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Madadi-Sanjani O, Herden U, Uecker M. [Kasai-hepatoportoenterostomy for the treatment of biliary atresia - What is important?]. CHIRURGIE (HEIDELBERG, GERMANY) 2025; 96:474-481. [PMID: 40016476 PMCID: PMC12098517 DOI: 10.1007/s00104-025-02259-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Accepted: 01/31/2025] [Indexed: 03/01/2025]
Abstract
Biliary atresia (BA) is a rare disease in neonates of unknown etiology. BA is defined by the extent of extra- and intrahepatic bile duct destruction, which results in liver deterioration and cirrhosis within the first years of life. Liver transplantation (LT) is the only curative treatment for BA, accompanied by LT-associated risks and complications; however, more than 60 years after it's first report, the Kasai hepatoportoenterostomy (KPE) is still an essential procedure in the sequential management of BA, as the primary surgical treatment option that can achieve long-term survival with a native liver. We highlight the key surgical steps of KPE and discuss relevant aspects.
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Affiliation(s)
- Omid Madadi-Sanjani
- Klinik für Viszerale Transplantationschirurgie, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland.
| | - Uta Herden
- Klinik für Viszerale Transplantationschirurgie, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
| | - Marie Uecker
- Klinik für Kinderchirurgie, Medizinische Hochschule Hannover, Hannover, Deutschland
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Okubo R, Nio M, Sasaki H, Wada M. Japanese biliary atresia registry. WORLD JOURNAL OF PEDIATRIC SURGERY 2025; 8:e001024. [PMID: 40433107 PMCID: PMC12107581 DOI: 10.1136/wjps-2025-001024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2025] [Accepted: 04/29/2025] [Indexed: 05/29/2025] Open
Abstract
The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS) to investigate the epidemiology and etiology of biliary atresia and to improve surgical outcomes. The JBAR collects data through initial, liver transplantation, and follow-up questionnaires. Pediatric surgeons from JBAS member institutions and hospitals affiliated with the Japanese Society of Pediatric Surgeons are responsible for registering patients and submitting data through an online system. Each patient is to be followed up for 40 years. As of 2023, 3951 patients had been registered, with 1688 undergoing liver transplantation. The native liver survival rates in the 10th, 20th, and 30th year surveys were 50.5%, 44.4%, and 40.9%, respectively. The overall survival rates in the 10th, 20th, and 30th year surveys were 88.9%, 87.6%, and 85.7%, respectively. The surgical outcome of biliary atresia has markedly improved owing to the cooperation between Kasai portoenterostomy and liver transplantation. A comprehensive Japanese database of patients with biliary atresia (https://jbas.net/en/national-registration/) is now available.
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Affiliation(s)
- Ryuji Okubo
- Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
- Japanese Biliary Atresia Society, Sendai, Japan
| | - Masaki Nio
- Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
- Japanese Biliary Atresia Society, Sendai, Japan
- KKR Tohoku Kosai Hospital, Sendai, Japan
| | - Hideyuki Sasaki
- Japanese Biliary Atresia Society, Sendai, Japan
- Department of Surgery, Miyagi Children’s Hospital, Sendai, Japan
| | - Motoshi Wada
- Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
- Japanese Biliary Atresia Society, Sendai, Japan
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Guthrie G, Vonderohe C, Meléndez Hebib V, Stoll B, Burrin D. Multicomponent parenteral lipid emulsions do not prevent liver injury in neonatal pigs with obstructive cholestasis. JCI Insight 2025; 10:e189196. [PMID: 40244694 DOI: 10.1172/jci.insight.189196] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2024] [Accepted: 04/10/2025] [Indexed: 04/19/2025] Open
Abstract
Biliary atresia (BA) is a pediatric liver disease that often necessitates parenteral nutrition (PN) to support growth due to impaired liver function. While soy-based lipid emulsions (SLE) are commonly used in PN, they may contribute to cholestatic liver injury. In contrast, mixed lipid emulsions (MLE) show promise in preventing cholestasis in infants without BA, potentially by restoring bile flow. However, their effectiveness in patients of complete bile duct obstruction, as seen in BA, remains uncertain. To explore the potential benefits of MLE in BA, we utilized a neonatal pig model of bile duct ligation (BDL). Pigs underwent either BDL or sham surgery and were subsequently fed either MLE or SLE via PN, or enterally with formula. The MLE-BDL pigs exhibited significantly greater weight gain compared with those fed SLE or formula enterally. Additionally, MLE-BDL pigs showed higher serum bile acid and γ-glutamyl transferase concentrations compared with SLE-BDL pigs. However, no significant differences in liver injury, assessed by ductular reaction or fibrosis, were observed between MLE- and SLE-BDL pigs. Based on weight gain alone, MLE may be a superior lipid emulsion for use in neonates with obstructive cholestasis.
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Affiliation(s)
- Greg Guthrie
- USDA-ARS Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, USA
| | - Caitlin Vonderohe
- USDA-ARS Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, USA
| | - Valeria Meléndez Hebib
- USDA-ARS Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, USA
| | - Barbara Stoll
- USDA-ARS Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, USA
| | - Douglas Burrin
- USDA-ARS Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, USA
- USDA-ARS Children's Nutrition Research Center, Department of Pediatrics, Section Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine, Houston, USA
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Davenport M, Makin E, Ong EG, Sharif K, Dawrant M, Alizai N. The Outcome of a Centralization Program in Biliary Atresia: Twenty Years and Beyond. Ann Surg 2025; 281:608-614. [PMID: 38506040 DOI: 10.1097/sla.0000000000006273] [Citation(s) in RCA: 9] [Impact Index Per Article: 9.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/21/2024]
Abstract
OBJECTIVE Biliary atresia is a rare disease and reported outcomes of surgical management, typically a Kasai portoenterostomy (KPE), vary considerably across the world. Centralization has been proposed to improve this. BACKGROUND A national centralization program was started in January 1999, involving 3 English units with colocated liver transplant facilities. As the program has now reached the 20-year point, the main aim was to update outcome statistics and identify trends. METHODS Prospective registry and database. The main measures of outcome were (1) time to KPE, (2) clearance of jaundice (CoJ), defined as reaching a bilirubin value of <20 µmol/L (≈1.5 mg/dL), and (3) actuarial native liver survival and overall survival (OS). Data are quoted as median (interquartile range) and nonparametric statistical comparison used with P <0.05 regarded as statistically significant. RESULTS A total of 867 infants were born with biliary atresia and managed between January 1999 and December 2019. Death occurred without intervention (n = 10, 1.1%) or were subject to primary transplant (n = 26, 3.0%); leaving 831 (95.9%) infants who underwent KPE at a median age of 51 (interquartile range: 39-64) days. Age at KPE reduced over the period ( P = 0.0001) becoming 48 (35-57) days in the last 5-year era. CoJ was achieved in 505/831 (60.6%), also increasing over the period ( P = 0.002). Forty-two (5.0%) died post-KPE and 384 were transplanted, leaving 405 alive with their native livers at the last follow-up. Of the 412 children transplanted, there were 23 (5.6%) deaths, leaving 387 alive. Five-year and 10-year native liver survivals were 51.3% (95% CI: 54.8-47.8) and 46.5% (95% CI: 50.1-42.9) and OSs were 91.5% (95% CI 93.2-89.4) and 90.5% (95% CI 92.3-88.2%), respectively. CONCLUSIONS There have been continued improvements in efficiency over the period of centralization with a significant reduction in time to KPE and improved CoJ after KPE. OS in this disease remains >90%.
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Ma Y, Yang Y, Du Y, Jin L, Liang B, Zhang Y, Wang Y, Liu L, Zhang Z, Jin Z, Qiu Z, Ye M, Wang Z, Tong C. Development of an artificial intelligence-based multimodal diagnostic system for early detection of biliary atresia. BMC Med 2025; 23:127. [PMID: 40016769 PMCID: PMC11866655 DOI: 10.1186/s12916-025-03962-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2024] [Accepted: 02/20/2025] [Indexed: 03/01/2025] Open
Abstract
BACKGROUND Early diagnosis of biliary atresia (BA) is crucial for improving patient outcomes, yet remains a significant global challenge. This challenge may be ameliorated through the application of artificial intelligence (AI). Despite the promise of AI in medical diagnostics, its application to multimodal BA data has not yet achieved substantial breakthroughs. This study aims to leverage diverse data sources and formats to develop an intelligent diagnostic system for BA. METHODS We constructed the largest known multimodal BA dataset, comprising ultrasound images, clinical data, and laboratory results. Using this dataset, we developed a novel deep learning model and simplified it using easily obtainable data, eliminating the need for blood samples. The models were externally validated in a prospective study. We compared the performance of our model with human experts of varying expertise levels and evaluated the AI system's potential to enhance its diagnostic accuracy. RESULTS The retrospective study included 1579 participants. The multimodal model achieved an AUC of 0.9870 on the internal test set, outperforming human experts. The simplified model yielded an AUC of 0.9799. In the prospective study's external test set of 171 cases, the multimodal model achieved an AUC of 0.9740, comparable to that of a radiologist with over 10 years of experience (AUC = 0.9766). For less experienced radiologists, the AI-assisted diagnostic AUC improved from 0.6667 to 0.9006. CONCLUSIONS This AI-based screening application effectively facilitates early diagnosis of BA and serves as a valuable reference for addressing common challenges in rare diseases. The model's high accuracy and its ability to enhance the diagnostic performance of human experts underscore its potential for significant clinical impact.
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Affiliation(s)
- Ya Ma
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China
| | - Yuancheng Yang
- School of Computer Science and Engineering, Beihang University, Beijing, China
- State Key Laboratory of Virtual Reality Technology and Systems, Beihang University, Beijing, China
| | - Yuxin Du
- School of Computer Science and Engineering, Beihang University, Beijing, China
- State Key Laboratory of Virtual Reality Technology and Systems, Beihang University, Beijing, China
| | - Luyang Jin
- School of Computer Science and Engineering, Beihang University, Beijing, China
- State Key Laboratory of Virtual Reality Technology and Systems, Beihang University, Beijing, China
| | - Baoyu Liang
- School of Computer Science and Engineering, Beihang University, Beijing, China
- State Key Laboratory of Virtual Reality Technology and Systems, Beihang University, Beijing, China
| | - Yuqi Zhang
- School of Computer Science and Engineering, Beihang University, Beijing, China
- State Key Laboratory of Virtual Reality Technology and Systems, Beihang University, Beijing, China
| | - Yedi Wang
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China
| | - Luyu Liu
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China
| | - Zijian Zhang
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China
| | - Zelong Jin
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China
| | - Zhimin Qiu
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China
| | - Mao Ye
- Department of General Surgery, Capital Institute of Pediatrics, Beijing, China
| | - Zhengrong Wang
- Department of Ultrasound, Capital Institute of Pediatrics, Beijing, China.
| | - Chao Tong
- School of Computer Science and Engineering, Beihang University, Beijing, China.
- State Key Laboratory of Virtual Reality Technology and Systems, Beihang University, Beijing, China.
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Jeropoulos RM, Arroyo J, Davenport M. Predicting and optimising outcome for biliary atresia. Semin Pediatr Surg 2024; 33:151479. [PMID: 39884180 DOI: 10.1016/j.sempedsurg.2025.151479] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2024] [Accepted: 01/07/2025] [Indexed: 02/01/2025]
Abstract
Biliary atresia (BA) remains a disease of significant morbidity and mortality world-wide. Early and accurate diagnosis facilitates early intervention and improves outcomes. The gold standard in diagnosing BA is a liver biopsy followed by cholangiography, usually performed intra-operatively. Serum markers, like the aspartate aminotransferase-to-platelet ratio, matrix metalloproteinase-7 and several inflammatory cytokines have been recently investigated as non-invasive alternatives with varying degrees of success. Newer immunohistochemical analysis of liver biopsies, such as the expression of secretin receptors and Ki-67, from infants with BA have improved our understanding of the disease process and has shed a little light in predicting post-operative outcomes. There is little standardisation in the care of BA post operatively, though administration of steroids, prevention and treatment of cholangitis with antibiotics and anti-viral therapy for CMV+ve infants are becoming universally accepted as treatment. Experimental stem cell treatments show promise although remain in the out-of-reach future for now in routine clinical practice. This chapter aims to comprehensively describe recent knowledge on predicting the clinical outcomes of infants with BA, as well as optimising their care post operatively.
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Affiliation(s)
- Renos M Jeropoulos
- Dept of Paediatric Surgery, Kings College Hospital, London SE59RS, England, United Kingdom
| | - Jorge Arroyo
- Dept of Paediatric Surgery, Kings College Hospital, London SE59RS, England, United Kingdom
| | - Mark Davenport
- Dept of Paediatric Surgery, Kings College Hospital, London SE59RS, England, United Kingdom.
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Marcellier G, Rivallin P, Hedjoudje A, Bourhrara K, Jais B, Smadhi R, Prat F. Management of an obstructed hepatoportocholecystostomy via an endoscopic cholecystoduodenostomy. Endoscopy 2024; 56:E1086-E1087. [PMID: 39657920 PMCID: PMC11631551 DOI: 10.1055/a-2474-7635] [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: 12/12/2024]
Affiliation(s)
| | | | | | | | | | - Ryad Smadhi
- Gastroenterology Unit, Centre Hospitalier Compiegne-Noyon, Compiegne, France
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Oita S, Toma M, Hirono K, Masuko T, Shimizu T, Shimizu S, Miyajima K, Asai N, Yanai T. Assessment of the utility of two-dimensional shear wave elastography and superb microvascular imaging in postoperative patients with biliary atresia. Pediatr Surg Int 2024; 40:219. [PMID: 39115726 DOI: 10.1007/s00383-024-05804-y] [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] [Accepted: 08/02/2024] [Indexed: 12/14/2024]
Abstract
PURPOSE We aimed to investigate whether prediction of liver fibrosis using two-dimensional shear wave elastography (2D-SWE) and vascular tree grading using superb microvascular imaging (SMI) are useful for postoperative follow-up in patients with biliary atresia (BA). METHODS We retrospectively collected data from medical records of 134 patients who underwent ultrasound examination with 2D-SWE or SMI, including 13 postoperative patients with BA and 121 non-BA patients. We investigated the distribution of liver stiffness values with SWE and vascular tree grading with SMI and evaluated correlations between these findings and biochemical indices of liver fibrosis in postoperative BA patients. RESULTS The SWE values of the BA group were not significantly different from that of any other disease groups in non-BA patients. In postoperative BA patients, SWE values correlated significantly with aspartate aminotransferase to platelet ratio index (Spearman rank correlation coefficient [rs] = 0.6380, p = 0.0256) and with the Fib-4 index (rs = 0.6526, p = 0.0214). SMI vascular tree grading of the BA group was significantly higher than that of the choledochal cyst group (p = 0.0008) and other hepatobiliary disorder group (p = 0.0030). In postoperative BA patients, SMI vascular tree grading was not positively correlated with any biochemical marker of fibrosis. CONCLUSION 2D-SWE appears to be useful for follow-up in postoperative BA patients.
