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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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Ye F, Ling W, Wu Q, Ma H, Huang Z, Fang Y, Lyu G, Weng Z. Porta hepatis lymph nodes on US: not only identify biliary atresia but also predict outcomes after Kasai portoenterostomy surgery. Insights Imaging 2024; 15:154. [PMID: 38900331 PMCID: PMC11189885 DOI: 10.1186/s13244-024-01735-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Accepted: 06/02/2024] [Indexed: 06/21/2024] Open
Abstract
OBJECTIVES To evaluate the usefulness of porta hepatis lymph nodes (PHLNs) on ultrasonography (US) scans in diagnosing biliary atresia (BA) and predicting the outcomes after Kasai portoenterostomy (KPE) surgery. METHODS A total of 668 patients from one hospital were enrolled in the study (542 non-BA and 126 BA). The independent and combined diagnostic efficacy of PHLNs, triangular cord (TC) thickness, and gallbladder morphology were assessed by drawing the receiver operating characteristic (ROC) curves and counting the area under the ROC curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). The US features, histopathological findings of PHLNs, and serum total bilirubin (TBIL) levels 3 months post-KPE were correlated. RESULTS The AUC, sensitivity, specificity, PPV, and NPV of PHLNs with hyperechogenicity and a maximum length larger than 8.4 mm were 0.898, 81.8%, 97.8%, 89.6%, and 95.8%, respectively. The combination of PHLNs, TC thickness, and gallbladder morphology achieved the best overall diagnostic efficacy among all indicators with an AUC of 0.927 and a sensitivity of 99.2%. The germinal center number and bile particle number of PHLNs were positively correlated with pathological size and US echogenicity intensity of PHLNs, respectively (r = 0.591, 0.377, p = 0.001, 0.004). The pathological size of PHLNs in BA patients was negatively correlated with jaundice clearance status 3 months after KPE surgery (r = -0.385, p = 0.047). CONCLUSION PHLNs with hyperechogenicity and a maximum length > 8.4 mm are useful US indicators for BA diagnosis. Additionally, the enlargement of PHLNs might play a role in predicting outcomes of KPE surgery. CRITICAL RELEVANCE STATEMENT The article proposed for the first time that PHLNs with hyperechogenicity and a maximum length > 8.4 mm are a useful US indicator for diagnosing BA. KEY POINTS PHLNs may be helpful in diagnosing BA and predicting outcomes after surgery. Enlarged hyperechoic PHLNs are a useful diagnostic indicator for BA, and play a role in predicting surgical outcomes. These findings can assist clinicians in more accurately diagnosing BA, enabling more timely treatments.
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Affiliation(s)
- 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
| | - 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
| | - 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
| | - Hong Ma
- Department of Pathology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Zhen Huang
- Department of Pathology, 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
| | - 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.
| | - Zongjie Weng
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
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Zhou W, Ye Z, Huang G, Zhang X, Xu M, Liu B, Zhuang B, Tang Z, Wang S, Chen D, Pan Y, Xie X, Wang R, Zhou L. Interpretable artificial intelligence-based app assists inexperienced radiologists in diagnosing biliary atresia from sonographic gallbladder images. BMC Med 2024; 22:29. [PMID: 38267950 PMCID: PMC10809457 DOI: 10.1186/s12916-024-03247-9] [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: 08/26/2023] [Accepted: 01/02/2024] [Indexed: 01/26/2024] Open
Abstract
BACKGROUND A previously trained deep learning-based smartphone app provides an artificial intelligence solution to help diagnose biliary atresia from sonographic gallbladder images, but it might be impractical to launch it in real clinical settings. This study aimed to redevelop a new model using original sonographic images and their derived smartphone photos and then test the new model's performance in assisting radiologists with different experiences to detect biliary atresia in real-world mimic settings. METHODS A new model was first trained retrospectively using 3659 original sonographic gallbladder images and their derived 51,226 smartphone photos and tested on 11,410 external validation smartphone photos. Afterward, the new model was tested in 333 prospectively collected sonographic gallbladder videos from 207 infants by 14 inexperienced radiologists (9 juniors and 5 seniors) and 4 experienced pediatric radiologists in real-world mimic settings. Diagnostic performance was expressed as the area under the receiver operating characteristic curve (AUC). RESULTS The new model outperformed the previously published model in diagnosing BA on the external validation set (AUC 0.924 vs 0.908, P = 0.004) with higher consistency (kappa value 0.708 vs 0.609). When tested in real-world mimic settings using 333 sonographic gallbladder videos, the new model performed comparable to experienced pediatric radiologists (average AUC 0.860 vs 0.876) and outperformed junior radiologists (average AUC 0.838 vs 0.773) and senior radiologists (average AUC 0.829 vs 0.749). Furthermore, the new model could aid both junior and senior radiologists to improve their diagnostic performances, with the average AUC increasing from 0.773 to 0.835 for junior radiologists and from 0.749 to 0.805 for senior radiologists. CONCLUSIONS The interpretable app-based model showed robust and satisfactory performance in diagnosing biliary atresia, and it could aid radiologists with limited experiences to improve their diagnostic performances in real-world mimic settings.
