravanbakhsh N, Chapin CA, Li Y, Jhaveri R. "Where Did This Come From?": Antibiotic Prophylaxis in Biliary Atresia After Kasai Procedure. J Pediatric Infect Dis Soc 2024; 13:282-284. [PMID: 38588463 DOI: 10.1093/jpids/piae028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2023] [Accepted: 03/25/2024] [Indexed: 04/10/2024]
Abstract
For patients with Biliary atresia, antibiotic prophylaxis after Kasai portoenterostomy is a common practice. Societal guidelines often cite one reference as supportive evidence for this practice. In this paper, we go back to review the quality of this evidence and suggest more research is required to demonstrate the efficacy of antibiotic prophylaxis in this population.
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Affiliation(s)
- Naseem Ravanbakhsh
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA
| | - Catherine A Chapin
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA
- Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Yun Li
- Department of Biostatistics, Epidemiology and Informatics, University of Pennsylvania, Philadelphia, PA, USA
- Children's Hospital of Philadelphia, Philadelphia, PA, USA
| | - Ravi Jhaveri
- Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
- Division of Pediatric Infectious Diseases, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA
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Lu X, Jiang J, Shen Z, Chen G, Np YW, Xiao X, Yan W, Zheng S. Effect of Adjuvant Steroid Therapy in Type 3 Biliary Atresia: A Single-Center, Open-Label, Randomized Controlled Trial. Ann Surg 2023; 277:e1200-e1207. [PMID: 35170539 DOI: 10.1097/sla.0000000000005407] [Citation(s) in RCA: 8] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
OBJECTIVE To evaluate the efficacy and side effects of additional postoperative steroid therapy for type 3 BA versus the current routine care. SUMMARY BACKGROUND DATA Whether steroid therapy post-Kasai portoen-terostomy improves the outcomes of BA remains controversial. Clinical evidence from 2 randomized trials in the UK and USA do not support the routine use of steroid in the treatment of BA. METHODS In this open-label randomized controlled trial, patients with type 3 BA were randomized to routine postoperative treatment with or without 10 to 12 weeks of adjuvant steroid treatment. The primary outcome was the postoperative jaundice clearance rate with native liver at 6 months. The secondary outcomes included postoperative jaundice clearance rate at 3, 12, and 24 months, survival with native liver at 12 and 24 months, and SAEs within 3 months. RESULTS Overall, 200 participants were randomized and allocated into either steroid or control group (n = 100/group). The proportion of participants that are jaundice free without liver transplantation was significantly higher in the steroid group than in the control group at 6 months (54.1% vs 31.0%, P = 0.0015). The native liver survival rate was higher postoperatively in the steroid group than in the control group at 12 (66.3% vs 50.0%, P = 0.02) and 24 (57.1% vs 40.0%, P = 0.02) months. The survival time with native liver was significantly longer in the steroid group than in the control group (median survival, steroid vs control: not reached vs 1.21 years, P = 0.02). There were no significant differences between the 2 groups in the mean occurrence of SAEs within 3 months (steroid vs control: 0.63 vs 0.45, P = 0.20). CONCLUSIONS Postoperative adjuvant steroid intervention improved bile drainage and survival with native liver in type 3 BA patients, without increasing early-stage SAEs.
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Affiliation(s)
- Xuexin Lu
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
| | - Jingying Jiang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
| | - Zhen Shen
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
| | - Gong Chen
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
| | - Ying Wu Np
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
| | - Xianmin Xiao
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
| | - Weili Yan
- Clinical Trial Unit (CTU), Children's Hospital of Fudan University, Shanghai, China
| | - Shan Zheng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China
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Matcovici M, Stoica I, Smith K, Davenport M. What Makes A "Successful" Kasai Portoenterostomy "Unsuccessful"? J Pediatr Gastroenterol Nutr 2023; 76:66-71. [PMID: 36574004 DOI: 10.1097/mpg.0000000000003638] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
OBJECTIVES Clearance of jaundice (CoJ) is the first key objective of Kasai portoenterostomy (KPE) for biliary atresia (BA) and its achievement is by far the best index of long-term prognosis. We sought to identify the reasons for failure [subsequent liver transplant (LT)] in this cohort. METHODS Review of single-center prospective BA database. Successful KPE was defined by achieving a postoperative bilirubin of ≤20 µmol/L. Pre-KPE and post-KPE variables were identified together with a multivariate logistic regression model to identify those observable at 3 months post-KPE. Data are quoted as median (range). A P value of ≤0.05 was significant. RESULTS One hundred thirty-five infants underwent KPE between January 2012 and December 2018, of which 90 (67%) achieved CoJ. From these 20 (22%) (Cohort A) underwent LT with the remainder continuing with native liver (Cohort B) (median follow-up of 4.15 years). There was no difference in age at KPE ( P = 0.41), APRi (aspartate aminotransferase-to-platelet ratio) ( P = 0.07), associated anomalies ( P = 0.7), and cytomegalovirus status ( P = 0.7) between the 2 groups. Postoperatively, both cholangitis [any episode, 18/20 (90%) vs 15/70 (21%); P < 0.0001] and portal hypertension (PHT) [gastrointestinal (GI) bleed, 10/20 (50%) vs 2/70 (2.8%); P < 0.0001] were significantly more common in cohort A. Univariate analysis showed that the most significant predictive values at 3 months for LT by 2 years were high APRi, bilirubin, international normalized ratio, and ultrasound (US)-detected ascites with multivariate logistic modeling confirming these variables with predictive values of r2 = 0.79, AUROC = 0.98. CONCLUSIONS Failure is not preordained at KPE but due to recurrent cholangitis and/or symptoms of PHT.
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Affiliation(s)
- Melania Matcovici
- From the Department of Paediatric Surgery, Kings College Hospital, London, United Kingdom
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Abstract
This article discusses current standard of care in neonatal biliary disease, particularly management of biliary atresia and choledochal cysts. It highlights surgical considerations, guidelines for adjuvant therapies, and promising therapeutic options that are under investigation.
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Affiliation(s)
- Sarah Mohamedaly
- Division of Pediatric Surgery, Department of Surgery, University of California, San Francisco, 513 Parnassus Avenue, HSW 1652, Campus Box 0570, San Francisco, CA 94143-0570, USA
| | - Amar Nijagal
- Division of Pediatric Surgery, Department of Surgery, University of California, San Francisco, 513 Parnassus Avenue, HSW 1652, Campus Box 0570, San Francisco, CA 94143-0570, USA; The Liver Center, University of California, San Francisco, CA, USA; The Pediatric Liver Center at UCSF Benioff Childrens' Hospitals, San Francisco, CA, USA.
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Calinescu AM, Madadi-Sanjani O, Mack C, Schreiber RA, Superina R, Kelly D, Petersen C, Wildhaber BE. Cholangitis Definition and Treatment after Kasai Hepatoportoenterostomy for Biliary Atresia: A Delphi Process and International Expert Panel. J Clin Med 2022; 11:jcm11030494. [PMID: 35159946 PMCID: PMC8836553 DOI: 10.3390/jcm11030494] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2021] [Accepted: 01/14/2022] [Indexed: 12/17/2022] Open
Abstract
(1) Background: Acute cholangitis during the first year after Kasai hepatoportoenterostomy (HPE) has a negative impact on patient and native liver survival. There are no consistent guidelines for the definition, treatment, and prophylaxis of cholangitis after HPE. The aim of this study was to develop definition, treatment, and prophylaxis guidelines to allow for expeditious management and for standardization in reporting. (2) Methods: the Delphi method, an extensive literature review, iterative rounds of surveys, and expert panel discussions were used to establish definition, treatment, and prophylaxis guidelines for cholangitis in the first year after HPE. (3) Results: Eight elements (pooled into two groups: clinical and laboratory/imaging) were identified to define cholangitis after HPE. The final proposed definitions for suspected and confirmed cholangitis are a combination of one element, respectively, two elements from each group; furthermore, the finding of a positive blood culture was added to the definition of confirmed cholangitis. The durations for prophylaxis and treatment of suspected and confirmed cholangitis were uniformly agreed upon by the experts. (4) Conclusions: for the first time, an international consensus was found for guidelines for definition, treatment, and prophylaxis for cholangitis during the first year after Kasai HPE. Applicability will need further prospective multicentered studies.