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Affiliation(s)
- Satoru Oita
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan.
| | - Miki Toma
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
- Department of Pediatric Ultrasound Diagnostic and Training Center, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Koji Hirono
- Department of Pediatric Ultrasound Diagnostic and Training Center, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Takayuki Masuko
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Toru Shimizu
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Sakika Shimizu
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Kojiro Miyajima
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Nobuyoshi Asai
- Department of Pediatric Ultrasound Diagnostic and Training Center, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
| | - Toshihiro Yanai
- Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito City, Ibaraki, 311-4145, Japan
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Liu F, Lui VCH, Wu Z, Blakeley PD, Tang CSM, Tam PKH, Wong KKY, Chung PHY. Animal and organoid models to elucidate the anti-fibrotic effect of steroid on biliary atresia. Pediatr Surg Int 2024; 40:214. [PMID: 39102048 PMCID: PMC11300555 DOI: 10.1007/s00383-024-05798-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 07/25/2024] [Indexed: 08/06/2024]
Abstract
PURPOSE We performed animal and organoid study to evaluate the anti-fibrotic effect of steroid on biliary atresia (BA) and the underlying patho-mechanism. METHODS BA animal models were created by inoculation of mice on post-natal day 1 with rhesus rotavirus (RRV). They received either 20 µl phosphate-buffered saline (PBS) or steroid from day 21 to day 34. On day 34, their serum samples were collected for hormonal markers. Necrosis, fibrosis and CK 19 expression in the liver were evaluated. Liver organoids were developed and their morphology as well as bulk RNA sequencing data were analyzed. RESULTS Twenty-four mice developed BA features after RRV injection and were equally divided into steroid and PBS groups. On day 34, the weight gain of steroid group increased significantly than PBS group (p < 0.0001). All mice in the PBS group developed liver fibrosis but only one mouse in the steroid group did. Serum bilirubin and liver parenchymal enzymes were significantly lower in steroid group. The morphology of liver organoids were different between the two groups. A total of 6359 differentially expressed genes were found between steroid group and PBS group. CONCLUSION Based on our findings obtained from RRV-induced BA animal and organoid models, steroid has the potential to mitigate liver fibrosis in BA.
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Affiliation(s)
- Fangran Liu
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China
| | - Vincent Chi Hang Lui
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China
- Dr. Li Dak-Sum Research Centre, The University of Hong Kong, Hong Kong SAR, China
| | - Zhongluan Wu
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China
| | - Paul David Blakeley
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China
| | - Clara Sze Man Tang
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China
- Dr. Li Dak-Sum Research Centre, The University of Hong Kong, Hong Kong SAR, China
| | - Paul Kwong Hang Tam
- Faculty of Medicine, Macau University of Science and Technology, Macau SAR, China
| | - Kenneth Kak Yuen Wong
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China
| | - Patrick Ho Yu Chung
- Department of Surgery, School of Clinical Medicine, Queen Mary Hospital, The University of Hong Kong, 102 Pokfulam Road, Hong Kong SAR, China.
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Bonn J, Gamm K, Ambrosino T, Orkin SH, Taylor A, Peters AL. Distinct effects of racial and socioeconomic disparities on biliary atresia diagnosis and outcome. J Pediatr Gastroenterol Nutr 2024; 78:1038-1046. [PMID: 38567627 DOI: 10.1002/jpn3.12197] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2023] [Revised: 01/18/2024] [Accepted: 02/08/2024] [Indexed: 04/04/2024]
Abstract
OBJECTIVES To identify and distinguish between racial and socioeconomic disparities in age at hepatology care, diagnosis, access to surgical therapy, and liver transplant-free survival in patients with biliary atresia (BA). METHODS Single-center retrospective cohort study of 69 BA patients from 2010 to 2021. Patients were grouped into White and non-White cohorts. The socioeconomic milieu was analyzed utilizing neighborhood deprivation index, a census tract-based calculation of six socioeconomic variables. The primary outcomes of this study were timing of the first hepatology encounter, surgical treatment with hepatic portoenterostomy (HPE), and survival with native liver (SNL) at 2 years. RESULTS Patients were 55% male and 72% White. White patients were referred at a median of 34 days (interquartile range [IQR]: 17-65) vs. 67 days (IQR: 42-133; p = 0.001) in non-White patients. White infants were more likely to undergo HPE (42/50 patients; 84%) compared to non-White (10/19; 53%), odds ratio (OR) 4.73 (95% confidence interval: 1.46-15.31; p = 0.01). Independent of race, patients exposed to increased neighborhood-level deprivation were less likely to receive HPE (OR: 0.49, p = 0.04) and achieve SNL (OR: 0.54, p = 0.02). CONCLUSIONS Racial and socioeconomic disparities are independently associated with timely BA diagnosis, access to surgical treatment, and transplant-free survival. Public health approaches to improve screening for pathologic jaundice in infants of diverse racial backgrounds and to test and implement interventions for socioeconomically at-risk families are needed.
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Affiliation(s)
- Julie Bonn
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
- Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - Kristen Gamm
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
| | - Teresa Ambrosino
- Department of Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
| | - Sarah H Orkin
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
- Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - Amy Taylor
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
- Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
| | - Anna L Peters
- Division of Pediatric Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
- Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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11
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Beati F, Mosca A, Pietrobattista A, Liccardo D, Ronci S, Monti L, Francalanci P, Spada M, Maggiore G, Bagolan P, Fusaro F. Biliary atresia in preterm infants: a single center experience and review of literature. Front Surg 2024; 11:1353424. [PMID: 38496210 PMCID: PMC10940420 DOI: 10.3389/fsurg.2024.1353424] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2023] [Accepted: 02/19/2024] [Indexed: 03/19/2024] Open
Abstract
Introduction The diagnosis of biliary atresia (BA) remains challenging, and there is still uncertainty regarding the optimal time to perform a Kasai portoenterostomy (KPE). Little is known about the difficulties in the diagnosis and outcomes of BA in preterm infants (PBA). This study, which represents the first Italian report of preterm infants with BA, aims to describe a single-center experience of BA in preterm newborns. Methods We retrospectively reviewed all infants consecutively diagnosed with BA who underwent a Kasai procedure at the Bambino Gesù Children's Hospital between January 1998 and December 2021. Prematurity was defined as a gestational age (GA) of <37 weeks. Demographic, laboratory, and histology data were recorded, and the main outcomes considered were clearance of jaundice (COJ), native liver survival, and mortality. Results A total of 21 PBA were compared with 117 term BA controls (TBA). The median GA of PBA was 35.1 (32-36.1) weeks, with a mean birth weight of 2,100 (1,897-2,800) g. Age at first presentation was significantly lower in PBA patients: 46 (22-68) vs. 61 (44-72) days; p = 0.02. The median age at KPE was similar between the two groups: 70 days (33 corrected) for PBA vs. 67 in TBA; p = 0.8. At the time of surgery, median serum bilirubin was lower in the PBA group (7.7 vs. 8.6 mg/dl, p = 0.04). Similarly, the median APRi at the time of KPE was lower but not significant in the PBA group: 1.09 vs. 1.16; p = 0.8. No differences were found in terms of COJ between the PBA and TBA groups: n = 9 (43%) vs. 34 (35%); p = 0.2. Overall native liver survival was similar between the two groups: 8.6 (4.8-12.2) for the PBA group vs. 7.6 (5.6-9.5) years for the TBA group with no significant differences; p = 0.45. Post-KPE native liver survival was similar between the two groups: 38% vs. 52% at 5 years for the TBA and PBA groups, respectively; p = 0.54. Conclusion The PBA and TBA groups appear to have similar outcomes in terms of COJ, overall native liver survival, and 5-year liver survival. Considering the corrected GA, early KPE is related to lower cholestatic damage. Further multicenter studies are required.
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Affiliation(s)
- Federico Beati
- Neonatal Surgery Unit, Area of Fetal, Neonatal and Cardiological Sciences, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Antonella Mosca
- Hepatogastroenterology and Liver Transplant Unit and Medical Genetics Laboratory, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Andrea Pietrobattista
- Hepatogastroenterology and Liver Transplant Unit and Medical Genetics Laboratory, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Daniela Liccardo
- Hepatogastroenterology and Liver Transplant Unit and Medical Genetics Laboratory, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Sara Ronci
- Neonatal Surgery Unit, Area of Fetal, Neonatal and Cardiological Sciences, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Lidia Monti
- Department of Radiology, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Paola Francalanci
- Pathology Unit, Department of Diagnostic and Laboratory Medicine, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Marco Spada
- Division of Abdominal Transplantation and Hepato-Bilio-Pancreatic Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Giuseppe Maggiore
- Hepatogastroenterology and Liver Transplant Unit and Medical Genetics Laboratory, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
| | - Pietro Bagolan
- Neonatal Surgery Unit, Area of Fetal, Neonatal and Cardiological Sciences, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
- Department of Systems Medicine, University of Tor Vergata, Rome, Italy
| | - Fabio Fusaro
- Neonatal Surgery Unit, Area of Fetal, Neonatal and Cardiological Sciences, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
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Sun M, Chen WM, Wu SY, Zhang J. Improved postoperative outcomes in pediatric major surgery: evidence from hospital volume analysis. Eur J Pediatr 2024; 183:619-628. [PMID: 37943333 DOI: 10.1007/s00431-023-05308-2] [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: 05/19/2023] [Revised: 10/21/2023] [Accepted: 10/24/2023] [Indexed: 11/10/2023]
Abstract
This study aimed to examine the association between hospital volume and postoperative outcomes in pediatric major surgery using a nationwide database. The study included pediatric patients who underwent first major elective inpatient surgery and hospitalization for more than 1 day. The results showed no significant difference in the risk of 30-day postoperative mortality based on hospital volume. However, patients in the middle- and high-volume groups had significantly lower rates of 30-day major complications, particularly deep wound infection. In terms of 90-day postoperative outcomes, patients in the high-volume group had a significantly lower risk of mortality and lower rates of major complications, particularly deep wound infection, pneumonia, and septicemia. Conclusions: The study suggests that pediatric patients undergoing major surgery in high and middle-volume groups have better outcomes in terms of major complications compared to the low-volume group. What is Known: • Limited evidence exists on the connection between hospital volume and pediatric surgery outcomes. What is New: • A Taiwan-based study, using national data, found that high and middle hospital-volume groups experienced significantly lower rates of major complications within 30 and 90 days after surgery. • High-volume hospitals demonstrated a substantial decrease in the risk of 90-day postoperative mortality. • The study underscores the importance of specialized pediatric surgical centers and advocates for clear guidelines for hospital selection, potentially improving outcomes and informing future health policies.
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Affiliation(s)
- Mingyang Sun
- Department of Anesthesiology and Perioperative Medicine, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan, China
| | - Wan-Ming Chen
- Graduate Institute of Business Administration, College of Management, Fu Jen Catholic University, Taipei, Taiwan
- Artificial Intelligence Development Center, Fu Jen Catholic University, Taipei, Taiwan
| | - Szu-Yuan Wu
- Graduate Institute of Business Administration, College of Management, Fu Jen Catholic University, Taipei, Taiwan.
- Artificial Intelligence Development Center, Fu Jen Catholic University, Taipei, Taiwan.
- Department of Food Nutrition and Health Biotechnology, College of Medical and Health Science, Asia University, Taichung, Taiwan.
- Big Data Center, Lo-Hsu Medical Foundation, Lotung Poh-Ai Hospital, Yilan, Taiwan.
- Division of Radiation Oncology, Department of Medicine, Lo-Hsu Medical Foundation, Lotung Poh-Ai Hospital, Yilan, Taiwan.
- Department of Healthcare Administration, College of Medical and Health Science, Asia University, Taichung, Taiwan.
- Cancer Center, Lo-Hsu Medical Foundation, Lotung Poh-Ai Hospital, No. 83, Nanchang St.Yilan County 265, Luodong Township, Taiwan.
- Centers for Regional Anesthesia and Pain Medicine, Taipei Municipal Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan.
- Department of Management, College of Management, Fo Guang University, Yilan, Taiwan.
| | - Jiaqiang Zhang
- Department of Anesthesiology and Perioperative Medicine, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan, China.