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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
| | - Zejun Ye
- School of Computer Science and Engineering, Sun Yat-Sen University, No. 132, East Outer Ring Road, Guangzhou, 510006, People's Republic of China
| | - Guangliang Huang
- 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
| | - Ming Xu
- 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
| | - Baoxian Liu
- 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
| | - Bowen Zhuang
- 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
| | - Zijian Tang
- Department of Ultrasound, Shenzhen Children's Hospital, No. 7019, Yitian Road, Futian District, Shenzhen, 518026, People's Republic of China
| | - Shan Wang
- Department of Ultrasound, Shenzhen Children's Hospital, No. 7019, Yitian Road, Futian District, Shenzhen, 518026, People's Republic of China
| | - Dan Chen
- Department of Ultrasound, Guangdong Women and Children's Hospital, No. 521 Xingnan Avenue, Panyu District, Guangzhou, 511400, People's Republic of China
| | - Yunxiang Pan
- Department of Ultrasound, Guangdong Women and Children's Hospital, No. 521 Xingnan Avenue, Panyu District, Guangzhou, 511400, 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
| | - Ruixuan Wang
- School of Computer Science and Engineering, Sun Yat-Sen University, No. 132, East Outer Ring Road, Guangzhou, 510006, 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.
- Department of Ultrasound, Shenzhen Children's Hospital, No. 7019, Yitian Road, Futian District, Shenzhen, 518026, People's Republic of China.
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Chau P, Yoon JS, Moses D, Pather N. A systematic review and meta-analysis of portal vein morphometry in pediatric and adult populations: Drawing the line between normal and abnormal findings. Eur J Radiol 2023; 168:111016. [PMID: 37742371 DOI: 10.1016/j.ejrad.2023.111016] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2022] [Revised: 07/13/2023] [Accepted: 07/26/2023] [Indexed: 09/26/2023]
Abstract
PURPOSE The morphometry of the hepatic portal vein is of clinical importance, particularly in pre-operative assessments, surgical management, and diagnoses of liver conditions. This systematic review and meta-analysis aimed to characterize the morphometry of the normal portal vein in both pediatric and adult patients. METHODS The study, conducted using the PRISMA guidelines and registered with PROSPERO, utilized the MEDLINE, EMBASE, SCOPUS and Web of Science databases up to May 2020, and updated to May 2023. All studies reporting extractable data on diameter, length, and cross-sectional area (CSA) of the main, left, and right portal veins (PV, LPV, RPV, respectively) were included. The AQUA Tool was used to assess the quality of the included studies. Data analysis included subgroup analyses based on geographical location, sex, age, and imaging modality. RESULTS A total of 122 studies with 11,637 subjects were eligible for inclusion. Overall, the pooled mean diameter of the PV (PVD) was 10.09 mm (95% CI: 9.56-10.62). Significant differences in diameter were found between pediatric (6.60 mm; 95% CI: 5.38-7.82) and adult (10.72 mm; 95% CI: 10.25-11.19) subjects. Additionally, there was a significantly larger PVD measurement from computed tomography (CT) than other imaging modalities: CT, 13.28 mm (95% CI: 11.71-14.84); magnetic resonance imaging (MRI), 10.50 mm (95% CI: 9.35-11.66) and ultrasound (US), 9.81 mm (95% CI: 9.47-10.16). The mean diameters of the LPV and RPV were 8.27 mm (95% CI: 6.78-9.77) and 8.33 mm (95% CI: 6.70-9.95), respectively. Mean PV length in adults is 48.63 mm (95% CI: 35.63-61.64). Mean CSA of the PV was 1.09 cm2. CONCLUSIONS The study obtained aim to improve the understanding of portal vein anatomy, especially with relevance to surgical interventions of the liver in both pediatric and adult patients. Measurements from ultrasound imaging closely approximates the generated pooled PVD mean for pediatric and adult patients. CT imaging, however, significantly exceeded the established 13 mm threshold for adults. For pediatric patients, a threshold of 8 mm is proposed as a diagnostic upper limit for a normal PVD. Although not significant, the PVD decreased from the portal confluence towards its bifurcation.