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Affiliation(s)
- Ana M. Calinescu
- Division of Child’s and Adolescent’s Surgery, Swiss Pediatric Liver Center, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland;
- Correspondence: ; Tel.: +41-22-382-46-62
| | - Omid Madadi-Sanjani
- Department of Pediatric Surgery, Hannover Medical School, 30625 Hannover, Germany; (O.M.-S.); (C.P.)
| | - Cara Mack
- Section of Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO 80011, USA;
| | - Richard A. Schreiber
- Division of Gastroenterology, Hepatology and Nutrition, BC Children’s Hospital, University of British Columbia, Vancouver, BC V5Z 4H4, Canada;
| | - Riccardo Superina
- Division of Transplant Surgery, Ann and Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA;
| | - Deirdre Kelly
- Liver Unit, Birmingham Women’s and Children’s Hospital, Birmingham B15 2TG, UK;
| | - Claus Petersen
- Department of Pediatric Surgery, Hannover Medical School, 30625 Hannover, Germany; (O.M.-S.); (C.P.)
| | - Barbara E. Wildhaber
- Division of Child’s and Adolescent’s Surgery, Swiss Pediatric Liver Center, Geneva University Hospitals, University of Geneva, 1205 Geneva, Switzerland;
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Fligor SC, Hirsch TI, Tsikis ST, Adeola A, Puder M. Current and emerging adjuvant therapies in biliary atresia. Front Pediatr 2022; 10:1007813. [PMID: 36313875 PMCID: PMC9614654 DOI: 10.3389/fped.2022.1007813] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/31/2022] [Accepted: 09/26/2022] [Indexed: 11/21/2022] Open
Abstract
Following Kasai hepatic portoenterostomy (HPE), most patients with biliary atresia will eventually require liver transplantation due to progressive cirrhosis and liver failure. Preventing liver transplantation, or even delaying eventual liver transplantation, is the key to improving long-term outcomes. This review first examines the commonly used adjuvant therapies in post-HPE biliary atresia and the strength of the evidence supporting these therapies. Next, it examines the evolving frontiers of management through a comprehensive evaluation of both recently completed and ongoing clinical trials in biliary atresia. Promising therapies used in other cholestatic liver diseases with potential benefit in biliary atresia are discussed. Improving post-HPE management is critical to prevent complications, delay liver transplantation, and ultimately improve the long-term survival of patients with biliary atresia.
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Affiliation(s)
- Scott C Fligor
- Vascular Biology Program, Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
| | - Thomas I Hirsch
- Vascular Biology Program, Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
| | - Savas T Tsikis
- Vascular Biology Program, Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
| | - Andrew Adeola
- Vascular Biology Program, Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
| | - Mark Puder
- Vascular Biology Program, Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States
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8
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Sakamoto S, Hashizume N, Yagi M, Sasaki H, Nio M. Postoperative pharmacotherapy for patients with biliary atresia in Japan. Pediatr Int 2022; 64:e14990. [PMID: 34528345 DOI: 10.1111/ped.14990] [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: 09/16/2020] [Revised: 09/07/2021] [Accepted: 09/13/2021] [Indexed: 11/27/2022]
Abstract
BACKGROUND Members of the Japanese Biliary Atresia Society were surveyed using questionnaires that assess their current practice regarding postoperative pharmacotherapy for outpatients with biliary atresia (BA). METHODS In September 2018, questionnaires were sent to 100 member institutions of the Japanese Biliary Atresia Society. Questionnaires included the number of BA outpatients per institution and pharmacotherapy for outpatients with native liver. Pharmacotherapies were categorized into antibiotics, cholagogues, hepatoprotective agents, branched-chain amino acid supplement, Japanese Kampo medicine, probiotics, laxative, glycerin enema, and "others." In each category, the questionnaires asked about the medicine's details and the time of withdrawal of administration. RESULTS Responses were collected from 58 of the 100 institutions. Fifty-four institutions (94.7%) had prescribed one or more medicines as postoperative pharmacotherapy, and three institutions (5.3%) did not prescribe any medicines. Fifty-three institutions (93.0%) had prescribed ursodeoxycholic acid (UDCA), and 32 (60.4%) of these continued prescribing UDCA as long as the condition of patients remained unchanged. Twenty-nine (50.9%) had prescribed Japanese Kampo medicines ("Inchinkoto" in all cases). Twenty-four (42.1%) had prescribed antibiotics, mainly trimethoprim-sulfamethoxazole, in 21 (87.5%). Twenty-three (40.4%) had prescribed probiotics. CONCLUSIONS There were many variations of pharmacotherapy in BA outpatients with native liver in Japan, including antibiotic, probiotic, and Inchinkoto prescriptions. Of the various drugs, the most commonly administered was UDCA.
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Affiliation(s)
- Saki Sakamoto
- Department of Pediatric Surgery, Kurume University School of Medicine, Kurume, Fukuoka, Japan
| | - Naoki Hashizume
- Department of Pediatric Surgery, Kurume University School of Medicine, Kurume, Fukuoka, Japan
| | - Minoru Yagi
- Department of Pediatric Surgery, Kurume University School of Medicine, Kurume, Fukuoka, Japan
| | - Hideyuki Sasaki
- Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Masaki Nio
- Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan
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9
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Watanabe S, Suzuki T, Tsuchiya T, Kondo Y. Long-term results of splenomegaly after surgery for biliary atresia in the native liver. Asian J Surg 2021; 45:849-853. [PMID: 34848145 DOI: 10.1016/j.asjsur.2021.07.056] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2021] [Revised: 07/05/2021] [Accepted: 07/27/2021] [Indexed: 11/02/2022] Open
Abstract
BACKGROUND Biliary atresia (BA) is a rare disorder characterized by obstructive jaundice in infants, shortly after birth. Postoperatively, some patients exhibit portal hypertension and progressive liver fibrosis. Splenomegaly is a symptom of portal hypertension. We aimed to investigate splenomegaly as a marker for complications of portal hypertension and the relationship between splenomegaly and liver fibrosis in the long-term native liver (NL). METHODS Between 1977 and 2018, 71 patients underwent hepaticojejunostomy. We included 54 patients (34 NL group, 20 liver transplant (LT) group) who fulfilled the eligibility criteria. Spleen volume (SV), total bile acids, hyaluronic acid, type IV collagen, and aspartate aminotransferase-to-platelet ratio index (APRi) were measured. Data were analyzed using Student's t-test, regression analysis, and receiver operating characteristic (ROC) curve analysis (P < 0.05). RESULTS Total bile acids, hyaluronic acid, type IV collagen, and APRi increased in NL patients with a large SV at >25 years. SV and type IV collagen were correlated with NL for >25 years (r = 0.79 [P = 0.006], y = 1.1 - [0.03 × type IV collagen] [P = 0.008]). In the ROC curve analysis, the cutoff value for type IV collagen was 165 ng/mL (P = 0.07). CONCLUSIONS We suggest that SV as a prognostic index for End-Stage Liver Disease may be useful in biliary atresia. Long-term follow-up is necessary because the clinical course may be favorable in childhood but worsen during adulthood.
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Affiliation(s)
- Shunsuke Watanabe
- Department of Pediatric Surgery, Fujita Health University Hospital, Aichi, Japan; Department of Pediatric Surgery, Fujita Health University Hospital, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi Prefecture, 470-1192, Japan.
| | - Tatsuya Suzuki
- Department of Pediatric Surgery, Fujita Health University Hospital, Aichi, Japan; Department of Pediatric Surgery, Fujita Health University Hospital, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi Prefecture, 470-1192, Japan.
| | - Tomonori Tsuchiya
- Department of Pediatric Surgery, Fujita Health University Hospital, Aichi, Japan; Department of Pediatric Surgery, Fujita Health University Hospital, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi Prefecture, 470-1192, Japan.
| | - Yasuhiro Kondo
- Department of Pediatric Surgery, Fujita Health University Hospital, Aichi, Japan; Department of Pediatric Surgery, Fujita Health University Hospital, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi Prefecture, 470-1192, Japan.
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10
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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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11
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Xiang XL, Cai P, Zhao JG, Zhao HW, Jiang YL, Zhu ML, Wang Q, Zhang RY, Zhu ZW, Chen JL, Gu ZC, Zhu J. Neonatal biliary atresia combined with preduodenal portal vein: A case report. World J Clin Cases 2021; 9:7542-7550. [PMID: 34616824 PMCID: PMC8464463 DOI: 10.12998/wjcc.v9.i25.7542] [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: 02/28/2021] [Revised: 05/28/2021] [Accepted: 07/20/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Congenital biliary atresia is a type of obstruction of the bile ducts inside and outside the liver, which can lead to cholestatic liver cirrhosis and eventually liver failure. The preduodenal portal vein (PD-PV) is a rare developmental malformation of the PV. The PV courses in front of the duodenum. However, very few cases of neonatal biliary atresia combined with PD-PV have been reported in the scientific literature.
CASE SUMMARY A 1-mo-and-4-d-old child was admitted to the hospital in January because of yellowish skin. After surgical consultation, surgical intervention was recommended. The child underwent Hilar-jejunal anastomosis, duodenal rhomboid anastomosis, and abdominal drainage under general anesthesia. During the operation, the PV was located at the anterior edge of the duodenum.
CONCLUSION Diagnoses: (1) Congenital biliary atresia; (2) PD-PV; and (3) Congenital cardiovascular malformations. Outcomes: Recommendation for liver transplantation. Lessons: The choice of treatment options for neonatal biliary atresia combined with PD-PV.