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Quaglia A, Roberts EA, Torbenson M. Developmental and Inherited Liver Disease. MACSWEEN'S PATHOLOGY OF THE LIVER 2024:122-294. [DOI: 10.1016/b978-0-7020-8228-3.00003-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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Sadiq J, Lloyd C, Hodson J, Trapero Marugan M, Ferguson J, Sharif K, Mirza DF, Hirschfield G, Kelly D. Long-term clinical and socioeconomic outcomes of children with biliary atresia. JGH Open 2023; 7:841-847. [PMID: 38162865 PMCID: PMC10757476 DOI: 10.1002/jgh3.12980] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2022] [Revised: 08/28/2023] [Accepted: 10/02/2023] [Indexed: 01/03/2024]
Abstract
Background Biliary atresia (BA) is rare liver disease of unknown etiology, and is a major indication for liver transplant (LT). Previous data indicate improved outcomes with early referral for Kasai portoenterostomy (KPE). Objective Evaluate the long-term outcomes in BA, with particular focus on those transitioned to adult care with native livers. Subjects and Methods Patients with BA treated between1980 and 2012 were identified. Data were collected from the time of referral, transition to adult care, and the most recent clinic notes, from which patient and native liver survival were calculated. Results Four hundred and fifty-four patients with BA were identified, who were followed up for median of 16.4 years from birth; 74 died (41 of whom had a LT), giving a 20-year survival rate of 83.6%. Two hundred and seventy-two patients received an LT, with the median native liver survival being 35 months. Of patients who transitioned to adult care, 54 of 180 (30.0%) retained their native liver. Of these, 72% (39 of 54) had evidence of chronic liver disease at transition, of whom 8 were subsequently lost to follow-up, 9 were transplanted, and 22 remained stable with compensated liver disease. Of the 15 of 54 patients (28%) with no evidence of chronic disease in their native liver disease at transition, 3 were subsequently lost to follow-up; none received transplants, although 3 patients developed new-onset liver disease. All patients transitioned to adult care completed secondary school education (N = 180), with 49% having attended college/university and 87% being in employment or education at the last follow-up. Of female patients, 34% had at least one pregnancy (27 children in 21 women), while 22% of males had fathered a child. Conclusion Long-term outcomes in BA are good, with patients surviving into adult life. Progression of chronic liver disease and associated morbidity is common in those who retained their native livers, suggesting that these patients require monitoring of liver disease throughout adult life, and early recognition of the need for LT.
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Affiliation(s)
- Javaid Sadiq
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
| | - Carla Lloyd
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
| | - James Hodson
- Institute of Translational MedicineUniversity Hospitals Birmingham NHS Foundation TrustBirminghamUK
- Research Development and InnovationUniversity Hospitals Birmingham NHS Foundation TrustBirminghamUK
| | - Maria Trapero Marugan
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - James Ferguson
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - Khalid Sharif
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
| | - Darius F Mirza
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - Gideon Hirschfield
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
| | - Deirdre Kelly
- Birmingham Women's & Children's Hospital& University Hospital BirminghamBirminghamUK
- Centre for Liver ResearchNIHR Biomedical Research Unit, University of BirminghamBirminghamUK
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Jhala T, Rentea RM, Aichner J, Szavay P. Surgical Simulation of Posterior Sagittal Anorectoplasty for Rectovestibular Fistula: Low-Cost High-Fidelity Animal-Tissue Model. J Pediatr Surg 2023; 58:1916-1920. [PMID: 36935227 DOI: 10.1016/j.jpedsurg.2023.02.055] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2023] [Revised: 01/31/2023] [Accepted: 02/08/2023] [Indexed: 02/25/2023]
Abstract
PURPOSE To provide a high-fidelity, animal tissue-based model for the advanced surgical simulation of a Posterior Sagittal Anorectoplasty (PSARP) for rectovestibular fistula in anorectal malformation (ARM). MATERIALS AND METHODS A chicken cadaver was used to assess the feasibility of simulating a PSARP for rectovestibular fistula in ARM. No modification was required to implement the surgical simulation. RESULTS A detailed description of the high-fidelity surgical simulation model is provided. The PSARP can be simulated while providing realistic anatomy (e.g. common wall between rectovestibular fistula and vagina), adequate rectal size, location and placement of the rectovestibular fistula, and proximity to the vagina. Haptic conditions of the tissue resemble human tissue and operative conditions as well. DISCUSSION Concerning the decreased exposure of index cases of pediatric surgical trainees and pediatric surgeons in practice, simulation-based training can provide means to acquire or maintain the necessary skills to perform complex surgical procedures [1-5] Surgical simulation models for ARM are limited. Few low-cost trainers are available with predominant artificial and mostly unrealistic tissue [6-8] Animal models have the advantage of realistic multilayer tissue haptic feedback [6]. CONCLUSION We provide a low-cost, high-fidelity model for correcting a rectovestibular fistula in a child with ARM, a complex operative procedure with low incidence but high-stake outcomes. The described tissue model utilizing the chicken cloaca anatomy provides a high-fidelity model for operative correction of rectovestibular ARM. For simulation purposes in the treatment of ARM, this model appears to be promising in terms of providing realistic pathology and haptic feedback in pediatric dimensions. LEVEL OF EVIDENCE V.
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Affiliation(s)
- Tobias Jhala
- Department of Pediatric Surgery, Luzerner Kantonsspital, Spitalstrasse, Luzern, Switzerland.
| | - Rebecca M Rentea
- Department of Surgery, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA
| | - Jonathan Aichner
- Department of Pediatric Surgery, Luzerner Kantonsspital, Spitalstrasse, Luzern, Switzerland
| | - Philipp Szavay
- Department of Pediatric Surgery, Luzerner Kantonsspital, Spitalstrasse, Luzern, Switzerland
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Chang CM, Kuo KC, Chen WH, Su CH, Lee CP, Chen KJ, Yang YH, Yen JB, Sheen JM. Maternal risk factors associated with offspring biliary atresia: population-based study. Pediatr Res 2023; 93:1064-1071. [PMID: 35760951 DOI: 10.1038/s41390-022-02166-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2022] [Revised: 05/30/2022] [Accepted: 06/06/2022] [Indexed: 11/09/2022]
Abstract
BACKGROUND Biliary atresia (BA) is a progressive, idiopathic, fibro-obliterative disease of the intra and extrahepatic biliary tree. If untreated, it results in severe liver injury and death. The etiology and pathogenesis of BA remain unclear. Few studies have investigated the association between maternal illness/drug use and the occurrence of BA in offspring. METHODS We used the data from the Birth Certificate Application of Taiwan and linked to National Health Insurance Research Database and Taiwan Maternal and Child Health Database for the years 2004 to 2017 (N = 1,647,231) on 2022/03, and identified BA cases according to diagnosis and procedure code. A total of 285 BA cases were identified. RESULTS Mothers with type 2 diabetes mellitus and non-dependent drug abuse had higher rates having BA children than non-BA children, with an odds ratio of 2.17 (95% confidence interval [CI] = 1.04-4.53) and OR: 3.02 (95% CI = 1.34-6.78), respectively. CONCLUSION These results support the notion that BA occurrence is related to maternal reasons. Further studies should be designed to identify additional maternal and pregnancy risk factors and to understand the underlying pathophysiology. IMPACT 1. The occurrence of offspring biliary atresia may be related to maternal illness/drug use. 2. Maternal drug abuse and type 2 diabetes mellitus pose a high risk for offspring biliary atresia. 3. If maternal etiology is found, biliary atresia might be a preventable disease.
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Affiliation(s)
- Ching-Min Chang
- Department of Pediatrics, Chiayi Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Chiayi, Taiwan
| | - Kuang-Che Kuo
- Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Wan-Hsuan Chen
- Department of Pediatrics, Chiayi Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Chiayi, Taiwan
| | - Chung-Hao Su
- Department of Pediatrics, Chiayi Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Chiayi, Taiwan
| | - Chuan-Pin Lee
- Health Information and Epidemiology Laboratory, Chang Gung Memorial Hospital, Chiayi, Taiwan
| | - Ko-Jung Chen
- Health Information and Epidemiology Laboratory, Chang Gung Memorial Hospital, Chiayi, Taiwan
| | - Yao-Hsu Yang
- Health Information and Epidemiology Laboratory, Chang Gung Memorial Hospital, Chiayi, Taiwan
- Department of Traditional Chinese Medicine, Chiayi Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Chiayi, Taiwan
| | - Ju-Bei Yen
- Department of Pediatrics, Chiayi Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Chiayi, Taiwan.
| | - Jiunn-Ming Sheen
- Department of Pediatrics, Chiayi Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Chiayi, Taiwan.
- Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
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Fusco J, Williams R, Malek M, Avansino JR, Hirose S, Perkins JA, Farmer D, Gow KW. Centers of Excellence: If We Build It, Will They Come? J Pediatr Surg 2023; 58:1048-1052. [PMID: 36925401 DOI: 10.1016/j.jpedsurg.2023.02.010] [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: 01/28/2023] [Accepted: 02/03/2023] [Indexed: 02/19/2023]
Abstract
Pediatric surgeons have been pursuing high quality, affordable care or value-based care for over 50 years. One approach to streamlining the clinical care for a complex problem was the development of a center of excellence (COE). The concept of COE focuses on a shared vision of providing high quality care through a multidisciplinary approach. The goal is to improve diagnostic accuracy as well as therapeutic outcomes using focused expertise within a group. COEs are often resource intensive before becoming fiscally viable and therefore require initial support from hospital leadership. This review discusses the key steps to consider before building a COE, strategies to help build one, and how to keep one successful as defined by quality, accessibility, equity, training, and maintaining teams within the group.
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Affiliation(s)
- Joseph Fusco
- Monroe Carrel Jr Children's Hospital at Vanderbilt, Nashville, TN, 37232, USA
| | | | - Marcus Malek
- University of Pittsburgh, Pittsburgh, PA, 15213, USA
| | - Jeffery R Avansino
- Seattle Children's Hospital and the University of Washington, Seattle, WA, 98105, USA
| | | | - Jonathan A Perkins
- Seattle Children's Hospital and the University of Washington, Seattle, WA, 98105, USA
| | | | - Kenneth W Gow
- Seattle Children's Hospital and the University of Washington, Seattle, WA, 98105, USA.
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MiR-100 rs1834306 A>G Increases Biliary Atresia Risk in Southern Han Chinese Children. BIOMED RESEARCH INTERNATIONAL 2023; 2023:4835839. [PMID: 36644163 PMCID: PMC9833893 DOI: 10.1155/2023/4835839] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/19/2022] [Revised: 11/29/2022] [Accepted: 12/24/2022] [Indexed: 01/06/2023]
Abstract
Background Biliary atresia (BA) is a type of severe cholestatic childhood disease that may have a genetic component. miR-100 plays a key role in regulating cell apoptosis, proliferation, and inflammatory reactions. A single-nucleotide polymorphism in miR-100 has been proven to modulate susceptibility to various diseases. Methods We conducted a case-control retrospective study to explore the correlation between miR-100 gene polymorphism (rs1834306 A>G) and biliary atresia susceptibility in 484 Chinese patients and 1445 matched control subjects. Results Our results showed that rs1834306 A>G was correlated with a significantly increased risk for BA (GG vs. AA: adjusted odds ratio (OR) = 1.44, 95%confidence interval (CI) = 1.02-2.03, p = 0.041; and GG vs. AA/AG: adjusted OR = 1.39, 95%CI = 1.02-1.89, p = 0.036). Conclusions Our results showed that the rs1834306 A>G polymorphism is associated with an increased risk for BA and contributes to BA susceptibility.
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Dong B, Weng Z, Lyu G, Yang X, Wang H. The diagnostic performance of ultrasound elastography for biliary atresia: A meta-analysis. Front Public Health 2022; 10:973125. [PMID: 36388297 PMCID: PMC9643747 DOI: 10.3389/fpubh.2022.973125] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2022] [Accepted: 10/07/2022] [Indexed: 01/25/2023] Open
Abstract
Background Biliary atresia (BA) is a severe inflammatory obliterative cholangiopathy of infancy that requires early diagnosis and prompt surgical intervention. In this study, we aimed to obtain comprehensive evidence on the diagnostic performance of liver stiffness measurement by ultrasound elastography in the detection of BA through a meta-analysis. Methods The PubMed, EMBASE, Cochrane Library, and Web of Science databases were searched for studies that investigated the diagnostic performance of ultrasound elastography in the detection of BA up to January 10, 2022. In this study, in order to summarize the diagnostic performance of ultrasound elastography, the summary receiver operating characteristic (SROC) modeling was constructed. Heterogeneity was estimated with the I 2 statistic. Multiple subgroup analyses were also performed. Results Fourteen studies from eleven articles, including 774 BA patients, 850 non-BA patients, and 173 controls were included in the present meta-analysis. The summary sensitivity and specificity of ultrasound elastography for liver stiffness were 85% [95% confidence interval (CI): 79-89%] and 82% (95% CI: 73-88%) with the I 2 value of 82.90 and 84.33%, respectively. The area under the SROC curve (AUROC) using ultrasound elastography for diagnosing BA was 0.90 (95% CI: 0.87-0.92). In addition, a subgroup analysis of 9 two-dimensional shear wave elastography studies was also performed. Subgroup analysis revealed that the summary sensitivity and specificity were 85% (95% CI: 77-91%) and 79% (95% CI: 71-86%), respectively, and the summary AUROC was 0.89 (95% CI: 0.86-0.92). Conclusions Ultrasound elastography exhibits good diagnostic accuracy for BA and can be served as a non-invasive tool to facilitate the differential diagnosis of BA.