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Affiliation(s)
- Patrick Chau
- Department of Anatomy, School of Biomedical Sciences, Faculty of Medicine and Health, UNSW Sydney, Sydney, Australia
| | - Ji Soo Yoon
- Department of Anatomy, School of Biomedical Sciences, Faculty of Medicine and Health, UNSW Sydney, Sydney, Australia
| | - Daniel Moses
- Department of Radiology, Prince of Wales Hospital, Sydney, Australia
| | - Nalini Pather
- Department of Anatomy, School of Biomedical Sciences, Faculty of Medicine and Health, UNSW Sydney, Sydney, Australia; Academy of Medical Education, Medical School, Faculty of Medicine, University of Queensland, Australia; Medical Education, Faculty of Medicine and Health, UNSW Sydney, Sydney, Australia.
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Quelhas P, Breton MC, Oliveira RC, Cipriano MA, Teixeira P, Cerski CT, Shivakumar P, Vieira SMG, Kieling CO, Verde I, Santos JLD. HIF-1alpha-pathway activation in cholangiocytes of patients with biliary atresia: An immunohistochemical/molecular exploratory study. J Pediatr Surg 2023; 58:587-594. [PMID: 36150932 DOI: 10.1016/j.jpedsurg.2022.08.020] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/04/2022] [Revised: 08/18/2022] [Accepted: 08/22/2022] [Indexed: 11/29/2022]
Abstract
BACKGROUND Biliary atresia is a neonatal disease characterized by choledochal obstruction and progressive cholangiopathy requiring liver transplantation in most patients. Hypoxia-ischemia affecting the biliary epithelium may lead to biliary obstruction. We hypothesized that ischemic cholangiopathy involving disruption of the peribiliary vascular plexus could act as a triggering event in biliary atresia pathogenesis. METHODS Liver and porta hepatis paraffin-embedded samples of patients with biliary atresia or intrahepatic neonatal cholestasis (controls) were immunohistochemically evaluated for HIF-1alpha-nuclear signals. Frozen histological samples were analyzed for gene expression in molecular profiles associated with hypoxia-ischemia. Prospective clinical-laboratory and histopathological data of biliary atresia patients and controls were reviewed. RESULTS Immunohistochemical HIF-1alpha signals localized to cholangiocytes were detected exclusively in liver specimens from biliary atresia patients. In 37.5% of liver specimens, HIF-1alpha signals were observed in biliary structures involving progenitor cell niches and peribiliary vascular plexus. HIF-1alpha signals were also detected in biliary remnants of 81.8% of porta hepatis specimens. Increased gene expression of molecules linked to REDOX status, biliary proliferation, and angiogenesis was identified in biliary atresia liver specimens. In addition, there was a trend towards decreased GSR expression levels in the HIF-1alpha-positive group compared to the HIF-1alpha-negative group. CONCLUSION Activation of the HIF-1alpha pathway may be associated with the pathogenesis of biliary atresia, and additional studies are necessary to confirm the significance of this finding. Ischemic cholangiopathy and REDOX status disturbance are putative explanations for HIF-1alpha activation. These findings may give rise to novel lines of clinical and therapeutic investigation in the BA field.
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Affiliation(s)
- Patrícia Quelhas
- Faculdade de Ciências da Saúde, Centro de Investigação em Ciências da Saúde, Universidade da Beira Interior (CICS-UBI), Covilhã, Portugal
| | - Michele Claire Breton
- Faculdade de Ciências da Saúde, Centro de Investigação em Ciências da Saúde, Universidade da Beira Interior (CICS-UBI), Covilhã, Portugal
| | - Rui Caetano Oliveira
- Serviço de Anatomia Patológica, Centro Hospitalar e Universitário, Universidade de Coimbra (SAP-CHUC), Portugal; Instituto de Biofísica, Faculdade de Medicina, Universidade de Coimbra, Portugal; Coimbra Institute for Clinical and Biomedical Research (iCBR), area of Environment Genetics and Oncobiology (CIMAGO), Faculty of Medicine, University of Coimbra, Portugal
| | - Maria Augusta Cipriano
- Serviço de Anatomia Patológica, Centro Hospitalar e Universitário, Universidade de Coimbra (SAP-CHUC), Portugal; Instituto de Biofísica, Faculdade de Medicina, Universidade de Coimbra, Portugal; Coimbra Institute for Clinical and Biomedical Research (iCBR), area of Environment Genetics and Oncobiology (CIMAGO), Faculty of Medicine, University of Coimbra, Portugal
| | - Paulo Teixeira