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Affiliation(s)
- Xian-Lan Xiang
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Peng Cai
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Jun-Gang Zhao
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Hao-Wei Zhao
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Yu-Liang Jiang
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Meng-Lei Zhu
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Qi Wang
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Rui-Yun Zhang
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Zhen-Wei Zhu
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Jian-Lei Chen
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Zhi-Cheng Gu
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
| | - Jie Zhu
- Department of Pediatric Surgery, Children’s Hospital of Soochow University, Suzhou 215000, Jiangsu Province, China
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Hukkinen M, Ruuska S, Pihlajoki M, Kyrönlahti A, Pakarinen MP. Long-term outcomes of biliary atresia patients surviving with their native livers. Best Pract Res Clin Gastroenterol 2021; 56-57:101764. [PMID: 35331404 DOI: 10.1016/j.bpg.2021.101764] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/18/2021] [Revised: 08/20/2021] [Accepted: 08/30/2021] [Indexed: 01/31/2023]
Abstract
Portoenterostomy (PE) has remained as the generally accepted first line surgical treatment for biliary atresia (BA) for over 50 years. Currently, close to half of BA patients survive beyond 10 years with their native livers, and most of them reach adulthood without liver transplantation (LT). Despite normalization of serum bilirubin by PE, ductular reaction and portal fibrosis persist in the native liver. The chronic cholangiopathy progresses to cirrhosis, complications of portal hypertension, recurrent cholangitis or hepatobiliary tumors necessitating LT later in life. Other common related health problems include impaired bone health, neuromotor development and quality of life. Only few high-quality trials are available for evidence-based guidance of post-PE adjuvant medical therapy or management of the disease complications. Better understanding of the pathophysiological mechanisms connecting native liver injury to clinical outcomes is critical for development of accurate follow-up tools and novel therapies designed to improve native liver function and survival.
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Affiliation(s)
- Maria Hukkinen
- Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Stenbackinkatu 11 PO Box 281, 00029, HUS, Finland.
| | - Satu Ruuska
- Department of Pediatric Gastroenterology, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Stenbäckinkatu 9/PO BOX 347, 00029, HUS, Finland.
| | - Marjut Pihlajoki
- Pediatric Research Center, Children's Hospital, University of Helsinki, Tukholmankatu 8, 00290, Helsinki, Finland.
| | - Antti Kyrönlahti
- Pediatric Research Center, Children's Hospital, University of Helsinki, Stenbackinkatu 11 PO Box 281, 00029, HUS, Finland.
| | - Mikko P Pakarinen
- Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Stenbackinkatu 11 PO Box 281, 00029, HUS, Finland.
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13
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Chen G, Liu J, Huang Y, Wu Y, Lu X, Dong R, Shen Z, Sun S, Jiang J, Zheng S. Preventive effect of prophylactic intravenous antibiotics against cholangitis in biliary atresia: a randomized controlled trial. Pediatr Surg Int 2021; 37:1089-1097. [PMID: 34013444 DOI: 10.1007/s00383-021-04916-z] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/24/2021] [Indexed: 12/07/2022]
Abstract
OBJECTIVE Biliary atresia (BA) is a neonatal liver disease and requires Kasai portoenterostomy. Many patients develop postoperative cholangitis, resulting in a poor prognosis. The preventive strategy of antibiotics is empirical and lacks a standard regimen. We aimed to analyze the effect of different durations of prophylactic intravenous antibiotics against post-Kasai cholangitis. STUDY DESIGN A single-center, open-labeled, randomized clinical trial was performed from June 2016 to August 2017. One hundred and eighty BA patients were recruited and randomized into a short-term (n = 90) and a long-term (n = 90) treatment group, and prophylactic intravenous antibiotics were used for 7 versus 14 days, respectively. The primary outcome was the overall cholangitis incidence within 6-months post-Kasai portoenterostomy. The secondary outcomes included cholangitis incidence within 1 and 3 months post-Kasai portoenterostomy, the onset and average episodes of cholangitis, jaundice clearance rate, native liver survival rate, and adverse events within 6-months post-Kasai portoenterostomy. RESULTS The cholangitis incidence within 6-months post-Kasai in the short-term group was similar to the long-term group (62% vs. 70%, p = 0.27) with intention-to-treat and pre-protocol analysis. There was no significant difference in jaundice clearance rate or native liver survival rate between the two groups. However, the percentage of early onset (61% vs. 38%, p = 0.02) and average episodes (2.4 ± 0.2 vs. 1.8 ± 0.1 episodes, p = 0.01) of cholangitis were lower in the long-term group. CONCLUSION Long-term intravenous antibiotics can be replaced by the short-term regimen in the general protection against post-Kasai cholangitis.
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Affiliation(s)
- Gong Chen
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Jia Liu
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - YanLei Huang
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Ying Wu
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - XueXin Lu
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Rui Dong
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Zhen Shen
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Song Sun
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Jingying Jiang
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China
| | - Shan Zheng
- Department of Pediatric Surgery, Shanghai Key Laboratory of Birth Defect, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, China.
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Madadi-Sanjani O, Schukfeh N, Uecker M, Eckmann S, Dingemann J, Ure BM, Petersen C, Kuebler JF. The Intestinal Flora at Kasai Procedure in Children with Biliary Atresia Appears Not to Affect Postoperative Cholangitis. Eur J Pediatr Surg 2021; 31:80-85. [PMID: 32820494 DOI: 10.1055/s-0040-1715614] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
INTRODUCTION Evidence supports long-term oral antibiotic prophylaxis to prevent cholangitis after Kasai procedure. Data regarding perioperative intravenous prophylaxis are lacking. Ascending pathogens from the intestine are made responsible for recurrent cholangitis. Therefore, we analyzed the flora in the upper jejunum during the Kasai procedure and their potential impact on postoperative cholangitis. MATERIALS AND METHODS In 26 patients, swabs were taken at the bowel prepared for the Roux-en-Y-loop. Our postoperative protocol includes intravenous third-generation cephalosporins for 2 weeks and rectal steroids starting at day 4. Cholangitis was defined as the postoperative reappearance of acholic stools or increase of serum bilirubin in combination with fevers or increase of inflammatory parameters. In this scenario, Tazocin was administered for another 2 weeks. RESULTS Swabs remained sterile in nine patients (34.6%). In 17 patients (65.4%), gram-positive and gram-negative pathogens were identified; all belonging to physiological intestinal flora. A total of 96.2% pathogens were covered by the antibiotic prophylaxis. The cholangitis incidence was 55.6% in the sterile cohort, and 23.5% in the gram-positive and gram-negative cohort (p = 0.06). In the cholangitis cohort, no significant differences were detected for the age at Kasai and the pre- and postoperative total bilirubin. CONCLUSION We found that our antibiotic regiment covered bacteria in the upper gastrointestinal (GI) tract in the majority of our patients at the time of Kasai. Nonetheless, a significant proportion of patients developed signs of cholangitis. There was no higher rate of cholangitis in patients with resistant bacteria. Thus, our data do not support the hypothesis of extended postoperative intravenous antibiotics to prevent ascending cholangitis.
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Affiliation(s)
| | - Nagoud Schukfeh
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Marie Uecker
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Stefanie Eckmann
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Jens Dingemann
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Benno M Ure
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Claus Petersen
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Joachim F Kuebler
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
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15
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Does the Treatment After Kasai Procedure Influence Biliary Atresia Outcome and Native Liver Survival? J Pediatr Gastroenterol Nutr 2020; 71:446-451. [PMID: 32960536 DOI: 10.1097/mpg.0000000000002837] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVES Biliary atresia (BA) is a rare and progressive idiopathic disease affecting the biliary tract that can lead to end-stage liver disease. The main treatment is Kasai portoenterostomy (KP). The use of adjuvant therapy (AT; prophylactic antibiotics and steroids) after KP aims to prevent cholangitis and reduce the need for liver transplantation (LT), but there is a lack of evidence on their effectiveness. We investigated the impact of significant changes in the post-KP protocol on the overall outcomes of BA. METHODS We enrolled 43 consecutive infants undergoing KP at Bambino Gesù Children's Hospital between July 2012 and October 2018. We compared AT (AT group; n=25) against no treatment (AT-free group; n = 18). RESULTS No significant differences in anthropometric and laboratory parameters were shown between the 2 groups at baseline and every study evaluation (1, 3, and 6 months). The incidences of clinical complications of liver disease were similar. Six months post-KP, the achievement of serum total bilirubin ≤1.5 mg/dL and satisfactory Pediatric End-Stage Liver Disease scores were not significantly different between the 2 groups. Cholangitis was observed in 30% of patients in the first 6 months postoperatively: 33% and 28% in the AT-free and AT groups, respectively (P = 0.18). Survival to LT listing at 12 months and without LT at 24 months were not significantly different between the 2 groups (P > 0.05). CONCLUSIONS AT after KP confirmed conflicting results; therefore, multicentered, prospective, randomized control studies are needed to better understand its utility after KP, especially in the multidrug resistance spread era.
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Long-Term Effects of Kasai Portoenterostomy for Biliary Atresia Treatment in Russia. Diagnostics (Basel) 2020; 10:diagnostics10090686. [PMID: 32932921 PMCID: PMC7555057 DOI: 10.3390/diagnostics10090686] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2020] [Revised: 09/06/2020] [Accepted: 09/08/2020] [Indexed: 12/25/2022] Open
Abstract
This prospective study enrolled 144 patients after surgical treatment of biliary atresia in early infancy. We analyzed the immediate effectiveness of the surgery and the age-related structure of complications in the up to 16-year follow-up. The immediate 2-year survival rate after the surgery constituted 49.5%. At the time of this writing, 17 of the patients had celebrated their 10th birthdays with good quality of life and no indications for transplantation of the liver. The obtained results underscore the critical importance of surgical correction of biliary atresia by Kasai surgery in the first 60 days of life and subsequent dynamic follow-up of patients for the purpose of the early detection and timely correction of possible complications.