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Affiliation(s)
- Bingtian Dong
- Department of Ultrasound, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China
| | - Zongjie Weng
- Department of Medical Ultrasonics, Fujian Provincial Maternity and Children's Hospital, Affiliated Hospital of Fujian Medical University, Fuzhou, China
| | - Guorong Lyu
- Department of Ultrasound, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China,Collaborative Innovation Center for Maternal and Infant Health Service Application Technology, Quanzhou Medical College, Quanzhou, China,*Correspondence: Guorong Lyu
| | - Xiaocen Yang
- Department of Ultrasound, Chenggong Hospital, Xiamen University, Xiamen, China
| | - Huaming Wang
- Department of Ultrasound, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China
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Zheng L, Wu Y, Gong Z, Lv Z, Xu W, Sheng Q, Huang X, Liu J. The composition of the gut microbiota is altered in biliary atresia with cholangitis. Front Surg 2022; 9:990101. [PMID: 36338646 PMCID: PMC9632985 DOI: 10.3389/fsurg.2022.990101] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2022] [Accepted: 09/05/2022] [Indexed: 12/02/2022] Open
Abstract
Aim To detect the composition of the gut microbiota in biliary atresia after Kasai surgery. Methods Infants within six months after the Kasai operation who were diagnosed by cholangiography at Shanghai Children’s Hospital were enrolled in the study. Fecal samples were collected from diapers, placed into sterile tubes in the inpatient department or outpatient department and frozen at −80°C within half an hour. The gut microbiota was detected by 16S rRNA sequences. Then, the patients that were followed up to one year after the Kasai operation who suffered from cholangitis at least one time were grouped into the BAcho group, and the others were grouped into the BAnoncho group. Results Nine of 18 BA patients were grouped into the BAcho group, and the others were grouped into the BAnoncho group. In the BAcho group, AST, ALT and GGT were significantly increased compared to the BAnoncho group. The number of total OTUs (operational taxonomic units) in feces was more elevated in the BAnoncho group than in the BAcho group. In the BAnoncho group, the Chao index at the OTU level was significantly increased compared to that in the BAcho group (66.37 ± 21.5 vs. 45.64 ± 11.25, p = 0.02 < 0.05). Bifidobacterium was the most abundant genus in the BAnoncho group, accounting for 22.14%, and Klebsiella accounted for 22.74% in the BAcho group. Compared with the BAnoncho group, Bacteroides was significantly decreased in the BAcho group (p = 0.037). Conclusion The composition of the gut microbiota was different between BA with cholangitis and BA without cholangitis.
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Betalli P, Cheli M, Colusso MM, Casotti V, Alberti D, Ferrari A, Starita G, Lucianetti A, Pinelli D, Colledan M, D'Antiga L. Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia. J Pediatr Surg 2022; 57:223-228. [PMID: 35063251 DOI: 10.1016/j.jpedsurg.2021.12.030] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/16/2021] [Revised: 11/09/2021] [Accepted: 12/29/2021] [Indexed: 12/11/2022]
Abstract
BACKGROUND Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT). METHODS Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (<5 KPE/year). We analyzed total transplant time (TTT), hepatectomy time, amount of plasma and red blood cell (RBC) transfusions, occurrence of bowel perforations at LT. RESULTS Among 115 patients, Group A (n 44) and Group B (n 71) were comparable for age, sex, PELD score, TTT. The groups differed for: median hepatectomy time (57 min, IQR = 50-67; vs 65, IQR 55-89, p = 0.045); RBC transfusions (95 ml, IQR 0-250; vs 200 ml, IQR 70-500, p = 0.017); bowel perforations (0/44 vs 15/71, p = 0.001). One-year graft loss in Group A vs Group B was 1/44 vs 7/71 (p = 0.239), whereas deaths were 0/44 vs 5/71 respectively (p = 0.183); 5/15 patients who had a perforation eventually lost the graft. CONCLUSIONS This study found an association between KPE performed in low caseload center and the incidence of complications at LT. These patients tend to have a worse outcome. The centralization of KPE to referral center represents an advantage at the time of LT. MINI ABSTRACT We studied the impact of Kasai portoenterostomy (KPE) caseload on complications at the time of liver transplantation (LT), in 115 patients. We found an association between KPE performed in low caseload center and increased bowel perforations and blood transfusions. We suggest to centralize to experienced center all children requiring KPE.
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Affiliation(s)
- Pietro Betalli
- Department of Paediatric Surgery, "Papa Giovanni XXIII" ASST, Piazza OMS1, Bergamo 24127, Italy.
| | - Maurizio Cheli
- Department of Paediatric Surgery, "Papa Giovanni XXIII" ASST, Piazza OMS1, Bergamo 24127, Italy
| | - Mara Marcella Colusso
- Department of Paediatric Surgery, "Papa Giovanni XXIII" ASST, Piazza OMS1, Bergamo 24127, Italy
| | - Valeria Casotti
- Department of Paediatric Hepatology, Gastroenterology and Transplantation, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Daniele Alberti
- Department of Paediatric Surgery, "Spedali Civili" Children's Hospital, Brescia, Italy
| | - Alberto Ferrari
- From Research Foundation, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Giusy Starita
- Department of Anaesthesia, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | | | - Domenico Pinelli
- Department of Surgery III, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Michele Colledan
- Department of Surgery III, "Papa Giovanni XXIII" ASST, Bergamo, Italy
| | - Lorenzo D'Antiga
- Department of Paediatric Hepatology, Gastroenterology and Transplantation, "Papa Giovanni XXIII" ASST, Bergamo, Italy
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Yang C, Ke M, Zhou Y, Xu H, Diao M, Li L. Impact of early Kasai portoenterostomy on short-term outcomes of biliary atresia: A systematic review and meta-analysis. Front Surg 2022; 9:924506. [PMID: 36117834 PMCID: PMC9475174 DOI: 10.3389/fsurg.2022.924506] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2022] [Accepted: 08/02/2022] [Indexed: 11/22/2022] Open
Abstract
Background Good outcomes of biliary atresia (BA) are conventionally achieved after early Kasai portoenterostomy (KP). However, in some recent pieces of literature, there are discrepancies in the influence of age in Kasai procedure on postoperative short-term prognosis. This meta-analysis aims to evaluate the effects of earlier KP on short-term surgical prognosis of BA and clarify these discrepancies in recent studies. Methods To identify related studies, PubMed, Embase, Web of Science, Cochrane, and the Chinese National Knowledge Infrastructure database were searched up to March 2022. Data for the impact of age at KP on clinical prognosis were extracted, including jaundice clearance rate (JCR) and native liver survival rate (NLSR). Results A total of 14 articles were included in the present study, which involve a total of 3,276 patients with BA who underwent Kasai procedure. Compared with patients older than 91 days of age, patients 90 days of age or younger exhibited significantly better JCR [odds ratio (OR), 3.05; 95% confidence interval (CI), 2.23–4.17; P < .001] and a more favorable NLSR (OR, 1.72; 95% CI, 1.37–2.15; P < .001). The NLSR of patients younger than 60 days of age was significantly higher than those of patients from 61 to 90 days of age (OR, 1.41; 95% CI, 1.18–1.68; P < .001). There was no significant difference in JCRs between patients aged 60 days of age or younger and those aged 61–90 days of age (OR, 1.31; 95% CI, 0.95–1.81; P = 0.10). Among patients 30 days of age or younger, 31–45 days of age, and 46–60 days of age, there were also no significant differences in JCR. Conclusion A significantly better short-term JCR and NLSRs were achieved among patients with BA treated using a KP procedure at ≤90 days of age compared with those treated at >90 days of age. There was no further improvement in the short-term JCR when the procedure was performed at ≤60 days compared with those treated at 61–90 days of age. However, treatment at ≤60 days of age was associated with a significant improvement in NLSR. Therefore, the timing of KP does exert an important effect on short-term clinical outcomes of patients with BA.
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Affiliation(s)
- Changzhen Yang
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment (2021RU015), Chinese Academy of Medical Sciences, Beijing, China
| | - Meng Ke
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment (2021RU015), Chinese Academy of Medical Sciences, Beijing, China
| | - Yan Zhou
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment (2021RU015), Chinese Academy of Medical Sciences, Beijing, China
| | - Hang Xu
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Mei Diao
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment (2021RU015), Chinese Academy of Medical Sciences, Beijing, China
| | - Long Li
- Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China
- Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
- Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment (2021RU015), Chinese Academy of Medical Sciences, Beijing, China
- Correspondence: Long Li
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Long-term follow-up of biliary atresia using liver transient elastography. Pediatr Surg Int 2022; 38:1013-1018. [PMID: 35523886 DOI: 10.1007/s00383-022-05137-8] [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] [Accepted: 05/01/2022] [Indexed: 10/18/2022]
Abstract
OBJECTIVE Liver transient elastography (TE) using FibroScan® has gained popularity as a non-invasive technique to assess hepatic fibrosis by measuring liver stiffness. This study focused on biliary atresia patients post Kasai operation for more than 10 years to prospectively correlate the hepatic fibrosis score to the biochemical changes of liver fibrosis and clinical development of portal hypertensive complications. METHODS TE was performed in 37 patients who had biliary atresia post Kasai operation done at median age of 60 days. Biochemical indices of liver fibrosis including aspartate aminotransferase/platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score based on age, platelet count, alanine aminotransferase and aspartate aminotransferase level were calculated at the time of TE. Platelet count, spleen size, varices, ascites and hepatic encephalopathy were evaluated as clinical markers of portal hypertension. RESULTS There were 22 female and 15 male with TE done at median age of 17.0 years. Median FibroScan® fibrosis score was 11.4. Fibrosis score of 6.8 kilopascal (kPa) was taken as the upper reference limit of normal. Nine patients (24%) had normal fibrosis score. Score above or equal to 6.8 kPa was significantly associated with lower platelet level (p = 0.001), higher INR (p = 0.043), higher APRI (p = 0.021), higher FIB-4 score (p = 0.013), and larger splenic diameter (p = 0.004). Higher FibroScan® fibrosis score was also significantly associated with portal hypertensive complications (p = 0.001). CONCLUSIONS The FibroScan® fibrosis score correlated well with the biochemical changes of liver fibrosis and development of portal hypertensive complications clinically. Screening of portal hypertensive complications such as varices is recommended for patients with raised fibrosis score upon long-term follow-up. LEVEL OF EVIDENCE Level III, retrospective comparative study.
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Obaid AM, Turki A, Bellaaj H, Ksontini M. Detection of Biliary Artesia using Sonographic Gallbladder Images with the help of Deep Learning approaches. 2022 8TH INTERNATIONAL CONFERENCE ON CONTROL, DECISION AND INFORMATION TECHNOLOGIES (CODIT) 2022:705-711. [DOI: 10.1109/codit55151.2022.9804084] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
Affiliation(s)
| | - Amina Turki
- Control and Energy Management Laboratory (CEMLab), National Engineering School of Sfax,Sfax,Tunisia,3038
| | - Hatem Bellaaj
- Research laboratory on Development and Control of Distributed Applications. (ReDCAD) IPEIS-Route Menzel Chaker Km 0,5-3018 Sfax Tunisie,Sfax,Tunisia,BP 1172-3018
| | - Mohamed Ksontini
- Control & Energy Management Laboratory (CEM-Lab), National Engineering School of Sfax, University of Sfax,Sfax,Tunisia,3038
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25
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Xu X, Dou R, Zhao S, Zhao J, Gou Q, Wang L, Zhan J. Outcomes of biliary atresia splenic malformation (BASM) syndrome following Kasai operation: a systematic review and meta-analysis. WORLD JOURNAL OF PEDIATRIC SURGERY 2022; 5:e000346. [DOI: 10.1136/wjps-2021-000346] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2021] [Accepted: 03/06/2022] [Indexed: 11/04/2022] Open
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26
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Identification of Hub Genes and Immune Infiltration in Pediatric Biliary Atresia by Comprehensive Bioinformatics Analysis. CHILDREN 2022; 9:children9050697. [PMID: 35626874 PMCID: PMC9140130 DOI: 10.3390/children9050697] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/16/2022] [Revised: 04/21/2022] [Accepted: 05/06/2022] [Indexed: 12/04/2022]
Abstract
Background: Biliary atresia (BA) is the leading cause of pediatric liver failure and pediatric liver transplantation worldwide. Evidence suggests that the immune system plays a central role in the pathogenesis of BA. Methods: In this work, the novel immune-related genes between BA and normal samples were investigated based on weighted gene co-expression network analysis (WGCNA) and the deconvolution algorithm of CIBERSORT. Results: Specifically, 650 DEGs were identified between the BA and normal groups. The blue module was the most positively correlated with BA containing 3274 genes. Totally, 610 overlapping BA-related genes of DEGs and WGCNA were further used to identify IRGs. Three IRGs including VCAM1, HLA-DRA, and CD74 were finally identified as the candidate biomarkers. Particularly, the CD74 biomarker was discovered for the first as a potential immune biomarker for BA. Conclusions: Possibly, these 3 IRGs might serve as candidate biomarkers and guide the individualized treatment strategies for BA patients. Our results would provide great insights for a deeper understanding of both the occurrence and the treatment of BA.
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27
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Direct health care cost of treatment and medication of biliary atresia patients using the National Database of Health Insurance Claims and Specific Health Checkups. Pediatr Surg Int 2022; 38:547-554. [PMID: 35165784 PMCID: PMC8913443 DOI: 10.1007/s00383-022-05079-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/27/2022] [Indexed: 11/22/2022]
Abstract
BACKGROUND Treatment of biliary atresia (BA), which typically requires an initial surgical intervention called the Kasai procedure (KP) and possible liver transplant (LT) afterwards, is quite resource-intensive and would affect patients and families for a lifetime; yet a comprehensive view of the economic burden has not been reported. We estimated direct health care costs from the public payer perspective using the National Database of Health Insurance Claims and Specific Health Checkups of Japan. METHODS Children newly diagnosed at ages 0 days to 4 years between April 2010 and September 2019 were identified. Costs of treatment were estimated for six phases of care: prediagnosis, KP and inpatient hospitalization, follow-up after KP, pre-transplant checkup, LT and inpatient hospitalization, and follow-up after LT. RESULTS Mean total prediagnosis medical cost was $6847 (USD) and KP and inpatient hospitalization was $42,157 per year. Follow-up after KP was $15,499, and pre-transplant checkup after KP was $36,015 per year. Mean cost for LT and inpatient hospitalization was $105,334, and follow-up after liver transplant was $25,459 per year. CONCLUSIONS Treatment of BA requires extensive medical resource consumption. The use of the comprehensive national database allowed us to estimate the costs which will be useful for health service planning and cost-effectiveness analysis.
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28
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Autologous bone marrow mononuclear cell infusion for liver cirrhosis after the Kasai operation in children with biliary atresia. Stem Cell Res Ther 2022; 13:108. [PMID: 35287722 PMCID: PMC8919575 DOI: 10.1186/s13287-022-02762-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2021] [Accepted: 02/01/2022] [Indexed: 02/06/2023] Open
Abstract
Aim To evaluate the safety and early outcomes of autologous bone marrow mononuclear cell (BMMNC) infusion for liver cirrhosis due to biliary atresia (BA) after Kasai operation.