- Serviço de Anatomia Patológica, Centro Hospitalar e Universitário, Universidade de Coimbra (SAP-CHUC), Portugal; Instituto de Biofísica, Faculdade de Medicina, Universidade de Coimbra, Portugal; Coimbra Institute for Clinical and Biomedical Research (iCBR), area of Environment Genetics and Oncobiology (CIMAGO), Faculty of Medicine, University of Coimbra, Portugal
| | - Carlos Thadeu Cerski
- Universidade Federal do Rio Grande do Sul (UFRGS), Department of Pathology, Brazil
| | - Pranavkumar Shivakumar
- Divisions of Gastroenterology, Hepatology and Nutrition and The Liver Care Center at Cincinnati Children's Hospital Medical Center, Cincinnati, United States; Department of Pediatrics, University of Cincinnati, College of Medicine, Cincinnati, OH, United States
| | - Sandra Maria Gonçalves Vieira
- Universidade Federal do Rio Grande do Sul (UFRGS), Department of Pediatrics, Brazil; Unidade de Gastroenterologia e Hepatologia, Hospital de Clínicas de Porto Alegre (HCPA), Brazil; Programa de Transplante de Fígado Pediátrico, Hospital de Clínicas de Porto Alegre (HCPA), Brazil
| | - Carlos Oscar Kieling
- Unidade de Gastroenterologia e Hepatologia, Hospital de Clínicas de Porto Alegre (HCPA), Brazil
| | - Ignacio Verde
- Faculdade de Ciências da Saúde, Centro de Investigação em Ciências da Saúde, Universidade da Beira Interior (CICS-UBI), Covilhã, Portugal
| | - Jorge Luiz Dos Santos
- Faculdade de Ciências da Saúde, Centro de Investigação em Ciências da Saúde, Universidade da Beira Interior (CICS-UBI), Covilhã, Portugal.
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Zhang K, Tang Y, Liu R, Zheng Z, Tang C, Liu Y, Jin Z. Intraoperative hepatic subcapsular spider-like telangiectasia sign for the definitive diagnosis of biliary atresia. BMC Pediatr 2023; 23:63. [PMID: 36747150 PMCID: PMC9900992 DOI: 10.1186/s12887-022-03831-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/29/2022] [Accepted: 12/29/2022] [Indexed: 02/08/2023] Open
Abstract
OBJECTIVE To evaluate the accuracy of intraoperative hepatic subcapsular spider-like telangiectasia (HSST) sign for differentiating biliary atresia (BA) from other causes of hepatic cholestasis. METHODS The data of 69 patients with jaundice treated from January 2019 to December 2021 were retrospectively analyzed. Based on intraoperative cholangiography (IOC), the patients were divided into two groups: the BA group (n = 49) and the non-BA group (n = 20). The biochemistry tests, liver ultrasound, liver stiffness value and HSST sign of the two groups were compared. RESULTS The incidence of abnormal gallbladder, elevated γ-glutamyl transpeptidase (γ-GGT) > 182.0U/L and abnormal liver stiffness (> 6.4 kPa) in BA group were significantly higher than those in non-BA group (P < 0.001). The HSST sign was present in all BA patients and not found in non-BA group. The area under receiver operating curve of direct bilirubin(DBIL), γ-GGT, abnormal gallbladder, liver stiffness value and HSST sign were 0.53, 0.84, 0.78, 0.96, and 1.00, respectively. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value(NPV) of HSST sign in the diagnosis of BA were all 100%. CONCLUSION Presence of HSST sign on diagnostic laparoscopy is highly suggestive of BA.It can be used in the differential diagnosis of BA and non-BA. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Kaizhi Zhang
- grid.413390.c0000 0004 1757 6938Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000 China ,Department of Pediatric Surgery, Guizhou Children’s Hospital, Zunyi, 563000 China
| | - Yan Tang
- grid.411292.d0000 0004 1798 8975Clinical Medical College, Chengdu University, Chengdu, 610081 China
| | - Rui Liu
- grid.413390.c0000 0004 1757 6938Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000 China ,Department of Pediatric Surgery, Guizhou Children’s Hospital, Zunyi, 563000 China
| | - Zebing Zheng
- grid.413390.c0000 0004 1757 6938Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000 China ,Department of Pediatric Surgery, Guizhou Children’s Hospital, Zunyi, 563000 China
| | - Chengyan Tang
- grid.413390.c0000 0004 1757 6938Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000 China ,Department of Pediatric Surgery, Guizhou Children’s Hospital, Zunyi, 563000 China
| | - Yuanmei Liu
- grid.413390.c0000 0004 1757 6938Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000 China ,Department of Pediatric Surgery, Guizhou Children’s Hospital, Zunyi, 563000 China
| | - Zhu Jin
- Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000, China. .,Department of Pediatric Surgery, Guizhou Children's Hospital, Zunyi, 563000, China.