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Ryon EL, Parreco JP, Sussman MS, Quiroz HJ, Willobee BA, Perez EA, Sola JE, Thorson CM. Drivers of Hospital Readmission and Early Liver Transplant after Kasai Portoenterostomy. J Surg Res 2020; 256:48-55. [PMID: 32683056 DOI: 10.1016/j.jss.2020.06.019] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2020] [Revised: 05/22/2020] [Accepted: 06/16/2020] [Indexed: 12/28/2022]
Abstract
BACKGROUND Kasai portoenterostomy (KPE) remains the first-line operation for patients with biliary atresia (BA), but ultimately fails in up to 60% of cases. This study sought to identify factors contributing to hospital readmission and early liver transplant. METHODS The Nationwide Readmissions Database from 2010 to 2014 was used to identify patients with BA who underwent KPE on index admission. Patient factors, hospital characteristics, and complications of BA were compared by readmission rates and rate of liver transplant within 1 y. The results were weighted for national estimates. RESULTS Nine hundred and sixty three patients were identified. The readmission rate within 30-d was 36% (n = 346) and within 1-y was 67% (n = 647). Only 9% (n = 90) received a liver transplant within a year. The most common complications after KPE were cholangitis in 58%, decompensated cirrhosis in 54%, and recurrent jaundice in 34%. Male patients (OR 1.5, P = 0.02) with comorbid gastrointestinal anomalies (OR 2.1, P < 0.01) from lower income households (OR 4.6, P < 0.01) and early development of cirrhosis (OR 3.0, P < 0.01) were more likely to be readmitted. Liver transplant was more common in men (OR 4.0, P < 0.01) and those from lower income households (OR 5.2, P < 0.01) with decompensated cirrhosis (OR 8.6, P < 0.01), cholangitis (OR 5.0, P < 0.01), or sepsis (OR 5.7, P < 0.01) on index admission. CONCLUSIONS This is the first nationwide study to evaluate readmissions in patients with BA undergoing KPE. Although KPE is a lifesaving procedure, hospital readmission rates are high and complications are common. Cholangitis, early progression of cirrhosis, and infections are highly associated with readmission and failure of KPE.
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Affiliation(s)
- Emily L Ryon
- Dewitt Daughtry Department of Surgery, University of Miami, Miller School of Medicine, Miami, Florida
| | - Josh P Parreco
- Dewitt Daughtry Department of Surgery, University of Miami, Miller School of Medicine, Miami, Florida
| | - Matthew S Sussman
- Dewitt Daughtry Department of Surgery, University of Miami, Miller School of Medicine, Miami, Florida
| | - Hallie J Quiroz
- Dewitt Daughtry Department of Surgery, University of Miami, Miller School of Medicine, Miami, Florida
| | - Brent A Willobee
- Dewitt Daughtry Department of Surgery, University of Miami, Miller School of Medicine, Miami, Florida
| | - Eduardo A Perez
- Division of Pediatric Surgery, DeWitt Daughtry Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida
| | - Juan E Sola
- Division of Pediatric Surgery, DeWitt Daughtry Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida
| | - Chad M Thorson
- Division of Pediatric Surgery, DeWitt Daughtry Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida.
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18
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Abstract
The treatment of biliary atresia (BA) is predominantly surgical with firstly an attempt at restoration of bile flow from the native liver by wide excision of the obstructed, obliterated extrahepatic biliary tree to the level of the porta hepatis and a portoenterostomy using a long Roux loop-Kasai portoenterostomy (KPE). Liver transplantation is reserved for those that fail this and for those where surgery is considered futile for reasons of age or stage of disease. As the aetiology of BA remains ill-defined, so adjuvant treatment has been largely based on pragmatism, trial and error. Systematic analysis of the few randomized placebo-controlled trial data and less well-controlled cohort studies have suggested benefit from post-operative high-dose steroids and ursodeoxycholic acid (UDCA) while the benefit of long-term prophylactic antibiotics, bile acid sequestrants (e.g., colestyramine) or probiotics remains unproven. Newer modalities such as antiviral therapy (AVT), immunoglobulin, FXR agonists (e.g., obeticholic acid), ileal bile acid transporter (IBAT) antagonists (e.g., maralixibat) remain unproven. This article reviews the current evidence for the efficacy of adjuvant medical therapy in BA.
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Affiliation(s)
- Jessica Burns
- Department of Paediatric Surgery, King's College Hospital, London, UK
| | - Mark Davenport
- Department of Paediatric Surgery, King's College Hospital, London, UK
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19
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Abstract
BACKGROUND Biliary atresia (BA) is the most common cause of obstructive jaundice in infants. Although the Kasai procedure has greatly improved the prognosis, most patients still need liver transplantation (LT) for long-term survival. The pathogenesis of BA has not been fully clarified, and liver fibrosis in BA is far beyond biliary obstructive cirrhosis. DATA SOURCES Literature reviews were underwent through PubMed. Persistent inflammation, immune response, biliary epithelial-mesenchymal transition, matrix deposition, decompensated angiogenesis, and unique biliary structure development all contribute to the fibrosis process. Observed evidences in such fields have been collected and form the backbone of this review. RESULTS Interactions of the multiple pathways accelerate this process. CONCLUSIONS Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.
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Affiliation(s)
- Wen-Jun Shen
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Gong Chen
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Min Wang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China
| | - Shan Zheng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China.
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20
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Biliary Atresia-associated Cholangitis: The Central Role and Effective Management of Bile Lakes. J Pediatr Gastroenterol Nutr 2019; 68:488-494. [PMID: 30628982 DOI: 10.1097/mpg.0000000000002243] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
OBJECTIVES Cholangitis and bile lakes are incompletely understood complications after portoenterostomy (PE). We investigated relationships between recurrent cholangitis, bile lakes, and clinical outcomes as well as surgical management of bile lakes. METHODS In this retrospective observational single institution study medical records and imaging studies of all patients who had undergone PE for biliary atresia during 1987 to 2016 (N = 61) were reviewed. We related occurrence of cholangitis episodes with the presence of intrahepatic bile lakes, patient characteristics, and PE outcomes. Risk factors for recurrent cholangitis and bile lakes, and management of bile lakes were analyzed. RESULTS Despite routine antibiotic prophylaxis median of 3.0 cholangitis episodes (0.75 episodes/year) occurred in 48 (79%) patients. Intrahepatic bile lakes were discovered in 8 (13%) patients by 16 months after PE. Overall, 54% had survived with their native liver at median age of 7.3 years and 28 (46%) patients had ≥1 cholangitis episodes/year. Number and frequency of cholangitis episodes were >5 times higher among patients with bile lakes (P < 0.001). Six patients underwent Roux-en-Y bile lake-jejunostomy, resulting in regression/disappearance of bile lakes and normalization of serum bilirubin in 5 with reduction of median yearly cholangitis rate from 8.8 to 1.1 (P = 0.028) and native liver survival of 6.3 (range, 1.3-17) years after the operation. CONCLUSIONS Bile lakes are a significant risk factor for recurrent cholangitis after PE and efficiently treated by operative intestinal drainage providing prolonged jaundice-free native liver survival. Bile lakes should be actively screened among patients presenting with recurrent cholangitis after PE.
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21
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Ramachandran P, Safwan M, Balaji MS, Unny AK, Akhtarkhavari A, Tamizhvanan V, Rela M. Early Cholangitis after Portoenterostomy in Children with Biliary Atresia. J Indian Assoc Pediatr Surg 2019; 24:185-188. [PMID: 31258267 PMCID: PMC6568148 DOI: 10.4103/jiaps.jiaps_96_18] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Abstract
Aims and Objectives Biliary atresia (BA) is a cholangiodestructive disease of the biliary tree. The first line of treatment is a Kasai portoenterostomy (PE) following which patients may develop cholangitis. We studied the effect of early cholangitis on the outcome of PE, namely jaundice clearance and early native liver survival (NLS). Methods We reviewed the data of all children who developed cholangitis after PE from our prospectively maintained database of children with BA. The standardized treatment of all children in the database is described. The frequency and nature of these episodes were characterized, and the outcome of PE and NLS 1 year after PE was calculated. Results Of 62 children who underwent PE in our institutions, 27 developed cholangitis. All episodes of cholangitis occurred within 14 months of PE. Of 25 children who cleared jaundice in the overall series, 19 had cholangitis. The incidence of cholangitis was significantly higher in children who cleared jaundice. Nine children who had cholangitis are alive with native livers for more than 1 year after PE. Twelve children had intractable cholangitis. Three of these children are alive with native liver 1 year after PE. Conclusion In our series, cholangitis occurred in most children who cleared jaundice. Furthermore, the 1-year NLS of children who developed cholangitis was 33%.