Methods An open-label clinical trial was performed from January 2017 to December 2019. Nineteen children with liver cirrhosis due to BA after Kasai operation were included. Bone marrow was harvested through anterior iliac crest puncture under general anesthesia. Mononuclear cells (MNCs) were isolated by Ficoll gradient centrifugation and then infused into the hepatic artery. The same procedure was repeated 6 months later. Serum bilirubin, albumin, alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, and prothrombin time were monitored at baseline, 3 months, 6 months, and 12 months after the first transplantation. Esophagoscopies and liver biopsies were performed in patients whose parents provided consent. Mixed-effect analysis was used to evaluate the changes in Pediatric End-Stage Liver Disease (PELD) scores.
Results The average MNC and CD34+ cell counts per kg body weight were 50.1 ± 58.5 × 106/kg and 3.5 ± 2.8 × 106 for the first transplantation and 57.1 ± 42.0 × 106/kg and 3.7 ± 2.7 × 106 for the second transplantation. No severe adverse events associated with the cell therapy were observed in the patients. One patient died 5 months after the first infusion at a provincial hospital due to the rupture of esophageal varices, while 18 patients survived. Liver function was maintained or improved after infusion, as assessed by biochemical tests. The severity of the disease reduced markedly, with a significant reduction in PELD scores.
Conclusion Autologous BMMNC administration for liver cirrhosis due to BA is safe and may maintain or improve liver function. Trial registration ClinicalTrials.gov identifier: NCT03468699. Name of the registry: Vinmec Research Institute of Stem Cell and Gene Technology. https://clinicaltrials.gov/ct2/show/NCT03468699?cond=biliary+atresia&cntry=VN&draw=2&rank=2. Registered on March 16, 2018. The trial results will also be published according to the CONSORT statement at conferences and reported in peer-reviewed journals.
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Konishi KI, Suzuki K, Komura M, Kudo H, Ishimaru T, Sugiyama M, Komuro H, Iwanaka T, Fujishiro J. Endoscopic nasobiliary drainage tube insertion for treatment of afferent loop obstruction in a pregnant woman after Kasai portoenterostomy. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102194] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
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The Need for Early Kasai Portoenterostomy: A Western Pediatric Surgery Research Consortium Study. Pediatr Surg Int 2022; 38:193-199. [PMID: 34854975 PMCID: PMC8742784 DOI: 10.1007/s00383-021-05047-1] [Citation(s) in RCA: 16] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/20/2021] [Indexed: 02/03/2023]
Abstract
PURPOSE The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia. METHODS A multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance. RESULTS Overall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3 months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97-0.99). CONCLUSION Earlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.
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Al-Hussaini A, Abanemai M, Alhebbi H, Saadah O, Bader R, Al Sarkhy A, Alhatlani M, Halabi H, Aladsani A, AlEdreesi M, Wali S, Alguofi T, Al-Drees K, Arain Z, Al Saleem B, Asery A, Holdar S, Alrashidi S, Alsayed F, Aldhalan S, NasserAllah A, Alghamdi R, Alhaffaf F, AlAwfi A, AlSweed A, Alshamrani A, AlShaikh M, Saeed A, Assiri H, Bashir MS. The Epidemiology and Outcome of Biliary Atresia: Saudi Arabian National Study (2000-2018). Front Pediatr 2022; 10:921948. [PMID: 35923790 PMCID: PMC9339784 DOI: 10.3389/fped.2022.921948] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2022] [Accepted: 05/27/2022] [Indexed: 11/13/2022] Open
Abstract
BACKGROUND The epidemiology and outcomes of biliary atresia (BA) have been well-documented in national cohorts from two main ethnicities, namely, the Asian Orientals and Caucasians, with incidence ranging from 1 in 5,000 to 1 in 9,000 live births in East Asia and 1 in 15,000 to 19,000 live births in Europe and North America. OBJECTIVE We report the first nationwide BA study outside North America, Europe, and East Asia to describe the epidemiology and outcomes of BA in Saudi Arabia. METHODS A national database of BA cases diagnosed between 2000 and 2018 was analyzed. We assessed clearance of jaundice (bilirubin <20 μmol/L) in all cases that underwent Kasai portoenterostomy (KPE). We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation (LT), death, or survival with native liver (SNL). RESULTS BA was diagnosed in 204 infants (106 females; 10% pre-term). The incidence of BA was 1 in 44,365, or 2.254 in 100,000 live births (range, 0.5-4 in 100,000). Polysplenia was diagnosed in 22 cases (11%). The median age at referral was 65 days. A total of 146 children (71.5%) underwent KPE at a median age of 70 days. Clearance of jaundice was achieved in 66 of the 146 (45%) infants. The 10-year SNL after KPE was 25.5%, and the overall 10-year estimated survival was 72.5%. The Kaplan-Meier survival curves for patients undergoing KPE at the age of <60, 61-90, and >90 days showed a SNL rate at 51.6, 33, and 12.5%, respectively, at 5 years (P < 0.001). The 2-, 5-, and 10-year post-LT survival rates were 92.5, 90.6, and 90%, respectively. Undergoing an initial KPE did not impact negatively on the overall LT survival rate when compared to BA cases that underwent primary LT (P = 0.88). CONCLUSION The incidence rate of BA in Saudi Arabia is lower than the incidence reported elsewhere. Late referral of BA cases remains a problem in Saudi Arabia; as a result, the SNL rate was lower than reported by other national registries. Hence, national policies devoted to timely referral and earlier age at KPE are needed.
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Affiliation(s)
- Abdulrahman Al-Hussaini
- Division of Pediatric Gastroenterology, Children's Specialized Hospital, King Fahad Medical City, Riyadh, Saudi Arabia.,College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.,Prince Abdullah Bin Khaled Celiac Disease Research Chair, Department of Pediatrics, Faculty of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Mohammed Abanemai
- Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - Homoud Alhebbi
- Division of Pediatric Gastroenterology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia
| | - Omar Saadah
- Division of Pediatric Gastroenterology, Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
| | - Razan Bader
- Multi-Organ Transplant Center, King Fahad Specialist Hospital, Dammam, Saudi Arabia.,King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Ahmed Al Sarkhy
- Gastroenterology Division, Department of Pediatrics, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
| | - Maher Alhatlani
- Al Imam Abdulrahman Bin Faisal Hospital, Ministry of National Guard Health Affairs, Dammam, Saudi Arabia
| | - Hana Halabi
- Maternity and Children's Hospital, Makkah, Saudi Arabia
| | - Ahmed Aladsani
- Department of Pediatrics, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia
| | - Mohammed AlEdreesi
- Specialty Pediatrics Division, Women and Children's Health Institute, Pediatric Gastroenterology, Johns Hopkins Aramco Healthcare, Dhahran, Saudi Arabia
| | - Sami Wali
- Division of Pediatric Gastroenterology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia
| | - Talal Alguofi
- Organs Transplant Centre, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - Khalid Al-Drees
- Department of Pediatrics, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia
| | - Zahid Arain
- Multi-Organ Transplant Center, King Fahad Specialist Hospital, Dammam, Saudi Arabia.,King Fahad Specialist Hospital, Dammam, Saudi Arabia
| | - Badr Al Saleem
- Division of Pediatric Gastroenterology, Children's Specialized Hospital, King Fahad Medical City, Riyadh, Saudi Arabia
| | - Ali Asery
- Division of Pediatric Gastroenterology, Children's Specialized Hospital, King Fahad Medical City, Riyadh, Saudi Arabia
| | - Sinan Holdar
- Division of Pediatric Gastroenterology, Department of Pediatrics, Royal Commission Hospital, Jubail, Saudi Arabia
| | - Sami Alrashidi
- Division of Pediatric Gastroenterology, Children's Specialized Hospital, King Fahad Medical City, Riyadh, Saudi Arabia
| | - Fahad Alsayed
- Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - Sulaiman Aldhalan
- Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | | | - Rawabi Alghamdi
- Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - Faisal Alhaffaf
- Division of Pediatric Gastroenterology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia
| | - Ahmed AlAwfi
- Division of Pediatric Gastroenterology, King Saud Medical City, Riyadh, Saudi Arabia
| | - Abdulrahman AlSweed
- Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | | | - Manal AlShaikh
- Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - Anjum Saeed
- Gastroenterology Division, Department of Pediatrics, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
| | - Heba Assiri
- Gastroenterology Division, Department of Pediatrics, King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia
| | - Muhammed Salman Bashir
- Department of Biostatistics, Research Services Administration, Research Center, King Fahad Medical City, Riyadh, Saudi Arabia
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32
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Karlsen TH, Sheron N, Zelber-Sagi S, Carrieri P, Dusheiko G, Bugianesi E, Pryke R, Hutchinson SJ, Sangro B, Martin NK, Cecchini M, Dirac MA, Belloni A, Serra-Burriel M, Ponsioen CY, Sheena B, Lerouge A, Devaux M, Scott N, Hellard M, Verkade HJ, Sturm E, Marchesini G, Yki-Järvinen H, Byrne CD, Targher G, Tur-Sinai A, Barrett D, Ninburg M, Reic T, Taylor A, Rhodes T, Treloar C, Petersen C, Schramm C, Flisiak R, Simonova MY, Pares A, Johnson P, Cucchetti A, Graupera I, Lionis C, Pose E, Fabrellas N, Ma AT, Mendive JM, Mazzaferro V, Rutter H, Cortez-Pinto H, Kelly D, Burton R, Lazarus JV, Ginès P, Buti M, Newsome PN, Burra P, Manns MP. The EASL-Lancet Liver Commission: protecting the next generation of Europeans against liver disease complications and premature mortality. Lancet 2022; 399:61-116. [PMID: 34863359 DOI: 10.1016/s0140-6736(21)01701-3] [Citation(s) in RCA: 361] [Impact Index Per Article: 120.3] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/18/2021] [Revised: 07/10/2021] [Accepted: 07/15/2021] [Indexed: 02/07/2023]
Affiliation(s)
- Tom H Karlsen
- Department of Transplantation Medicine and Research Institute for Internal Medicine, Division of Surgery, Inflammatory Diseases and Transplantation, Oslo University Hospital Rikshospitalet and University of Oslo, Oslo, Norway.