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Value of gallbladder length-to-width ratio for diagnosis of biliary atresia by correlation with age. Updates Surg 2022:10.1007/s13304-022-01427-x. [PMID: 36418718 DOI: 10.1007/s13304-022-01427-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2022] [Accepted: 11/14/2022] [Indexed: 11/25/2022]
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Quelhas P, Jacinto J, Cerski C, Oliveira R, Oliveira J, Carvalho E, dos Santos J. Protocols of Investigation of Neonatal Cholestasis-A Critical Appraisal. Healthcare (Basel) 2022; 10:2012. [PMID: 36292464 PMCID: PMC9602084 DOI: 10.3390/healthcare10102012] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2022] [Revised: 10/06/2022] [Accepted: 10/08/2022] [Indexed: 11/04/2022] Open
Abstract
Neonatal cholestasis (NC) starts during the first three months of life and comprises extrahepatic and intrahepatic groups of diseases, some of which have high morbimortality rates if not timely identified and treated. Prolonged jaundice, clay-colored or acholic stools, and choluria in an infant indicate the urgent need to investigate the presence of NC, and thenceforth the differential diagnosis of extra- and intrahepatic causes of NC. The differential diagnosis of NC is a laborious process demanding the accurate exclusion of a wide range of diseases, through the skillful use and interpretation of several diagnostic tests. A wise integration of clinical-laboratory, histopathological, molecular, and genetic evaluations is imperative, employing extensive knowledge about each evaluated disease as well as the pitfalls of each diagnostic test. Here, we review the difficulties involved in correctly diagnosing the cause of cholestasis in an affected infant.
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Affiliation(s)
- Patricia Quelhas
- Faculty of Health Sciences, Health Science Investigation Center of University of Beira Interior (CICS-UBI), 6200-506 Covilha, Portugal
| | - Joana Jacinto
- Medicine Department, University of Beira Interior (UBI), Faculty of Health Sciences, 6201-001 Covilha, Portugal
| | - Carlos Cerski
- Pathology Department of Universidade Federal do Rio Grande do Sul (UFRGS), Pathology Service of Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre 90035-903, Brazil
| | - Rui Oliveira
- Centro de Diagnóstico Histopatológico (CEDAP), 3000-377 Coimbra, Portugal
| | - Jorge Oliveira
- Center for Predictive and Preventive Genetics (CGPP), IBMC, UnIGENe, i3S, University of Porto, 4200-135 Porto, Portugal
| | - Elisa Carvalho
- Department of Gastroenterology and Hepatology, Hospital de Base do Distrito Federal, Hospital da Criança de Brasília, Brasília 70330-150, Brazil
| | - Jorge dos Santos
- Faculty of Health Sciences, Health Science Investigation Center of University of Beira Interior (CICS-UBI), 6200-506 Covilha, Portugal
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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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10
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Quelhas P, Cerski C, Dos Santos JL. Update on Etiology and Pathogenesis of Biliary Atresia. Curr Pediatr Rev 2022; 19:48-67. [PMID: 35538816 DOI: 10.2174/1573396318666220510130259] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2021] [Revised: 01/16/2022] [Accepted: 02/15/2022] [Indexed: 01/31/2023]
Abstract
Biliary atresia is a rare inflammatory sclerosing obstructive cholangiopathy that initiates in infancy as complete choledochal blockage and progresses to the involvement of intrahepatic biliary epithelium. Growing evidence shows that biliary atresia is not a single entity with a single etiology but a phenotype resulting from multifactorial events whose common path is obliterative cholangiopathy. The etiology of biliary atresia has been explained as resulting from genetic variants, toxins, viral infection, chronic inflammation or bile duct lesions mediated by autoimmunity, abnormalities in the development of the bile ducts, and defects in embryogenesis, abnormal fetal or prenatal circulation and susceptibility factors. It is increasingly evident that the genetic and epigenetic predisposition combined with the environmental factors to which the mother is exposed are potential triggers for biliary atresia. There is also an indication that a progressive thickening of the arterial middle layer occurs in this disease, suggestive of vascular remodeling and disappearance of the interlobular bile ducts. It is suggested that the hypoxia/ischemia process can affect portal structures in biliary atresia and is associated with both the extent of biliary proliferation and the thickening of the medial layer.