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Affiliation(s)
- Priya Ramachandran
- Department of Pediatric Surgery, CHILDS Trust Medical Research Foundation, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India.,Department of Liver Surgery and Transplantation, Institute of Liver Diseases and Transplantation, Gleneagles Global Health City, Chennai, Tamil Nadu, India
| | - Mohamed Safwan
- Department of Liver Surgery and Transplantation, Institute of Liver Diseases and Transplantation, Gleneagles Global Health City, Chennai, Tamil Nadu, India
| | - Muthukrishnan Saravana Balaji
- Department of Pediatric Surgery, CHILDS Trust Medical Research Foundation, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
| | - Ashitha K Unny
- Department of Pediatric Surgery, CHILDS Trust Medical Research Foundation, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
| | - Anis Akhtarkhavari
- Department of Pediatric Surgery, CHILDS Trust Medical Research Foundation, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
| | - Vidya Tamizhvanan
- Department of Pediatric Surgery, CHILDS Trust Medical Research Foundation, Kanchi Kamakoti Childs Trust Hospital, Chennai, Tamil Nadu, India
| | - Mohamed Rela
- Department of Liver Surgery and Transplantation, Institute of Liver Diseases and Transplantation, Gleneagles Global Health City, Chennai, Tamil Nadu, India
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Hyun JJ, Irani SS, Kozarek RA. Endoscopic retrograde cholangiopancreatography in adult patients with biliary atresia: PROCESS-compliant case series. Medicine (Baltimore) 2018; 97:e0603. [PMID: 29718863 PMCID: PMC6392765 DOI: 10.1097/md.0000000000010603] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2018] [Accepted: 04/06/2018] [Indexed: 11/26/2022] Open
Abstract
INTRODUCTION Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood. METHODS Two jaundiced biliary atresia patients who have lived well beyond 20 years with their native liver after undergoing Kasai portoenterostomy and underwent endoscopic retrograde cholangiopancreatography (ERCP) were identified. The data on patients' clinical information, procedures performed, and outcomes were retrospectively collected by chart review. RESULTS Presence of a long Roux limb and acute angulation from external adhesions along with ductal anomaly from disease itself rendered ERCP challenging, and intraoperative ERCP had to be performed in 1 patient. As enteroscopes had to be used, availability of accessory devices was limited. CONCLUSION Management of adult biliary atresia patients with biliary obstruction with ERCP is feasible, at times, through multidisciplinary means.
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Affiliation(s)
- Jong Jin Hyun
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA
- Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
| | - Shayan S. Irani
- Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA
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A review of long-term outcome and quality of life of patients after Kasai operation surviving with native livers. Pediatr Surg Int 2017; 33:1283-1287. [PMID: 28940041 DOI: 10.1007/s00383-017-4158-4] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/05/2017] [Indexed: 02/07/2023]
Abstract
Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy. These morbidities impose a huge impact on the quality of life of the patients and their families. Herein, we performed a comprehensive review on the clinical status and quality of life of long-term survivors of biliary atresia with their native livers, to facilitate meticulous longitudinal follow-up of these patients, and alert caregivers the probable complications to be aware of.
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Davenport M. Adjuvant therapy in biliary atresia: hopelessly optimistic or potential for change? Pediatr Surg Int 2017; 33:1263-1273. [PMID: 28940004 DOI: 10.1007/s00383-017-4157-5] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/05/2017] [Indexed: 12/17/2022]
Abstract
Given that the aetiology of biliary atresia (BA) is complex and that there is a multiplicity of possible pathogenic mechanisms then it is perhaps not surprising that the evidence for effect of a number of different agents is contradictory. Post-operative cholangitis for instance is common, bacterial in origin and various antibiotic regimens have been tested (although none in a randomized trial) but continuation beyond the early post-operative period does not appear to offer any greater protection. There is an inflammatory reaction in about 25-35% of cases of BA illustrated by abnormal expression of class II antigen and upregulation of ICAM, VCAM and E-selectin with an infiltrate of immune-activated T cells (predominantly CD4 + Th1 and Th17) and NK cells and a systemic surge in inflammatory cytokines (e.g. TNF-α, IL-2, IL-12). This has potential as a therapeutic target and is the main hypothesis behind the rationale use of steroids. The first report of steroids was published in 1985 by Karrer and Lilly as "blast" therapy to treat recalcitrant cholangitis, followed by a multiplicity of small-scale uncontrolled studies suggesting benefit. To date there has been one randomized placebo-controlled study with a low-dose (prednisolone 2 mg/kg/day) regimen (2007); one with a high-dose (IV prednisolone 4 mg/kg/day regimen) (2014); two prospective high-dose open-label studies (2013); a prospective comparison of low- and high-dose regimen and a large (380 infants) retrospective comparison. The most recent meta-analysis (2016) identified a significant difference in clearance of jaundice at 6 months (OR 1.59, 95% CI 1.03-2.45, P = 0.04), in patients treated with high-dose steroids, particularly if < 70 days at surgery. Ursodeoxycholic acid (UDCA) may increase choleresis or change the ratio of endogenous bile acids to a less hydrophobic and, therefore, less toxic millieu. UDCA may protect cholangiocyte membranes against damage and perhaps reduce the tendency to fibrogenesis. Biochemical benefit has been shown in a single crossover trial in older BA children who had cleared their jaundice. Other potential adjuvant therapies include immunoglobulin therapy, anti-viral agents and Chinese herbs although real evidence of benefit is lacking.
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Affiliation(s)
- Mark Davenport
- Department of Paediatric Surgery, King's College Hospital, London, SE5 9RS, UK.
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25
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Abstract
The oldest survivors from the Kasai portoenterostomy originate in Sendai, Japan and are approaching their 60th birthday. These represent the tip of an expanding cohort of adults born with this previously fatal condition. Increasingly transition to adult-biased hepatologists and physicians will be the expectation of many with this condition. However unlike their usual patients with alcohol, drugs, virally mediated liver disease these are different with different expectations of health and quality of life. Cure is not on the cards for most of these and they survive still with impaired bile flow and increased liver fibrosis and cirrhosis with the threat of cholangitis and portal hypertension still apparent. We review the reported statistics on long-term survival essentially from Japan and Western Europe (such as the UK and France) and the range of complications that may still beset this group.
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Affiliation(s)
- Arun Kelay
- Department of Paediatric Surgery, Kings College Hospital, London SE5 9RS
| | - Mark Davenport
- Department of Paediatric Surgery, Kings College Hospital, London SE5 9RS.
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Webb NL, Jiwane A, Ooi CY, Nightinghale S, Adams SE, Krishnan U. Clinical significance of liver histology on outcomes in biliary atresia. J Paediatr Child Health 2017; 53:252-256. [PMID: 27717116 DOI: 10.1111/jpc.13371] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2015] [Revised: 07/18/2016] [Accepted: 08/15/2016] [Indexed: 01/16/2023]
Abstract
AIM Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS Retrospective chart review of all BA cases between 1999 and 2014. Age at KP, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of PHT and ascending cholangitis post-KP were recorded and related to the primary post-KP outcome measures. RESULTS BA was diagnosed in 29 patients. Twenty-four of 29 patients underwent KP. Median age at KP was 68 days (29-104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of KP. Median follow-up was 8.4 years (2.08-15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5-year NLS was 45.8% (11/24). Fourteen of 24 (58.3%) had PHT and 18 of 24 (75%) patients had ascending cholangitis post-KP. Absence of bridging fibrosis in liver histology at KP was the only factor to be significantly associated with improved 5-year NLS. None of the other variables examined had a significant association with either 5-year NLS or bilirubin normalisation by 6 months. CONCLUSION Five-year NLS in this series was 45.8%. Absence of bridging fibrosis at time of KP was the only factor significantly associated with improved 5-year NLS.
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Affiliation(s)
- Nicholas L Webb
- Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia
| | - Ashish Jiwane
- School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.,Department of Pediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia
| | - Chee Y Ooi
- School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.,Department of Gastroenterology, Sydney Children's Hospital, Sydney, New South Wales, Australia
| | - Scott Nightinghale
- Department of Gastroenterology, John Hunter Children's Hospital, Newcastle, New South Wales, Australia.,Discipline of Paediatrics and Child Health, School of Medicine and Public Health, University of Newcastle, Newcastle, New South Wales, Australia
| | - Susan E Adams
- School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.,Department of Pediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia
| | - Usha Krishnan
- School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.,Department of Gastroenterology, Sydney Children's Hospital, Sydney, New South Wales, Australia
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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: 157] [Impact Index Per Article: 19.6] [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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Decharun K, Leys CM, West KW, Finnell SME. Prophylactic Antibiotics for Prevention of Cholangitis in Patients With Biliary Atresia Status Post-Kasai Portoenterostomy: A Systematic Review. Clin Pediatr (Phila) 2016; 55:66-72. [PMID: 26183324 DOI: 10.1177/0009922815594760] [Citation(s) in RCA: 44] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
PURPOSE To determine effectiveness of prophylactic antibiotics in preventing cholangitis, we conducted a systematic review comparing cholangitis occurrence in biliary atresia patients after Kasai portoenterostomy (KP) with and without antibiotics. METHODS We searched online bibliographic databases from April 1, 2013, using search terms "biliary atresia" OR "cholangitis" AND "antibiotics," selecting studies with control group data. RESULTS Four of 509 titles met inclusion criteria, yielding a total of 319 patients from 3 countries. Three studies were retrospective cohorts and one was a randomized clinical trial. Two cohort studies concluded that prophylactic antibiotics reduced incidence of cholangitis and one did not. The randomized clinical trial supported prophylaxis after comparing the prospective randomized groups to a historical control group. CONCLUSION Few published studies measure the effect of prophylactic antibiotics after Kasai portoenterostomy. We identified 4 studies and they presented contradictory results. Prospective research is needed.