| | - Nick Sheron
- Institute of Hepatology, Foundation for Liver Research, Kings College London, London, UK
| | - Shira Zelber-Sagi
- School of Public Health, Faculty of Social Welfare and Health Sciences, University of Haifa, Haifa, Israel; Department of Gastroenterology, Tel Aviv Medical Center, Tel Aviv, Israel
| | - Patrizia Carrieri
- Aix-Marseille University, Inserm, Institut de recherche pour le développement, Sciences Economiques et Sociales de la Santé et Traitement de l'Information Médicale (SESSTIM), ISSPAM, Marseille, France
| | - Geoffrey Dusheiko
- School of Medicine, University College London, London, UK; Kings College Hospital, London, UK
| | - Elisabetta Bugianesi
- Department of Medical Sciences, Division of Gastroenterology, University of Torino, Torino, Italy
| | | | - Sharon J Hutchinson
- School of Health and Life Sciences, Glasgow Caledonian University, Glasgow, UK; Clinical and Protecting Health Directorate, Public Health Scotland, Glasgow, UK
| | - Bruno Sangro
- Liver Unit, Clinica Universidad de Navarra-IDISNA and Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBEREHD), Pamplona, Spain
| | - Natasha K Martin
- Division of Infectious Diseases and Global Public Health, University of California San Diego, San Diego, CA, USA; Population Health Sciences, University of Bristol, Bristol, UK
| | - Michele Cecchini
- Health Division, Organisation for Economic Co-operation and Development, Paris, France
| | - Mae Ashworth Dirac
- Department of Health Metrics Sciences, University of Washington, Seattle, WA, USA; Department of Family Medicine, University of Washington, Seattle, WA, USA; Institute for Health Metrics and Evaluation, University of Washington, Seattle, WA, USA
| | - Annalisa Belloni
- Health Economics and Modelling Division, Public Health England, London, UK
| | - Miquel Serra-Burriel
- Epidemiology, Biostatistics and Prevention Institute, University of Zurich, Zurich, Switzerland
| | - Cyriel Y Ponsioen
- Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam University Medical Centers, Amsterdam, Netherlands
| | - Brittney Sheena
- Institute for Health Metrics and Evaluation, University of Washington, Seattle, WA, USA
| | - Alienor Lerouge
- Health Division, Organisation for Economic Co-operation and Development, Paris, France
| | - Marion Devaux
- Health Division, Organisation for Economic Co-operation and Development, Paris, France
| | - Nick Scott
- Disease Elimination Program, Burnet Institute, Melbourne, VIC, Australia
| | - Margaret Hellard
- Disease Elimination Program, Burnet Institute, Melbourne, VIC, Australia; Department of Infectious Diseases, Alfred Hospital, Melbourne, VIC, Australia; Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia; Doherty Institute and School of Population and Global Health, University of Melbourne, Melbourne, VIC, Australia
| | - Henkjan J Verkade
- Paediatric Gastroenterology and Hepatology, Department of Paediatrics, University Medical Centre Groningen, University of Groningen, Netherlands; European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany
| | - Ekkehard Sturm
- Division of Paediatric Gastroenterology and Hepatology, University Children's Hospital Tübingen, Tübingen, Germany; European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany
| | | | | | - Chris D Byrne
- Department of Nutrition and Metabolism, Faculty of Medicine, University of Southampton, Southampton, UK; Southampton National Institute for Health Research, Biomedical Research Centre, University Hospital Southampton and Southampton General Hospital, Southampton, UK
| | - Giovanni Targher
- Department of Medicine, Section of Endocrinology, Diabetes, and Metabolism, University of Verona, Verona, Italy
| | - Aviad Tur-Sinai
- Department of Health Systems Management, The Max Stern Yezreel Valley College, Yezreel Valley, Israel
| | - Damon Barrett
- School of Public Health and Community Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
| | | | - Tatjana Reic
- European Liver Patients Organization, Brussels, Belgium; Croatian Society for Liver Diseases-Hepatos, Split, Croatia
| | | | - Tim Rhodes
- London School of Hygiene & Tropical Medicine, London, UK
| | - Carla Treloar
- Centre for Social Research in Health, University of New South Wales, Sydney, NSW, Australia
| | - Claus Petersen
- Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
| | - Christoph Schramm
- Martin Zeitz Center for Rare Diseases, Hamburg Center for Translational Immunology (HCTI), and First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany; European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany
| | - Robert Flisiak
- Department of Infectious Diseases and Hepatology, Medical University of Białystok, Poland
| | - Marieta Y Simonova
- Department of Gastroenterology, HPB Surgery and Transplantation, Clinic of Gastroentrology, Military Medical Academy, Sofia, Bulgaria
| | - Albert Pares
- Liver Unit, Hospital Clinic of Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; CIBEREHD, Madrid, Spain
| | - Philip Johnson
- Department of Molecular and Clinical Cancer Medicine, Institute of Systems, Molecular and Integrative Biology, University of Liverpool, Liverpool, UK
| | - Alessandro Cucchetti
- Department of Medical and Surgical Sciences-DIMEC, Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - Isabel Graupera
- Liver Unit, Hospital Clinic of Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; CIBEREHD, Madrid, Spain; Faculty of Medicine and Health Sciences, University of Barcelona, Spain
| | - Christos Lionis
- Clinic of Social and Family Medicine, Medical School, University of Crete, Heraklion, Greece
| | - Elisa Pose
- Liver Unit, Hospital Clinic of Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Faculty of Medicine and Health Sciences, University of Barcelona, Spain
| | - Núria Fabrellas
- Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; CIBEREHD, Madrid, Spain; Faculty of Medicine and Health Sciences, University of Barcelona, Spain
| | - Ann T Ma
- Liver Unit, Hospital Clinic of Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
| | - Juan M Mendive
- Prevention and Health Promotion Research Network (redIAPP), Institute of Health Carlos III, Madrid, Spain; La Mina Health Centre, Catalan Institute of Health (ICS), Barcelona, Spain
| | - Vincenzo Mazzaferro
- HPB Surgery and Liver Transplantation, Istituto Nazionale Tumori IRCCS Foundation (INT), Milan, Italy; Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy
| | - Harry Rutter
- Department of Social and Policy Sciences, University of Bath, Bath, UK
| | - Helena Cortez-Pinto
- Clínica Universitária de Gastrenterologia and Laboratório de Nutrição, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Deirdre Kelly
- Liver Unit, Birmingham Women's and Children's Hospital and University of Birmingham, UK
| | - Robyn Burton
- Alcohol, Drugs, Tobacco and Justice Division, Public Health England, London, UK
| | - Jeffrey V Lazarus
- Barcelona Institute for Global Health (ISGlobal), Hospital Clínic, Barcelona, Spain
| | - Pere Ginès
- Liver Unit, Hospital Clinic of Barcelona, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; CIBEREHD, Madrid, Spain; Faculty of Medicine and Health Sciences, University of Barcelona, Spain
| | - Maria Buti
- CIBEREHD del Instituto de Salud Carlos III, Madrid, Spain; Liver Unit, Hospital Universitario Valle Hebron, Barcelona, Spain
| | - Philip N Newsome
- National Institute for Health Research Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust, University of Birmingham, Birmingham, UK
| | - Patrizia Burra
- Multivisceral Transplant Unit, Gastroenterology, Department of Surgery, Oncology and Gastroenterology, Padua University Hospital, Padua, Italy
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Yang C, Xing H, Tan B, Zhang M. Immune Characteristics in Biliary Atresia Based on Immune Genes and Immune Cell Infiltration. Front Pediatr 2022; 10:902571. [PMID: 35676907 PMCID: PMC9168997 DOI: 10.3389/fped.2022.902571] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2022] [Accepted: 04/25/2022] [Indexed: 11/13/2022] Open
Abstract
BACKGROUND Biliary atresia (BA) is a serious biliary disease in infancy. Jaundice is the most visual and prominent symptom, and it mainly involves bile duct cells leading to the loss of intrahepatic and extrahepatic bile ducts. If left untreated, it will eventually progress to liver cirrhosis. The pathogenesis of BA is not clear, and it is now generally accepted that BA is an autoimmune disease. However, few studies have revealed the infiltration of immune cells in the liver of BA from a global perspective. We used liver tissue sequencing data to predict the infiltration and relative content of immune cells in BA. METHODS The BA datasets GSE46960, GSE15235, and GSE84044, and patient information were downloaded from the Gene Expression Omnibus (GEO) database. After batch normalization, the differentially expressed immune genes (DE-IGs) in BA liver, normal liver, and hepatitis B liver were analyzed with the cut-off value of |log2fold change (log2FC)| >1 and false discovery rate (FDR) <0.05. CIBERSORT software was used to predict the proportions of 22 immune cells in all samples of the datasets. RESULTS 73 DE-IGs have been screened out between BA and normal tissue; among them, 20 genes were highly expressed and another 53 were expressed at a low level. A total of 30 DE-IGs existed between inflammation and fibrosis livers of BA, and all of them were expressed at low levels in fibrosis livers of BA. In GO term analysis, these DE-IGs were mainly associated with the MHC protein complex, cytokine, chemokine activity, and MHC-II receptor activity. In KEGG pathway analysis, the DE-IGs were mainly enriched in pathways of Th1 and Th2 cell differentiation, Th17 cell differentiation, IL-17 signaling pathway, Toll-like receptor signaling pathway, TNF signaling pathway, and autoimmune diseases. There were significant differences in immune infiltration among different pathological types of BA, and there were also obvious differences in immune infiltration of hepatitis B as a disease control of BA. CONCLUSION Based on immune genes and immune cell infiltration, this study reveals the immune characteristics of BA from a global point of view, which provides a new perspective for understanding the pathogenesis of BA and provides a direction for the diagnosis and treatment of BA.
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Affiliation(s)
- Chenyu Yang
- Department of Hepatobiliary Surgery Children's Hospital of Chongqing Medical University, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China.,Chongqing Higher Institution Engineering Research Center of Children's Medical Big Data Intelligent Application, Chongqing, China
| | - Huiwu Xing
- Department of Hepatobiliary Surgery Children's Hospital of Chongqing Medical University, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China.,Chongqing Higher Institution Engineering Research Center of Children's Medical Big Data Intelligent Application, Chongqing, China
| | - Bingqian Tan
- Department of Hepatobiliary Surgery Children's Hospital of Chongqing Medical University, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China.,Chongqing Higher Institution Engineering Research Center of Children's Medical Big Data Intelligent Application, Chongqing, China
| | - Mingman Zhang
- Department of Hepatobiliary Surgery Children's Hospital of Chongqing Medical University, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China.,Chongqing Higher Institution Engineering Research Center of Children's Medical Big Data Intelligent Application, Chongqing, China
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Weng Z, Ye F, Zhou L, Chen F, Ling W, Fang Y, Liu M, Wu Q, Qiu X, Lyu G. A nomogram model based on preoperative grey-scale US features and routine serum biomarkers to predict the outcome of infants with biliary atresia after Kasai portoenterostomy. Front Pediatr 2022; 10:972855. [PMID: 36340714 PMCID: PMC9631934 DOI: 10.3389/fped.2022.972855] [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: 06/19/2022] [Accepted: 09/27/2022] [Indexed: 11/13/2022] Open
Abstract
OBJECTIVE To establish a nomogram to predict the outcome of biliary atresia (BA) infants 3-months post- Kasai portoenterostomy (KPE). METHODS BA Infants who underwent KPE from two hospitals were included in the training (n = 161) and validation cohorts (n = 64). A logistic regression equation (Equation A) for predicting the serum total bilirubin (TBIL) level 3-month post-KPE was established in the training cohort. Then, a nomogram was developed based on Equation A in the training cohort and validated in the validation cohort. Moreover, a new equation (Equation B) was generated based on the nomogram and the size of the enlarged hilar lymph nodes (LNs) in the validation cohort. The predictive performance of the nomogram was evaluated by the receiver operating characteristic (ROC) curve and by calculating the area under the ROC curve (AUC), sensitivity, specificity, and positive (PPV) and negative (NPV) prediction values. RESULTS A nomogram based on gallbladder morphology and serum levels of TBIL and total protein (TP) was established with AUC (95%CI) of 0.673 (0.595, 0.745) and 0.647 (0.518, 0.763), sensitivity (95%CI) of 71.4% (62.1%,79.6%) and 81.8% (59.7%,94.8%), specificity (95%CI) of 63.3% (48.3%,76.6%) and 47.6% (32.0%,63.6%), PPV (95%CI) of 81.6% (72.5%,88.9%) and 45.0% (29.3%,61.5%), and NPV (95%CI) 49.2% (36.4%,62.1%) and 83.3% (62.6%,95.3%), respectively, in the training and validation cohorts. Furthermore, in the validation cohort, the AUC (95%CI) of Equation B was 0.798 (95%CI: 0.679, 0.888), which was significantly higher than that of the nomogram (P = 0.042). CONCLUSION A nomogram based on the pre-KPE gallbladder morphology, TBIL, and TP to predict the outcome of BA 3-months post-KPE is established. Moreover, the addition of the size of the enlarged hilar LNs into the nomogram further improves its predictive value.
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Affiliation(s)
- Zongjie Weng
- Department of Medical Ultrasonics, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China.,Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Fengying Ye
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Fa Chen
- Department of Epidemiology and Health Statistics, School of Public Health, Fujian Medical University, Fuzhou, China
| | - Wen Ling
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Yifan Fang
- Department of Pediatric Surgery, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Min Liu
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Qiumei Wu
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Xiuqing Qiu
- Department of Gynecology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Guorong Lyu
- Department of Medical Ultrasonics, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China.,Department of Clinical Medicine, Quanzhou Medical College, Quanzhou, China
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Zhou W, Zhou L. Ultrasound for the Diagnosis of Biliary Atresia: From Conventional Ultrasound to Artificial Intelligence. Diagnostics (Basel) 2021; 12:diagnostics12010051. [PMID: 35054217 PMCID: PMC8775261 DOI: 10.3390/diagnostics12010051] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2021] [Revised: 12/08/2021] [Accepted: 12/10/2021] [Indexed: 12/21/2022] Open
Abstract
Biliary atresia is an aggressive liver disease of infancy and can cause death without timely surgical intervention. Early diagnosis of biliary atresia is critical to the recovery of bile drainage and long-term transplant-free survival. Ultrasound is recommended as the initial imaging strategy for the diagnosis of biliary atresia. Numerous ultrasound features have been proved helpful for the diagnosis of biliary atresia. In recent years, with the help of new technologies such as elastography ultrasound, contrast-enhanced ultrasound and artificial intelligence, the diagnostic performance of ultrasound has been significantly improved. In this review, various ultrasound features in the diagnosis of biliary atresia are summarized. A diagnostic decision flow chart for biliary atresia is proposed on the basis of the hybrid technologies, combining conventional ultrasound, elastography and contrast-enhanced ultrasound. In addition, the application of artificial intelligence in the diagnosis of biliary atresia with ultrasound images is also introduced.
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Affiliation(s)
| | - Luyao Zhou
- Correspondence: ; Tel.: +86-134-2753-9467
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Affiliation(s)
- Rachel Harwood
- Department of Paediatric Surgery, Alder Hey Children's Hospital NHS Foundation Trust, Liverpool, UK
- Institute of Systems and Molecular Biology, University of Liverpool, Liverpool, UK
| | - Paul D Losty
- Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, UK
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Ranucci G, Di Dato F, Liccardo D, Spada M, Maggiore G, Iorio R. An Unexpected Hepatic Hydrothorax After a Successful Kasai Portoenterostomy: A Case Report. Front Pediatr 2021; 9:766187. [PMID: 34790637 PMCID: PMC8592304 DOI: 10.3389/fped.2021.766187] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2021] [Accepted: 09/30/2021] [Indexed: 11/13/2022] Open
Abstract
Hepatic hydrothorax (HH) represents a rare complication of portal hypertension among adult cirrhotic patients. Here, we describe a pediatric case of HH, observed in a biliary atresia infant. The child presented with recurrent right-sided pleural effusion, after a successful Kasai portoenterostomy with restoration of bile flow and without overt signs of hepatic failure. Recurrence of HH led the patient to liver transplant despite a low pediatric end-stage liver disease value. Although rare, HH can also occur in children and should be suspected in patients with portal hypertension and respiratory distress. HH may be an indication for liver transplantation.
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Affiliation(s)
- Giulia Ranucci
- Section of Pediatrics, Department of Translational Medical Science, University of Naples Federico II, Naples, Italy
| | - Fabiola Di Dato
- Section of Pediatrics, Department of Translational Medical Science, University of Naples Federico II, Naples, Italy
| | - Daniela Liccardo
- Hepatology, Gastroenterology, Nutrition and Liver Transplant Unit, IRCCS Bambino Gesù Pediatric Hospital, Rome, Italy
| | - Marco Spada
- Division of Hepatobiliopancreatic Surgery, Liver and Kidney Transplantation, IRCCS Bambino Gesù Pediatric Hospital, Rome, Italy
| | - Giuseppe Maggiore
- Hepatology, Gastroenterology, Nutrition and Liver Transplant Unit, IRCCS Bambino Gesù Pediatric Hospital, Rome, Italy
| | - Raffaele Iorio
- Section of Pediatrics, Department of Translational Medical Science, University of Naples Federico II, Naples, Italy
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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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Maguire C, Tyack Z, Kimble RM, Griffin BR, Yesberg HJ, Choo FM, McBride CA. What to measure in biliary atresia research: study protocol for developing a core outcome set. BMJ Open 2021; 11:e047224. [PMID: 34385246 PMCID: PMC8362727 DOI: 10.1136/bmjopen-2020-047224] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
AIM Extrahepatic biliary atresia is a rare disorder. This creates challenges in the quality and quantity of research conducted. This issue is exacerbated by the potential heterogeneity in the reported outcomes in research examining the management of biliary atresia. A core outcome set is required to standardise reporting on the management of biliary atresia in research, facilitate systematic reviews that include outcomes of greatest importance to patients and clinicians, and to evaluate the quality of the existing evidence base on the management of biliary atresia. METHODS A list of all potential outcomes will be developed through a systematic review of the literature. This list will be refined through a three-stage Delphi approach, involving key stakeholders in the management of biliary atresia. This will include patients and their parents, clinicians, nurses and allied health professionals. In this way, outcomes will be prioritised into a set of consensus core outcomes. CONCLUSION The development of a core outcome set in biliary atresia management is needed to guide future research and assist in evaluating the quality of existing research. ETHICS AND DISSEMINATION Ethical approval has been granted by the Children's Health Queensland Hospital and Health Service Human Research Ethics Committee (HREC), Ref: HREC/20/QCHQ/62448. Results of the study will be published in an open access format.