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Affiliation(s)
- Patrícia Quelhas
- CICS-UBI - Centro de Investigação em Ciências da Saúde, University of Beira Interior, 6200-506 Covilhã, Portugal
| | - Carlos Cerski
- Department of Pathology, University Federal Rio Grande do Sul, 90040-060, Porto Alegre, Brasil
| | - Jorge Luiz Dos Santos
- CICS-UBI - Centro de Investigação em Ciências da Saúde, University of Beira Interior, 6200-506 Covilhã, Portugal
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Li Y, Rong L, Tang J, Niu H, Jin Z, Zhou Y, Cao G, Zhang X, Chi S, Tang S. Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants. Front Pediatr 2022; 10:850449. [PMID: 35547547 PMCID: PMC9081763 DOI: 10.3389/fped.2022.850449] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2022] [Accepted: 03/23/2022] [Indexed: 01/20/2023] Open
Abstract
OBJECTIVE Operative cholangiography, the gold standard for the diagnosis of biliary atresia (BA), is being challenged due to an increase in the studies of misdiagnosis. A previous study has shown that the laparoscopic hepatic subcapsular spider-like telangiectasis (HSST) sign was accurate for diagnosing BA. This study aims to compare the performance of the HSST sign with cholangiography in the identification of BA. METHODS We prospectively screened consecutive infants with cholestasis who underwent laparoscopic exploration in this multicenter study. Demographics, intraoperative findings (videos and images), and outcomes were retrospectively analyzed. The data of the HSST sign and cholangiography were compared according to the final diagnosis. Then, the diagnostic accuracy of the BA using the HSST sign and cholangiography was validated in other independent cohorts. RESULTS A total of 2,216 patients were enrolled in this study. The sensitivity and negative predictive values were both 100% for diagnosing BA based on the HSST sign and cholangiography. The specificity, negative predictive value, and accuracy of the HSST sign (97.2, 99.2, 99.3%) in discriminating BA were significantly higher than operative cholangiography (81.6, 94.9, 95.8; p < 0.001). Moreover, to realize the early diagnosis of BA, the accuracy of the HSST sign in identifying BA was better than cholangiography in the subgroup of neonates (98.7% vs. 95.0%; p = 0.032). Interestingly, 92 non-BA patients without the HSST sign had positive cholangiography. Among them, 28 infants had negative cholangiography when the common bile duct was compressed and 39 patients displayed visible bile ducts due to repeated postoperative biliary irrigation. The other 25 patients (18 with the Alagille syndrome, 5 with progressive familial intrahepatic cholestasis, and 2 with the neonatal hepatitis syndrome) had consistently positive cholangiography. In the independent validation cohort, the diagnostic accuracy of the HSST sign (99.2%) was higher than cholangiography (95.0%, p = 0.012). CONCLUSION The laparoscopic HSST sign is superior to cholangiography in the diagnosis of BA in the infants with cholestasis and has advantages in early diagnosis. This method is expected to become a novel shift for diagnosing BA during ongoing laparoscopy.
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Affiliation(s)
- Yibo Li
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Liying Rong
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jingfeng Tang
- Department of Hepatobiliary Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Huizhong Niu
- Department of Pediatric General Surgery, Hebei Children's Hospital of Hebei Medical University, Shijiazhuang, China
| | - Zhu Jin
- Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, China
| | - Yun Zhou
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Guoqing Cao
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xi Zhang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Shuiqing Chi
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Shaotao Tang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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12
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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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13
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Yang P, Tang Y, Wang H, Zhang X, Yang B. Latest diagnostic performance of different ultrasonic features for biliary atresia. Acta Radiol 2021; 63:1593-1602. [PMID: 34854739 DOI: 10.1177/02841851211055820] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND Early diagnosis of biliary atresia (BA) is an important clinical challenge. PURPOSE To summarize the latest diagnostic performance of different ultrasonic (US) features for BA. MATERIAL AND METHODS MeSH terms "biliary atresia" and "ultrasonography" and related hyponyms were used to search PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials. Eligible articles were included and data were retrieved. The methodologic quality was assessed by version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Estimated sensitivity and specificity of each US feature were calculated by Stata 14.0. RESULTS Fifty eligible studies on 5622 patients were included. Respective summary sensitivity and specificity were 77% (95% CI=69-84) and 98% (95% CI=96-99) for triangular cord sign (TCS) in 32 studies, 86% (95% CI=78-92) and 86% (95% CI=72-94) for shear wave elastography (SWE) in seven studies, 75% (95% CI=65-83) and 92% (95% CI=86-95) for gallbladder and biliary system abnormality (GBA) in 25 studies, and 81% (95% CI=69-90) and 79% (95% CI=67-87) for hepatic artery (HA) enlargement in seven studies. The overall US features from 11 studies yielded a summary sensitivity of 84% (95% CI=72-92) and specificity of 86% (95% CI=77-92). CONCLUSION TCS and GBA were the two most widely accepted US features currently used for differential diagnosis of BA. The newly developed SWE was an objective and convenient method with good diagnostic performance. HA enlargement can be used as an auxiliary sign.