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Affiliation(s)
- Katawaetee Decharun
- Indiana University School of Medicine, Indianapolis, IN, USA Riley Hospital for Children at IU Health, Indianapolis, IN, USA Chulalongkorn University, Bangkok, Thailand
| | - Charles M Leys
- Indiana University School of Medicine, Indianapolis, IN, USA Riley Hospital for Children at IU Health, Indianapolis, IN, USA
| | - Karen W West
- Indiana University School of Medicine, Indianapolis, IN, USA Riley Hospital for Children at IU Health, Indianapolis, IN, USA
| | - S Maria E Finnell
- Indiana University School of Medicine, Indianapolis, IN, USA Riley Hospital for Children at IU Health, Indianapolis, IN, USA
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Bessho K. Complications and Quality of Life in Long-Term Survivors of Biliary Atresia with Their Native Livers. J Pediatr 2015; 167:1202-6. [PMID: 26382628 DOI: 10.1016/j.jpeds.2015.08.041] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/14/2015] [Revised: 07/23/2015] [Accepted: 08/20/2015] [Indexed: 12/12/2022]
Affiliation(s)
- Kazuhiko Bessho
- Department of Pediatrics, Graduate School of Medicine, Osaka University, Suita-shi, Osaka, Japan.
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Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation. J Pediatr Gastroenterol Nutr 2015; 60:654-8. [PMID: 25534776 DOI: 10.1097/mpg.0000000000000676] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
OBJECTIVES Recurrent cholangitis may aggravate cholestatic liver cirrhosis in biliary atresia (BA) after the Kasai operation. This pilot study aimed to investigate whether Lactobacillus casei rhamnosus has the prophylactic efficacy for recurrent cholangitis in comparison with the conventional neomycin prophylaxis. METHODS Twenty jaundice-free patients with BA ages 0 to 3 years who underwent a Kasai operation were enrolled and randomized into 2 groups with 10 patients each: neomycin (25 mg · kg · day for 4 days/wk) and L casei rhamnosus (8 × 10 colony-forming unit per day) groups. The treatment duration was 6 months. Bacterial stool cultures were performed before treatment and 1, 3, and 6 months after starting treatment. In addition, 10 patients with BA with similar status but without prophylaxis served as the historical control group. RESULTS In the Lactobacillus group, 2 patients (20%, mean 0.03 ± 0.07 episodes per month) developed cholangitis during the study period, with the same frequency as in the neomycin group and significantly lower than that in the control group (80%, P = 0.005, mean 0.22 ± 0.16 episodes per month). The mean change in body weight z score during the 6 months in the Lactobacillus group was 0.97 ± 0.59, which was significantly better than that in the control group (-0.01 ± 0.79, P = 0.006). In bacterial stool cultures, the Lactobacillus and Escherichia coli populations significantly increased and decreased, respectively, in the Lactobacillus group. CONCLUSIONS The use of L casei rhamnosus was as effective as neomycin in preventing cholangitis in patients with BA who underwent Kasai operation, and therefore could be considered as a potential alternative prophylactic regimen.
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Thakur RK, Davenport M. Improving treatment outcomes in patients with biliary atresia. Expert Opin Orphan Drugs 2014. [DOI: 10.1517/21678707.2014.973402] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
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Ng VL, Haber BH, Magee JC, Miethke A, Murray KF, Michail S, Karpen SJ, Kerkar N, Molleston JP, Romero R, Rosenthal P, Schwarz KB, Shneider BL, Turmelle YP, Alonso EM, Sherker AH, Sokol RJ. Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium. J Pediatr 2014; 165:539-546.e2. [PMID: 25015575 PMCID: PMC4144331 DOI: 10.1016/j.jpeds.2014.05.038] [Citation(s) in RCA: 55] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/03/2014] [Revised: 04/21/2014] [Accepted: 05/21/2014] [Indexed: 12/23/2022]
Abstract
OBJECTIVES To examine the medical status of children with biliary atresia (BA) with their native livers after hepato- portoenterostomy (HPE) surgery. STUDY DESIGN The Childhood Liver Disease Research and Education Network database was utilized to examine subjects with BA living with their native livers 5 or more years after HPE and to describe the prevalence of subjects with BA with an "ideal" outcome, defined as no clinical evidence of chronic liver disease, normal liver biochemical indices (aspartate aminotransferase, alanine aminotransferase, γ-glutamyl transpeptidase, platelet count, total bilirubin, international normalized ratio, and albumin), and normal health-related quality of life 5 or more years after HPE. RESULTS Children with BA (n = 219; 43% male) with median age 9.7 years were studied. Median age at HPE was 56 (range 7-125) days. Median age- and sex-adjusted height and weight z-scores at 5-year follow-up were 0.487 (IQR -0.27 to 1.02) and 0.00 (IQR -0.74 to 0.70), respectively. During the 12 preceding months, cholangitis and bone fractures occurred in 17% and 5.5%, respectively. Health-related quality of life was reported normal by 53% of patients. However, only 1.8% met the study definition of "ideal" outcome. Individual tests of liver synthetic function (total bilirubin, albumin, and international normalized ratio) were normal in 75%, 85%, and 73% of the study cohort. CONCLUSION Cholangitis and fractures in long-term survivors underscore the importance of ongoing medical surveillance. Over 98% of this North American cohort of subjects with BA living with native livers 5 or more years after HPE have clinical or biochemical evidence of chronic liver disease.
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Affiliation(s)
- Vicky Lee Ng
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
| | - Barbara H Haber
- Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA
| | - John C Magee
- Department of Surgery, University of Michigan Medical School, Ann Arbor, MI
| | - Alexander Miethke
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
| | - Karen F Murray
- Hepatobiliary Program, Seattle Children's Hospital and University of Washington, Seattle, WA
| | - Sonia Michail
- Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA
| | - Saul J Karpen
- Texas Children's Hospital, Houston, TX; Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA
| | - Nanda Kerkar
- Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Keck School of Medicine University of Southern California, Los Angeles, CA; Division of Pediatric Hepatology, Mount Sinai School of Medicine, New York, NY
| | - Jean P Molleston
- Pediatric Gastroenterology, Hepatology, and Nutrition, Riley Hospital for Children, Indiana University, Indianapolis, IN
| | - Rene Romero
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Healthcare of Atlanta, Emory University, Atlanta, GA
| | - Philip Rosenthal
- Pediatrics Gastroenterology, Hepatology, and Nutrition, University of California San Francisco Benioff Children's Hospital, University of California, San Francisco, CA
| | - Kathleen B Schwarz
- Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Medical Institutions, Baltimore, MD
| | - Benjamin L Shneider
- Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's Hospital Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA
| | - Yumirle P Turmelle
- Division of Gastroenterology and Nutrition, Washington University, St. Louis, MO
| | - Estella M Alonso
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Ann and Robert H. Lurie Children's Hospital and Northwestern University, Chicago, IL
| | | | - Ronald J Sokol
- Department of Gastroenterology, Hepatology, and Nutrition, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO
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Tessier MEM, Harpavat S, Shepherd RW, Hiremath GS, Brandt ML, Fisher A, Goss JA. Beyond the Pediatric end-stage liver disease system: Solutions for infants with biliary atresia requiring liver transplant. World J Gastroenterol 2014; 20:11062-11068. [PMID: 25170195 PMCID: PMC4145749 DOI: 10.3748/wjg.v20.i32.11062] [Citation(s) in RCA: 15] [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: 02/13/2014] [Revised: 05/13/2014] [Accepted: 06/23/2014] [Indexed: 02/06/2023] Open
Abstract
Biliary atresia (BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches: (1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and (2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.
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Squires RH, Ng V, Romero R, Ekong U, Hardikar W, Emre S, Mazariegos GV. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Hepatology 2014; 60:362-98. [PMID: 24782219 DOI: 10.1002/hep.27191] [Citation(s) in RCA: 142] [Impact Index Per Article: 12.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2014] [Accepted: 04/22/2014] [Indexed: 12/16/2022]
Affiliation(s)
- Robert H Squires
- Department of Pediatrics, University of Pittsburgh School of Medicine; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA
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Adherence of Randomized Trials Within Children's Surgical Specialties Published During 2000 to 2009 to Standard Reporting Guidelines. J Am Coll Surg 2013; 217:394-399.e7. [DOI: 10.1016/j.jamcollsurg.2013.03.032] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2012] [Revised: 03/02/2013] [Accepted: 03/05/2013] [Indexed: 02/07/2023]
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Wildhaber BE. Biliary atresia: 50 years after the first kasai. ISRN SURGERY 2012; 2012:132089. [PMID: 23304557 PMCID: PMC3523408 DOI: 10.5402/2012/132089] [Citation(s) in RCA: 71] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/23/2012] [Accepted: 10/30/2012] [Indexed: 12/20/2022]
Abstract
Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a "Kasai," modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.