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Affiliation(s)
| | - Zephanie Tyack
- Centre for Children's Burns and Trauma Research, University of Queensland, South Brisbane, Queensland, Australia
| | - Roy M Kimble
- Lady Cilento Children's Hospital, Centre for Children's Burns & Trauma Research, Brisbane, Queensland, Australia
| | - Bronwyn Ruth Griffin
- Centre for Children's Burns and Trauma, Queensland University of Technology Faculty of Health, Kelvin Grove, Queensland, Australia
- Pegg Leditschke Children's Burns Centre, Children's Health Queensland, South Brisbane, Queensland, Australia
| | | | - Fang Ming Choo
- Faculty of Science, University of Queensland, Brisbane, Queensland, Australia
| | - Craig Antony McBride
- Lady Cilento Children's Hospital, Queensland Health, Brisbane, Queensland, Australia
- School of Medicine, University of Queensland, Brisbane, Queensland, Australia
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40
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Zhou W, Li X, Zhang N, Liao B, Xie X, Zhang X, Wang G, Zhou L. The combination of conventional ultrasound and shear-wave elastography in evaluating the segmental heterogeneity of liver fibrosis in biliary atresia patients after Kasai portoenterostomy. Pediatr Surg Int 2021; 37:1099-1108. [PMID: 33966098 DOI: 10.1007/s00383-021-04920-3] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/28/2021] [Indexed: 12/17/2022]
Abstract
OBJECTIVE To retrospectively assess the value of the combination of conventional ultrasound and shear-wave elastography (SWE) in evaluating the segmental heterogeneity of liver fibrosis in biliary atresia (BA) patients after Kasai portoenterostomy. METHODS A total of 35 BA patients with liver segmental deformation were enrolled. The segmental deformation was assessed by conventional ultrasound followed with SWE examinations for evaluating the liver stiffness. Liver biopsy was performed in 11 patients in the region of SWE measurement and liver fibrosis was assessed using the Metavir classification. Aminotransferase to platelet ratio index (APRI) was calculated for comparison. The correlations between serum biochemical tests with SWE values were evaluated. Spearman's rank coefficient test was performed to evaluate the correlation between variables. RESULTS The SWE values of the biopsy segments had significant positive correlations with liver fibrosis severity (r = 0.828, p = 0.001), which was better than APRI (r = 0.366, p = 0.242). The levels of bilirubin and transaminase showed significant correlations with the SWE values at hypertrophic segments in all patients (r from 0.336 to 0.576, all p < 0.05). CONCLUSIONS Awareness of the segmental heterogeneity of liver fibrosis evaluated by conventional ultrasound and SWE may assist in selecting an appropriate biopsy location and predicting postoperative surveillance for patients with BA.
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Affiliation(s)
- Wenying Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Xiaoju Li
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Nan Zhang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Bing Liao
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Xiaoyan Xie
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Xiaoer Zhang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Guotao Wang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China.
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Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart. Pediatr Radiol 2021; 51:1545-1554. [PMID: 33974103 DOI: 10.1007/s00247-021-05034-7] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2020] [Revised: 11/23/2020] [Accepted: 02/21/2021] [Indexed: 12/11/2022]
Abstract
We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. In this second part, we discuss the roles of magnetic resonance (MR) cholecystopancreatography, hepatobiliary scintigraphy, percutaneous biopsy and percutaneous cholecysto-cholangiography. Among imaging techniques, ultrasound (US) signs have a high specificity, although a normal US examination does not rule out biliary atresia. Other imaging techniques with direct opacification of the biliary tree combined with percutaneous liver biopsy have roles in equivocal cases. MR cholecystopancreatography and hepatobiliary scintigraphy are not useful for the diagnosis of biliary atresia. We propose a decisional flowchart for biliary atresia diagnosis based on US signs, including elastography, percutaneous cholecysto-cholangiography or endoscopic retrograde cholangiopancreatography and liver biopsy.
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Goh L, Phua KB, Low Y, Chiang LW, Yong C, Chiou FK. Analysis of Cholangitis Rates with Extended Perioperative Antibiotics and Adjuvant Corticosteroids in Biliary Atresia. Pediatr Gastroenterol Hepatol Nutr 2021; 24:366-376. [PMID: 34316471 PMCID: PMC8279824 DOI: 10.5223/pghn.2021.24.4.366] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/17/2020] [Revised: 03/24/2021] [Accepted: 05/15/2021] [Indexed: 12/14/2022] Open
Abstract
PURPOSE There is no consensus regarding adjuvant therapies following Kasai portoenterostomy (KP) for biliary atresia (BA). This study aimed to analyze the effect of extended perioperative intravenous antibiotics (PI-Abx) and adjuvant corticosteroid on cholangitis and jaundice clearance rates in the 3 years post-KP in children with BA. METHODS Data of patients who underwent KP between 1999-2018 at a single center were retrospectively analyzed. Group A (1999-2010) received PI-Abx for 5 days, Group B (2010-2012) received PI-Abx for 5 days plus low-dose prednisolone (2 mg/kg), and Group C (2012-2017) received PI-Abx for 14 days plus high-dose prednisolone (5 mg/kg). RESULTS Fifty-four patients were included with groups A, B, and C comprising 25, 9, and 20 patients, respectively. The number of episodes of cholangitis was 1.0, 1.6, and 1.3 per patient (p=NS) within the first year and 1.8, 2.3, and 1.7 (p=NS) over 3 years in Groups A, B, and C, respectively. The jaundice clearance rate at 6 months was 52%, 78%, and 50% (p=NS), and the 3-year native liver survival (NLS) rate was 76%, 100%, and 80% (p=NS) in Groups A, B, and C, respectively. A near-significant association was observed between the incidence of cholangitis within the first year and decompensated liver cirrhosis/death at 3 years post KP (p=0.09). Persistence of jaundice at 6 months was significantly associated with decompensated cirrhosis/death at 3 years (p<0.001). CONCLUSION The extended duration of PI-Abx and adjuvant corticosteroids was not associated with improved rates of cholangitis, jaundice clearance, or NLS in patients with BA.
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Affiliation(s)
- Lynette Goh
- Gastroenterology, Hepatology & Nutrition Service, Pediatric Medicine, KK Women's and Children's Hospital, Singapore
| | - Kong Boo Phua
- Gastroenterology, Hepatology & Nutrition Service, Pediatric Medicine, KK Women's and Children's Hospital, Singapore
| | - Yee Low
- Department of Pediatric Surgery, KK Women's and Children's Hospital, Singapore
| | - Li Wei Chiang
- Department of Pediatric Surgery, KK Women's and Children's Hospital, Singapore
| | - Chen Yong
- Department of Pediatric Surgery, KK Women's and Children's Hospital, Singapore
| | - Fang Kuan Chiou
- Gastroenterology, Hepatology & Nutrition Service, Pediatric Medicine, KK Women's and Children's Hospital, Singapore
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Liang J, Li H, Fu J, Liang Q, Liu T, Yang F, Zhang B, Bai X, Wen Z. A model incorporating serum C3 complement levels may be useful for diagnosing biliary atresia in infants. GASTROENTEROLOGIA Y HEPATOLOGIA 2021; 45:47-58. [PMID: 34029644 DOI: 10.1016/j.gastrohep.2021.02.020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/27/2020] [Revised: 02/01/2021] [Accepted: 02/08/2021] [Indexed: 11/15/2022]
Abstract
INTRODUCTION Correctly identifying patients with biliary atresia (BA), while avoiding invasive diagnostic methods is challenging. The purpose of this study was to determine the value of serum immune indicators for distinguishing BA from other causes of cholestasis in infants. PATIENTS AND METHODS The data of infants with a surgical/histological diagnosis of BA and those with other causes of cholestatic jaundice were retrospectively analyzed. Patients were divided into a BA group and a cholestasis control (CC) group. Biochemical parameters, major lymphocyte subsets, immunoglobin and C3 and C4 complement levels were compared between the groups. RESULTS A total of 129 infants with BA and 63 with other causes of cholestasis (CC control group) with a median age of 2.2 months were included in the analysis. The levels of CD3+ T cells, CD3+CD4+ T cells, and premature T cells and the levels of C3 and C4 were all significantly higher in the BA group compared to the CC group (all P<0.05). Pair-wise correlation analyses indicated that C3 and C4 had a significant positive correlation with γ-GT in the BA group, but not in the CC group. Five indices were found to be significantly associated with BA: stool color, globulin, γ-GT, C3 and C4. A model incorporating stool color, gamma-glutamyl transpeptidase level, and C3 level exhibited an area under the ROC curve (AUC) of 0.93, and a sensitivity of 93% and specificity of 83% for the diagnosis of BA. CONCLUSIONS Models incorporating serum C3 levels may be useful for accurately diagnosing BA in infants.
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Affiliation(s)
- Jiankun Liang
- Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Huixian Li
- Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Jie Fu
- Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Qifeng Liang
- Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Tao Liu
- Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Fengxia Yang
- Isolation Clinic, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Bingbing Zhang
- Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Xiaoling Bai
- Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Zhe Wen
- Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
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Oh C, Lee S, Chang HK, Ahn SM, Chae K, Kim S, Kim S, Seo JM. Analysis of Pediatric Surgery Using the National Healthcare Insurance Service Database in Korea: How Many Pediatric Surgeons Do We Need in Korea? J Korean Med Sci 2021; 36:e116. [PMID: 33975393 PMCID: PMC8111040 DOI: 10.3346/jkms.2021.36.e116] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2021] [Accepted: 03/25/2021] [Indexed: 11/20/2022] Open
Abstract
BACKGROUND In the past, general surgeons (GSs) without a pediatric surgical subspecialty often performed surgery on children and, even now, GSs are performing many pediatric surgeries. We aimed to investigate the involvement of pediatric surgeons (PSs) and GSs in pediatric surgery, compare the outcomes of surgery in the neonatal intensive care unit (NICU), and estimate the appropriate PS workforce in Korea. METHODS We used surgical data from the National Health Insurance Service database that was collected from patients under the age of 19 years in hospitals nationwide from January 2002 to December 2017. In this database, we found 37 hospitals where PSs worked by using the index operation (congenital diaphragmatic hernia, esophageal atresia, hypertrophic pyloric stenosis, Hirschsprung's disease, abdominal wall defect, jejunoileal atresia, malrotation, anorectal malformation, and biliary atresia). It was assumed that the surgery in the 37 hospitals was performed by PS and that the surgery in other hospitals was performed by GS. Mortality was analyzed to compare the outcomes of acute abdominal surgery in the NICU. We estimated the number of PS currently needed in Korea for each situation under the assumption that PS would perform all operations for the index operation, main pediatric diseases (index operation + gastroesophageal reflux disease, choledochal cyst, inguinal hernia, and appendicitis), acute abdominal surgery in the NICU, and all pediatric surgeries. Additionally, we estimated the appropriate number of PS required for more advanced pediatric surgery in the future. RESULTS The number of pediatric surgeries from 2002 to 2017 increased by 124%. Approximately 10.25% of the total pediatric surgeries were performed by PSs, and the percentage of the surgery performed by PSs increased from 8.32% in 2002 to 15.92% in 2017. The percentage of index operations performed by PSs annually was 62.44% in average. It was only 47.81% in 2002, and increased to 88.79% in 2017. During the last 5 years of the study period, the average annual number of surgeries for main pediatric diseases was approximately 33,228. The ratio of the number of surgeries performed by PS vs. GS steadily increased in main pediatric diseases, however, the ratio of the number of surgery performed by PS for inguinal hernia and appendicitis remained low in the most recent years. The percentage of the number of acute abdominal surgery performed by PS in the NICU was 44% in 2002, but it had recently risen to 89.7%. After 30 days of birth, mortality was significantly lower in all groups that were operated on by PS, rather than GS, during the last 5 years. In 2019, 49 PSs who were under the age of 65 years were actively working in Korea. Assuming that all pediatric surgeries of the patients under the age of 19 years should be performed by PS, the minimum number of PS currently required was about 63 if they perform all of the index operations, the main pediatric surgery was about 209, the NICU operation was about 63, and the all pediatric surgeries was about 366. Additionally, it was determined that approximately 165 to 206 PS will be appropriate for Korea to implement more advanced pediatric surgery in the future. CONCLUSION The proportion of the pediatric surgery performed by PS rather than GS is increasing in Korea, but it is still widely performed by GS. PSs have better operative outcomes for acute abdominal surgery in the NICU than GSs. We believe that at least the index operation or the NICU operation should be performed by PS for better outcome, and that a minimum of 63 PSs are needed in Korea to do so. In addition, approximately 200 PSs will be required in Korea in order to manage main pediatric diseases and to achieve more advanced pediatric surgery in the future.