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Affiliation(s)
- Pan Yang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
| | - Yajie Tang
- Department of Cardiovascular Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, PR China
| | - Hongying Wang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
| | - Xiangxiang Zhang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
| | - Boyang Yang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
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14
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Dike PN, Mahmood N, Harpavat S. Recent advances in the use of ultrasound and related techniques in diagnosing and predicting outcomes in biliary atresia. Curr Opin Pediatr 2021; 33:515-520. [PMID: 34369411 PMCID: PMC8615294 DOI: 10.1097/mop.0000000000001048] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
PURPOSE OF REVIEW Biliary atresia (BA) is the leading cause of chronic liver disease and the most common indication for pediatric liver transplantation. The use of ultrasound (US) and related techniques continues to evolve to help diagnose BA as well as potentially to help predict outcomes after treatment with the Kasai portoenterostomy (KP). RECENT FINDINGS There are no US findings that are definitive for BA; however, signs which are consistent with BA include gallbladder abnormalities, the triangular cord sign, presence of hepatic subcapsular flow, and hilar lymphadenopathy. Elastography techniques to measure liver stiffness may also increase the diagnostic accuracy of detecting BA, particularly in older infants or without other US findings. In addition, both US and elastography are still being studied as potential methods to predict outcomes after KP such as the development of portal hypertension and the need for liver transplant. SUMMARY US findings in the diagnosis of BA are well characterized. Future studies will help determine the utility of elastography in diagnosing BA, as well as both US and elastography in monitoring and predicting disease outcomes after KP.
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Affiliation(s)
- Peace N. Dike
- Section of Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX, USA
| | - Nadia Mahmood
- E.B. Singleton Department of Pediatric Radiology, Texas Children’s Hospital, Houston, TX, USA
| | - Sanjiv Harpavat
- Section of Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, Baylor College of Medicine and Texas Children’s Hospital, Houston, TX, USA
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15
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Wang G, Zhang N, Zhang X, Zhou W, Xie X, Zhou L. Ultrasound characteristics combined with gamma-glutamyl transpeptidase for diagnosis of biliary atresia in infants less than 30 days. Pediatr Surg Int 2021; 37:1175-1182. [PMID: 34008061 DOI: 10.1007/s00383-021-04923-0] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/05/2021] [Indexed: 01/09/2023]
Abstract
PURPOSE To retrospectively assess the diagnostic performance of grey-scale ultrasound (US) characteristics and gamma-glutamyl transpeptidase (GGT) alone or combined in distinguishing biliary atresia (BA) from other cholestasis diseases in infants younger than 30 days. MATERIALS AND METHODS Between January 2012 and October 2020, the demographic characteristics, laboratory results and US characteristics of 35 BA and 52 non-BA infants younger than 30 days were retrospectively evaluated. Areas under the receiver operating characteristic curves (AUCs) were used to estimate the probability of predicting BA, which were compared by DeLong test. RESULTS The diagnostic performance of gallbladder classification in identifying BA was higher than that of fibrotic cord thickness (AUC 0.900 vs. 0.771, P = 0.03). With the cutoff level of 188 IU/L, serum GGT had a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 77.1%, 69.2%, 62.8%, and 81.8%, respectively. Combined with gallbladder classification and GGT, the sensitivity, specificity, PPV, NPV and accuracy were 100.0%, 63.5%, 64.8%, 100.0% and 78.2%. CONCLUSIONS Gallbladder classification was more valuable than fibrotic cord thickness in the diagnosis of BA among infants less than 30 days. Combined with gallbladder classification and GGT, the sensitivity for the diagnosis of BA can reach 100.0%.
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Affiliation(s)
- Guotao Wang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, 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, 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, 58 Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Wenying Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, 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, 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, 58 Zhongshan Er Road, Guangzhou, 510080, People's Republic of China.