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Affiliation(s)
- Barbara E Wildhaber
- Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Geneva, 1211 Geneva, Switzerland
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Wu JF, Kao PC, Chen HL, Lai HS, Hsu HY, Chang MH, Ni YH. A high serum interleukin-12p40 level prior to Kasai surgery predict a favourable outcome in children with biliary atresia. Liver Int 2012; 32:1557-63. [PMID: 22958268 DOI: 10.1111/liv.12001] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/13/2012] [Accepted: 07/31/2012] [Indexed: 02/13/2023]
Abstract
BACKGROUND Biliary atresia (BA) is a paediatric cholestatic disease characterized by a progressive fibro-inflammation of the biliary tree. Current treatment of choice is to establish good bile flow via the Kasai operation. AIMS We aimed to identify outcome-predictive serum biomarkers in BA infant. METHODS Thirty-three BA children recruited from 1986 to 2007 served as the baseline-study group. An additional 11 children recruited from 2008 to 2011 served as the validation group. Serum samples were collected immediately before and 6 months after the Kasai operation for the assessment of serum cytokines, including tumour necrosis factor-α (TNF-α), transforming growth factor-β (TGF-β), interferon-γ (IFN-γ), interleukin-2 (IL-2), IL-10, IL-12p40 and IL-12p70 as the candidate biomarkers. RESULTS Increased serum TGF-β levels indicated a lower Knodell hepatitis activity index at Kasai operation. The serum TGF-β levels declined after the operation. Serum IL-12p40 levels before the Kasai operation were higher in the subjects with a 3-month jaundice-free status than in others (P = 0.001). A serum pre-operative IL-12p40 level of 33 pg/ml was predictive of a 3-month jaundice-free status after surgery (positive predictive value=81.0%; negative predictive value=83.3%). This biomarker was also predictive of a better outcome, in terms of 3-year survival with native liver (risk ratio [RR = 4.00]; P < 0.001), and 3-year jaundice-free survival with native liver (RR = 12.00; P < 0.001). We confirmed the predictive power of a high pre-operative IL-12p40 level on 3-month jaundice-free status in the validation group. CONCLUSIONS The pre-operative IL-12p40 level was a good predictive biomarker of clinical outcome in children with BA undergoing the Kasai operation.
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Affiliation(s)
- Jia-Feng Wu
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
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Nordin N, Alex G, Clarnette T, Stephens N, Oliver M. Common bile duct stones in infancy: a medical approach. J Paediatr Child Health 2012; 48:705-9. [PMID: 22497643 DOI: 10.1111/j.1440-1754.2012.02452.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
Symptomatic choledocholithiasis in infancy is not common. It usually presents with jaundice and acholic stools and is diagnosed on abdominal ultrasonography. Favourable outcome of conservative management has been reported, but specific management guidelines are not well defined in the literature. We describe three cases using a combination of ursodeoxycholic acid and antibiotics as a treatment paradigm, which could potentially negate more invasive treatment. All three patients had ultrasonography proven choledocholithiasis with concomitant obstructive liver function test. They were treated with a combination of ursodeoxycolic acid and antibiotics. Patient 1 had an Escherichia coli urinary tract infection and was treated with oral bactrim. Intravenous amoxicillin, gentamicin and metronidazole were used for the other two patients. All three patients responded with a return to normal-coloured stools within 48 h of combination treatment. Repeat ultrasonography done within 11 days after the first study for all three patients confirmed complete resolution of choledocholithiasis. It is postulated that this improvement is as a result of a reduction in inflammation and oedema, associated with a low-grade cholangitis, following antibiotic treatment. This is coupled with improved bile flow with ursodeoxycholic acid therapy. The findings suggest the potential application of this safe, non-invasive therapeutic strategy as initial management in infants with this condition. A follow-up prospective randomised controlled trial may be an answer to prove the validity of this observation but due to the rarity of this problem, it would be a challenge to recruit sufficient number of patients.
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Affiliation(s)
- Nazrul Nordin
- Departments of Gastroenterology and Clinical Nutrition General Surgery Medical Imaging, Royal Children's Hospital, Flemington Road, Melbourne, VIC 3052, Australia.
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Orman ES, Miller CB, Grimm IS, Barritt AS. Single-balloon enteroscopy-assisted endoscopic retrograde cholangiopancreatography for treatment of cholangitis in a patient with a Kasai portoenterostomy. J Pediatr Surg 2012; 47:E1-5. [PMID: 22595601 PMCID: PMC3356930 DOI: 10.1016/j.jpedsurg.2011.12.017] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2011] [Revised: 12/09/2011] [Accepted: 12/09/2011] [Indexed: 10/28/2022]
Abstract
Primary therapy for biliary atresia is a surgical hepatoportoenterostomy (Kasai procedure), which has been shown to reduce mortality, but is frequently complicated by ascending cholangitis and the development of biliary cirrhosis. Previously reported therapy for recurrent cholangitis caused by biliary obstruction has included surgical revision and percutaneous biliary drainage, but endoscopic retrograde cholangiopancreatography has not been previously described. Here, we report a patient with recurrent cholangitis after a Kasai procedure and an anastomotic stricture successfully treated with single-balloon enteroscopy-assisted endoscopic retrograde cholangiopancreatography. This novel technique could be considered in patients with this common complication of the Kasai procedure and may impact long-term outcomes in this patient population.
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Affiliation(s)
- Eric S. Orman
- Department of Medicine, Division of Gastroenterology and Hepatology, University of North Carolina, Chapel Hill, NC
| | - C. Brock Miller
- Department of Medicine, Division of Gastroenterology and Hepatology, University of North Carolina, Chapel Hill, NC
| | - Ian S. Grimm
- Department of Medicine, Division of Gastroenterology and Hepatology, University of North Carolina, Chapel Hill, NC
| | - A. Sidney Barritt
- Department of Medicine, Division of Gastroenterology and Hepatology, University of North Carolina, Chapel Hill, NC
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de Vries W, de Langen ZJ, Groen H, Scheenstra R, Peeters PMJG, Hulscher JBF, Verkade HJ. Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008. J Pediatr 2012; 160:638-644.e2. [PMID: 22082947 DOI: 10.1016/j.jpeds.2011.09.061] [Citation(s) in RCA: 84] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2011] [Revised: 08/26/2011] [Accepted: 09/28/2011] [Indexed: 12/22/2022]
Abstract
OBJECTIVE To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database. STUDY DESIGN All children born between January 1987 and December 2008 who underwent the Kasai surgical procedure for BA were retrieved from the Netherlands Study Group on Biliary Atresia Registry database. Outcomes were measured in terms of clearance of jaundice (bilirubin <1.17 g/dL, or 20 μmol/L, within 6 months after surgery) and 4-year transplant-free survival. Two cohorts, one from 1987-1997 and the other from 1998-2008, were compared. Survival rates were determined using Kaplan-Meier analysis, and prognostic factors were tested with univariate and multivariate analyses. RESULTS Between January 1987 and December 2008, 214 patients underwent Kasai surgery for BA. In this series, the 4-year transplant-free survival was 46%±4%, and 4-year overall survival was 73%±3%. Clearance of jaundice, surgery within 60 days, and postoperative antibiotic prophylaxis use were independently associated with increased transplant-free survival. The yearly caseload per center (range, 0.5-2.1) was not correlated with transplant-free survival (r=0.024; P=.73). CONCLUSION During the past 2 decades, outcome parameters have remained constant and are comparable with those reported from other Western countries, despite a relatively low annual caseload per center. Timely surgical correction and postoperative antibiotic therapy were associated with a higher transplant-free survival rate.
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Affiliation(s)
- Willemien de Vries
- Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
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Abstract
Primary sclerosing cholangitis (PSC) is a chronic cholestatic condition affecting the extrahepatic and intrahepatic biliary tree. The incidence is seemingly increasing in children as a result of increased use of cholangiographic screening techniques in children with inflammatory bowel disease. The clinical presentation of PSC in childhood is variable and frequently without obvious cholestatic features, and cholangiography is essential for the diagnosis of this disorder. Histologic findings may help to exclude autoimmune sclerosing cholangitis. The underlying pathogenesis remains poorly understood and, as a result, therapeutic agents that halt disease progression and improve prognosis are lacking. PSC treatment is mainly supportive and directed at controlling cholestatic symptoms and preventing complications. Ursodeoxycholic acid is helpful in inducing biochemical improvement; long-term pediatric studies to determine a benefit of this agent in young patients are lacking, although results from adult studies have not been promising. Some agents such as antibiotics are under investigation with some promising results. Liver transplantation is required for children who progress to end-stage liver disease. Prospective multicenter trials in children with PSC are needed.