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Affiliation(s)
- Chaeyoun Oh
- Department of Pediatric Surgery, Korea University Ansan Hospital, Korea University College of Medicine, Seoul, Korea
| | - Sanghoon Lee
- Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Hye Kyung Chang
- Department of Pediatric Surgery, Kyung Hee University Hospital, Kyung Hee University College of Medicine, Seoul, Korea
| | - Soo Min Ahn
- Department of Pediatric Surgery, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
| | - Kyunghee Chae
- Department of Preventive Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Sujeong Kim
- Department of Preventive Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Sukil Kim
- Department of Preventive Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Jeong Meen Seo
- Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
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The Burden of Disease and Pathology at a Rapidly Expanding Tertiary Paediatric Surgical Unit in South Africa. World J Surg 2021; 45:2378-2385. [PMID: 33950352 DOI: 10.1007/s00268-021-06144-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/10/2021] [Indexed: 02/01/2023]
Abstract
BACKGROUND The precise burden of paediatric surgical care in South Africa is unknown. In the absence of epidemiological data, hospital-based study is a first step to gauge the burden and profile of paediatric surgical disease. We aim to describe the profile of pathology, pattern of referrals, and complications of paediatric surgical care at Chris Hani Baragwanath Academic Hospital (CHBAH). METHODS A 1-year retrospective record review for the period 3/1/2019 to 1/1/2020 was conducted by evaluation of the morbidity and mortality databases of the Department of Paediatric Surgery (DPS). Number of admissions, consultations, complications, and surgeries performed were analysed and classified. RESULTS A total of 11,932 unique patient encounters occurred. Emergencies (79%, 1841/2329) accounted for the majority of admissions. Trauma accounted for 49% (896/1841) of emergency admissions. Elective surgery constituted 52% (1202/2316) and emergency surgery 48% (1114/2316) of all procedures performed. The emergency department (55%, 1271/2329), outpatients department (19%, 447/2329), and peripheral hospitals (16%, 378/2329) were the source of the majority of admissions. A complication rate of 9% (208/2316) was observed. CONCLUSION The high-volume subspecialist environment at CHBAH presents the ideal environment for delivery of specialist paediatric surgical services and training. Injury prevention, optimal use of existing resources, and additional physical, human and financial resources are required to meet the existing and predicted future burden of paediatric surgical disease.
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van der Schyff F, Terblanche AJ, Botha JF. Improving Poor Outcomes of Children With Biliary Atresia in South Africa by Early Referral to Centralized Units. JPGN REPORTS 2021; 2:e073. [PMID: 37207069 PMCID: PMC10191604 DOI: 10.1097/pg9.0000000000000073] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/30/2020] [Accepted: 02/18/2021] [Indexed: 05/21/2023]
Abstract
Biliary atresia (BA) is a progressive fibrosing cholangiopathy of infancy, the most common cause of cholestatic jaundice in infants and the top indication for liver transplantation in children. Kasai portoenterostomy (KPE) when successful may delay the requirement for liver transplantation, which in the majority offers the only cure. Good outcomes demand early surgical intervention, appropriate management of liver cirrhosis, and in most cases, liver transplantation. These parameters were audited of children with BA treated at the Steve Biko Academic Hospital (SBAH) in Pretoria, South Africa. Methods All children with BA who were managed at SBAH between June 2007 and July 2018 were included. Parameters measured centered on patient demographics, timing of referral and surgical intervention, immediate and long-term outcomes of surgery, and follow-up management. Results Of 104 children treated, 94 (90%) were KPE naive. Only 23/86 (26%) of children were referred before 60 days of life and 42/86 (49%) after 120 days. Median time to surgical assessment and surgery was 4 (IQR 1-70) and 5 (IQR 1-27) days post presentation, respectively. The median age at KPE was 91 days (IQR 28-165), with only 4/41 (12%) of KPEs performed before 60 days of life. Of those with recorded outcomes, 12/33 (36%) achieved resolution of jaundice. Only a third of the cohort were referred for transplantation. Conclusion Children with BA have poor outcomes in the public health sector in South Africa. Late referrals, delayed diagnostics, advanced age at KPE with low drainage rates, poor follow-up, and low transplant rates account for low survival. Early referral to units offering expert intervention at all stages of care, including transplantation, would offer the best outcomes.
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Affiliation(s)
- Francisca van der Schyff
- From the Department of Transplantation, Wits Donald Gordon Medical Center, University of Witwatersrand, Johannesburg, South Africa
| | - Alberta J. Terblanche
- Department of Paediatrics, School of Clinical Medicine, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa
| | - Jean F. Botha
- Department of Transplantation, Wits Donald Gordon Medical Center, University of Witwatersrand, Johannesburg, South Africa
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Operational Definitions for Chronic Liver Disease Manifestations and Recurring Clinical Events in Biliary Atresia. J Pediatr Gastroenterol Nutr 2021; 72:584-591. [PMID: 33306582 DOI: 10.1097/mpg.0000000000002992] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
BACKGROUND The disease course of biliary atresia (BA) may be complicated by development of chronic liver disease (CLD) manifestations (eg, ascites) and recurring clinical events (eg, cholangitis). Most pediatric CLD-manifestations/events lack standardized operational definitions, leading to inconsistent identification in clinical research. This study aimed to develop operational research definitions of CLD-manifestations/events in BA for application in retrospective analysis. METHODS Operational definitions of CLD-manifestations/events were developed by literature review and revised by a panel of experienced pediatric hepatologists. Definitions were applied to a single-center review of infants with BA post-Kasai. Manifestations/events were captured until last clinical visit with native liver (SNL), liver transplantation (LT), or death. Native liver survival and event-free survival were estimated by Kaplan-Meier method. Cluster analysis was performed to identify similar patterns of manifestation/event development. RESULTS Of 65 infants with BA post-Kasai (2006-18; median Kasai 56.8 days; 65% girls), 29 underwent LT (median 12.9 months) and 4 died without LT (median 6.9 months). Seventy-six percent of CLD-manifestations/events presented within the first year. Presence of definite clinically evident portal hypertension, thrombocytopenia, and dCE ascites were associated with poor transplant-free survival (P < 0.01). Similar pattern developments of CLD-manifestations/events identified 3 outcome groups: poor outcomes (87.8% LT/death), SNL with manifestations/events, and SNL with few/no events. CONCLUSIONS Operational definitions can provide a timely description of the disease course progression in infants with BA. Research definitions may provide better consistency in future pediatric CLD studies. Furthermore, definitions may serve as endpoints in therapeutic trials or used as variables for disease pattern identification in potential multicenter studies.
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Zhou W, Yang Y, Yu C, Liu J, Duan X, Weng Z, Chen D, Liang Q, Fang Q, Zhou J, Ju H, Luo Z, Guo W, Ma X, Xie X, Wang R, Zhou L. Ensembled deep learning model outperforms human experts in diagnosing biliary atresia from sonographic gallbladder images. Nat Commun 2021; 12:1259. [PMID: 33627641 PMCID: PMC7904842 DOI: 10.1038/s41467-021-21466-z] [Citation(s) in RCA: 50] [Impact Index Per Article: 12.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2020] [Accepted: 01/27/2021] [Indexed: 02/05/2023] Open
Abstract
It is still challenging to make accurate diagnosis of biliary atresia (BA) with sonographic gallbladder images particularly in rural area without relevant expertise. To help diagnose BA based on sonographic gallbladder images, an ensembled deep learning model is developed. The model yields a patient-level sensitivity 93.1% and specificity 93.9% [with areas under the receiver operating characteristic curve of 0.956 (95% confidence interval: 0.928-0.977)] on the multi-center external validation dataset, superior to that of human experts. With the help of the model, the performances of human experts with various levels are improved. Moreover, the diagnosis based on smartphone photos of sonographic gallbladder images through a smartphone app and based on video sequences by the model still yields expert-level performances. The ensembled deep learning model in this study provides a solution to help radiologists improve the diagnosis of BA in various clinical application scenarios, particularly in rural and undeveloped regions with limited expertise.
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Affiliation(s)
- Wenying Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China
| | - Yang Yang
- School of Computer Science and Engineering, Sun Yat-sen University, Guangzhou, P. R. China
| | - Cheng Yu
- Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, P. R. China
| | - Juxian Liu
- Department of Ultrasound, West China Hospital, Sichuan University, Chengdu, P. R. China
| | - Xingxing Duan
- Department of Ultrasound, Hunan Children's Hospital, Changsha, P. R. China
| | - Zongjie Weng
- Department of Medical Ultrasonics, Fujian Provincial Maternity and Children's Hospital, Affiliated Hospital of Fujian Medical University, Fuzhou City, P. R. China
| | - Dan Chen
- Department of Ultrasound, Guangdong Women and Children' Hospital, Guangzhou, P. R. China
| | - Qianhong Liang
- Department of Ultrasound, Hexian Memorial Affiliated Hospital of Southern Medical University, Guangzhou, P. R. China
| | - Qin Fang
- Department of Ultrasound, The First People's Hospital of Foshan, Foshan City, P. R. China
| | - Jiaojiao Zhou
- Department of Ultrasound, West China Hospital, Sichuan University, Chengdu, P. R. China
| | - Hao Ju
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang, P. R. China
| | - Zhenhua Luo
- Institute of Precision Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China
| | - Weihao Guo
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China
| | - Xiaoyan Ma
- Department of Ultrasound, Guangdong Women and Children' Hospital, Guangzhou, P. R. China
| | - Xiaoyan Xie
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China.
| | - Ruixuan Wang
- School of Computer Science and Engineering, Sun Yat-sen University, Guangzhou, P. R. China.
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China.
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Okubo R, Nio M, Sasaki H. Impacts of Early Kasai Portoenterostomy on Short-Term and Long-Term Outcomes of Biliary Atresia. Hepatol Commun 2021; 5:234-243. [PMID: 33553971 PMCID: PMC7850309 DOI: 10.1002/hep4.1615] [Citation(s) in RCA: 33] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2020] [Revised: 08/31/2020] [Accepted: 09/12/2020] [Indexed: 02/04/2023] Open
Abstract
There are discrepancies regarding the clinical impact of age at Kasai portoenterostomy (KP) on surgical outcomes. Hence, we re-assessed the clinical significance of age at KP. We analyzed 224 patients with type III (atresia of bile duct at the porta hepatis) biliary atresia at Tohoku University Hospital. We classified patients into two groups: KP at ≤60 days of age (group TE) and >60 days of age (group TL). Group TE was subdivided into three groups (TE1, TE2, and TE3) according to age at time of surgery. Subsequently, 2,643 patients in the Japanese Biliary Atresia Registry were classified similarly. Background and surgical outcomes were compared. Of the 2,643 cases, 323 patients who underwent revision KP were analyzed separately. The jaundice clearance rates (JCRs) were 81.4%, 100%, 64.7%, 83.0%, and 65.2% of patients in the TE, TE1, TE2, TE3, and TL groups, respectively. The 15-year native liver survival rates of patients in the TE, TE1, TE2, TE3, and TL groups were 62.2%, 88.9%, 33.9%, 64.4%, and 42.9%, respectively. The 30-year native liver survival rates of patients in the TE, TE1, TE2, TE3, and TL groups were 38.6%, 74.1%, 25.4%, 35.8%, and 31.7%, respectively. The JCRs were 66.2%, 69.4%, 64.1%, 66.7%, and 59.7% for patients in groups JE, JE1, JE2, JE3, and JL, respectively. The 15-year native liver survival rates were 48.1%, 56.7%, 43.9%, 48.9%, and 37.2% for patients in groups JE, JE1, JE2, JE3, and JL, respectively. The JCRs following revision KP were higher in the JE1 group than in the other groups. Conclusion: Early KP was associated with favorable outcomes except in patients aged 31-45 days.
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Affiliation(s)
- Ryuji Okubo
- Department of Pediatric SurgeryTohoku University Graduate School of MedicineSendaiMiyagiJapan.,The Japanese Biliary Atresia SocietyOffice of the Japanese Biliary Atresia SocietyDepartment of Pediatric SurgeryTohoku University Graduate School of MedicineSendaiMiyagiJapan
| | - Masaki Nio
- Department of Pediatric SurgeryTohoku University Graduate School of MedicineSendaiMiyagiJapan.,The Japanese Biliary Atresia SocietyOffice of the Japanese Biliary Atresia SocietyDepartment of Pediatric SurgeryTohoku University Graduate School of MedicineSendaiMiyagiJapan
| | - Hideyuki Sasaki
- Department of Pediatric SurgeryTohoku University Graduate School of MedicineSendaiMiyagiJapan.,The Japanese Biliary Atresia SocietyOffice of the Japanese Biliary Atresia SocietyDepartment of Pediatric SurgeryTohoku University Graduate School of MedicineSendaiMiyagiJapan
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Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol 2021; 51:314-331. [PMID: 33201318 DOI: 10.1007/s00247-020-04840-9] [Citation(s) in RCA: 31] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2020] [Revised: 06/16/2020] [Accepted: 09/07/2020] [Indexed: 12/27/2022]
Abstract
We present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses towards biliary cirrhosis, end-stage liver failure and death by age 3. Differentiation of biliary atresia from other nonsurgical causes of neonatal cholestasis is challenging because there is no single method for diagnosing biliary atresia, and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. Concerning imaging, our systematic literature review shows that ultrasonography is the main tool for pre- and neonatal diagnosis. Key prenatal features, when present, are non-visualisation of the gallbladder, cyst in the liver hilum, heterotaxy syndrome and irregular gallbladder walls. Postnatal imaging features have a very high specificity when present, but a variable sensitivity. Triangular cord sign and abnormal gallbladder have the highest sensitivity and specificity. The presence of macro- or microcyst or polysplenia syndrome is highly specific but less sensitive. The diameter of the hepatic artery and hepatic subcapsular flow are less reliable. When present in the context of acholic stools, dilated intrahepatic bile ducts rule out biliary atresia. Importantly, a normal US exam does not rule out biliary atresia. Signs of chronic hepatopathy and portal hypertension (portosystemic derivations such as patent ductus venosus, recanalised umbilical vein, splenomegaly and ascites) should be actively identified for - but are not specific for - biliary atresia.
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