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Affiliation(s)
- Samar H Ibrahim
- Department of Pediatrics, Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
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Nydert P, Lindemalm S, Nemeth A. Off-label drug use evaluation in paediatrics--applied to ciprofloxacin when used as treatment of cholangitis. Pharmacoepidemiol Drug Saf 2011; 20:393-8. [PMID: 21280136 DOI: 10.1002/pds.2080] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2010] [Revised: 11/02/2010] [Accepted: 11/05/2010] [Indexed: 11/05/2022]
Abstract
PURPOSE To evaluate drug and indication specific off-label use in paediatrics, applied to ciprofloxacin (CPFX) in cholangitis. METHODS We collected four different sets of data for an off-label drug use evaluation. (1) Literature review from medical journals, (2) the use and safety profile from the whole Swedish paediatric population by extracting data from national registers, (3) locally performed retrospective drug chart reviews, and (4) interviews regarding paediatric patients with CPFX treated cholangitis. RESULTS The literature reviews show a lack of information for paediatric use of CPFX in cholangitis. The prescribing of CPFX to Swedish children has grown over the last decade and generated a small number of reports for adverse drug reactions. In our local biliary atresia population 32 patients had suffered from at least one episode of cholangitis and 13 patients had been prescribed CPFX. The dosing strategy had an empirical prescribing approach, since monitoring of bacterial resistance and efficacy is difficult in the biliary ducts. No clear relationship was seen between dosing and age/weight. Reports of suspected side effects could not be found in the retrospective chart reviews. The interviews show that the existing dosage forms are well accepted. CONCLUSIONS This drug use evaluation creates awareness of the off-label situation. The international and national data are sparse for the paediatric use of CPFX in cholangitis. Locally we have highlighted a heterogeneous dosing strategy of CPFX, drug/drug interactions, and the need to monitor and report the risk of short- and long-term adverse drug reactions.
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Affiliation(s)
- Per Nydert
- Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden.
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Lien TH, Chang MH, Wu JF, Chen HL, Lee HC, Chen AC, Tiao MM, Wu TC, Yang YJ, Lin CC, Lai MW, Hsu HY, Ni YH. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology 2011; 53:202-8. [PMID: 21140377 DOI: 10.1002/hep.24023] [Citation(s) in RCA: 128] [Impact Index Per Article: 9.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/04/2010] [Accepted: 09/22/2010] [Indexed: 12/11/2022]
Abstract
UNLABELLED In Taiwan, a screening system using an infant stool color card to promote the early diagnosis of biliary atresia (BA) was established in 2002. This study aimed to investigate the 5-year outcome of BA before and after using the screening program. BA patients were divided into three cohorts according to their birth dates. The patients in cohort A (n = 89) were born before the stool card screening program (1990-2000); those in cohort B (n = 28) were screened by the stool card regional screening program (2002-2003); and those in cohort C (n = 74) were screened by the stool card universal screening program (2004-2005). The relative odds ratios were computed using logistic regression to compare the different factors affecting survival time. The rate of age at Kasai operation <60 days was 49.4% and 65.7% in cohorts A and B+C, respectively (P = 0.02). The jaundice-free (total serum bilirubin <2.0 mg/dL) rate 3 months after surgery was 34.8% and 60.8% in cohorts A and B+C, respectively (P < 0.001). The 3-year jaundice-free survival rate with native liver was 31.5% in cohort A and 56.9% in cohort B+C (P < 0.001), whereas the 3-year overall survival rates were 64.0% and 89.2%, respectively (P < 0.001). The 5-year jaundice-free survival rate with native liver was 27.3% in cohort A and 64.3% in cohort B (P < 0.001), and the 5-year overall survival rates were 55.7% and 89.3%, respectively (P < 0.001). CONCLUSION The stool color card screening program for BA allows for earlier Kasai operation, which increases the jaundice-free rate at 3 months postsurgery. With higher surgical success rates, the 3- and 5-year outcome of BA patients in Taiwan improves remarkably.
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Affiliation(s)
- Tien-Hau Lien
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
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Lao OB, Larison C, Garrison M, Healey PJ, Goldin AB. Steroid use after the Kasai procedure for biliary atresia. Am J Surg 2010; 199:680-4. [PMID: 20466116 DOI: 10.1016/j.amjsurg.2010.01.014] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2009] [Revised: 01/19/2010] [Accepted: 01/19/2010] [Indexed: 11/29/2022]
Abstract
BACKGROUND The aim of this study was to describe patients undergoing the Kasai procedure at children's hospitals, evaluate outcomes, and analyze the association of these outcomes with systemic steroid use. METHODS Biliary atresia patients (International Classification of Diseases, Ninth Revision, code 751.61) who underwent Kasai procedures at freestanding children's hospitals in the Pediatric Health Information System database from 2003 to 2008 were identified. Descriptive characteristics were examined, and regression analyses were used to determine whether postoperative steroid use was associated with length of stay, mortality, or cholangitis. RESULTS Of the 516 children identified (40% male, 50% aged < 2 months), 239 (46%) received perioperative steroids. The mean total and postoperative lengths of stay were 14.5 +/- 19.7 and 11.3 +/- 16.3 days, respectively. Postoperative steroid use was significantly associated with a 3.5-day decrease in postoperative length of stay (95% confidence interval, .03-6.97). CONCLUSIONS Perioperative steroids after the Kasai procedure are associated with shorter postoperative length of stay. Work is needed to ascertain whether this relationship is causal.
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Affiliation(s)
- Oliver B Lao
- Department of Surgery, Seattle Children's Hospital, Seattle, WA, USA.
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Erlichman J, Hohlweg K, Haber BA. Biliary atresia: how medical complications and therapies impact outcome. Expert Rev Gastroenterol Hepatol 2009; 3:425-34. [PMID: 19673629 DOI: 10.1586/egh.09.30] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. Untreated, BA is a uniformly fatal disease and, yet, even with our existing therapies, at least 50% of children with BA will undergo liver transplantation by the age of 2 years. Current treatment strategies are, at best, palliative; they focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. The purpose of this article is to discuss the current treatment paradigm for BA and to assess the impact these strategies have on outcomes. As more children with BA survive into adulthood with their native liver, it is important to understand which factors predict good and poor outcomes.
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Affiliation(s)
- Jessi Erlichman
- Division of GI, Hepatology and Nutrition, The Childrens' Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, USA
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Haber BA, Erlichman J, Loomes KM. Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs 2009; 17:1911-24. [PMID: 19012506 DOI: 10.1517/13543780802514120] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
BACKGROUND Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction before 2 months of age. Untreated BA is a uniformly fatal disease and even with our current therapies only 50% of children with BA will be transplant-free by 2 years of age. Despite descriptions of this disorder dating back to the 1800s our current therapies are palliative. They focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. OBJECTIVE To present the literature supporting current treatment strategies and potential future therapies. METHOD Each of the aspects of care is described and the literature about nuances of care is provided. CONCLUSION Therapies will not improve outcomes until novel treatments are introduced, such as those suggested, which may intervene in the inflammatory or fibrotic steps of the disease process.
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Affiliation(s)
- Barbara A Haber
- Associate Professor of Pediatrics The Childrens Hospital of Philadelphia, Division of GI, Hepatology & Nutrition, Philadelphia, PA 19104, USA.
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Abstract
BACKGROUND Postoperative cholangitis characterized by fever and acholic stool and positive blood culture is a common and serious complication following Kasai's operation for biliary atresia. The aim of this review was to describe the pathogenesis, clinical manifestations, medical treatment and outcome of postoperative cholangitis. DATA SOURCES Articles on biliary atresia retrieved from Pubmed and MEDLINE in the recent 10 years were reviewed. RESULTS The pathogenesis of postoperative cholangitis is still controversial. Recent methods for the diagnosis of postoperative cholangitis include urinary sulfated bile acids (USBA) and magnetic resonance cholangio-pancreaticography (MRCP). High-dose steroids and oral antibiotics have been used to reduce the incidence of postoperative cholangitis, and recurrent cholangitis leads to a lower survival rate. CONCLUSIONS Cholangitis is one of the most important determinants of long-term survival after the Kasai's procedure. The knowledge on postoperative cholangitis has been increasing in the past 10 years, showing a lower incidence of the disease and better therapeutic results.
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Affiliation(s)
- Yi Luo
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai 200032, China
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Ling SC. Congenital cholestatic syndromes: what happens when children grow up? CANADIAN JOURNAL OF GASTROENTEROLOGY = JOURNAL CANADIEN DE GASTROENTEROLOGIE 2007; 21:743-51. [PMID: 18026579 PMCID: PMC2658590 DOI: 10.1155/2007/581247] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/13/2006] [Accepted: 01/22/2007] [Indexed: 12/18/2022]
Abstract
Although advances in the management of children with congenital cholestasis have enabled many to survive into adulthood with their native livers, even the most common of these conditions remains rare in adult hepatology practice. Among four congenital cholestatic syndromes (biliary atresia, Alagille syndrome, Caroli disease and congenital hepatic fibrosis, and progressive familial intrahepatic cholestasis), the published data on outcomes of the syndromes into adulthood suggest that a spectrum of severity of liver disease can be expected, from cirrhosis (almost universal in adults with biliary atresia who have not required liver transplantation) to mild and subclinical (eg, in the previously undiagnosed affected parent of an infant with Alagille syndrome). Complications associated with portal hypertension and nutritional deficiencies are common, and other associated features of the cholestatic syndrome may require appropriate attention, such as congenital heart disease in Alagille syndrome. Indications for liver transplantation include synthetic failure, progressive encephalopathy, intractable pruritus, recurrent biliary sepsis and recurrent complications of portal hypertension. Improved understanding of biliary physiology will hopefully translate into improved therapy for children and adults with cholestasis.
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Affiliation(s)
- S C Ling
- Department of Gastroenterolgy, The Hospital for Sick Children, Toronto, Canada.
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Affiliation(s)
- Benjamin L Shneider
- Department of Pediatrics, Thomas E. Starzl Transplantation Institute, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA.
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