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16
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El-Guindi MAS, Saber MA, Shoeir SA, Abdallah AR, Sira AM. Variant etiologies of neonatal cholestasis and their outcome: a Middle East single-center experience. Clin Exp Hepatol 2021; 7:205-214. [PMID: 34295989 PMCID: PMC8284164 DOI: 10.5114/ceh.2021.107066] [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: 01/14/2021] [Accepted: 03/21/2021] [Indexed: 11/17/2022] Open
Abstract
AIM OF THE STUDY Neonatal cholestasis (NC) constitutes a large proportion of pediatric liver disorders. Nevertheless, awareness of the variant etiologies and how to manage them appropriately are lacking. So, out of a few specialized centers, many cases pass without appropriate management. This study aimed to present our tertiary level center's experience in NC that could increase the pediatrician's awareness of handling this problematic and common medical morbidity efficiently. MATERIAL AND METHODS It is a retrospective study in which we analyzed the NC cases admitted to the inpatient department within three years. For all recruited patients, the available data were retrieved and recorded. RESULTS A total of 412 patients were reviewed with 20 different etiologies diagnosed. The most common cause was biliary atresia (n = 151, 37%), followed by progressive familial intrahepatic cholestasis (n = 51, 12%), neonatal sepsis (n = 39, 9%), and cytomegalovirus (n = 33, 8%). Of the 412 patients, 394 (81%) had follow-up ranging from 1 to 36 months. A total of 173 patients improved with supportive and/or specific therapy, while 108 patients died at a median age of 6 months. The commonest cause of death was liver failure (40.7%), followed by pneumonia (28.7%), sudden death (13%), septicemia (6.5%), and hepatorenal syndrome (5.5%). CONCLUSIONS NC constitutes more than one-third of the inpatient admissions of all pediatric liver disorders and has a high rate of mortality. Awareness of the variety of etiologies and a rapid stepwise approach to diagnosis could have an impact on the outcome of this devastating disease.
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Affiliation(s)
- Mohamed Abdel-Salam El-Guindi
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, Egypt
| | - Magdy Anwar Saber
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, Egypt
| | - Samar Ahmed Shoeir
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, Egypt
| | - Ayat Roushdy Abdallah
- Department of Epidemiology and Preventive Medicine, National Liver Institute, Menoufia University, Egypt
| | - Ahmad Mohamed Sira
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, Egypt
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El-Araby HA, Saber MA, Radwan NM, Taie DM, Adawy NM, Sira AM. Temporal histopathological changes in biliary atresia: A perspective for rapid fibrosis progression. Ann Hepatol 2021; 21:100263. [PMID: 33007429 DOI: 10.1016/j.aohep.2020.09.007] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2020] [Revised: 09/18/2020] [Accepted: 09/19/2020] [Indexed: 02/04/2023]
Abstract
INTRODUCTION AND OBJECTIVES Biliary atresia (BA) is characterized by rapid progression of fibrosis with no definite causes. Histopathological findings have been extensively described, but very few studies have assessed temporal changes in BA. Understanding these short-term changes and their relationship with fibrosis progression could have an impact on ameliorating rapid fibrogenesis. We aimed to study the relationship between temporal histopathological changes and fibrosis progression in BA within a short time interval. PATIENTS AND METHODS Forty-nine infants with BA who underwent Kasai portoenterostomy, a diagnostic liver biopsy, and an intraoperative liver biopsy were recruited. Histopathological characteristics of the two biopsies were examined. Temporal histopathological changes were assessed by comparing the two types of biopsies. Correlation of temporal changes in fibrosis with age, interval between biopsies, laboratory profiles, and temporal histopathological changes were studied. RESULTS In the univariate analysis, bile ductular proliferation (BDP), portal infiltrate, giant cells, hepatocellular swelling, and fibrosis showed significant temporal changes within a short interval (5-31 days). BDP and fibrosis showed the most frequent increase in their grades (32/49 and 31/49 cases, respectively). In the multivariate analysis, BDP was the only independent pathological feature showing a significant temporal increase (p = 0.021, 95% confidence interval: 1.249-16.017). Fibrosis progression was correlated with temporal changes in BDP (r = 0.456, p = 0.001), but not with age (p = 0.283) or the interval between the biopsies (p = 0.309). CONCLUSIONS Fibrosis in BA progresses rapidly and is significantly correlated with BDP. Assessment of targeting BDP as an adjuvant medical therapy is recommended.
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Affiliation(s)
- Hanaa A El-Araby
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt
| | - Magdy A Saber
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt
| | - Noha M Radwan
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt
| | - Doha M Taie
- Department of Pathology, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt
| | - Nermin M Adawy
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt
| | - Ahmad M Sira
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menoufia University, 32511 Shebin El-Koom, Menoufia, Egypt.
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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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19
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Kohno M. Diagnostic Modalities for Biliary Atresia. INTRODUCTION TO BILIARY ATRESIA 2021:123-131. [DOI: 10.1007/978-981-16-2160-4_19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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20
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Carlson CL, Boehnke MW, Paltiel HJ. Gallbladder and Biliary Tract. PEDIATRIC ULTRASOUND 2021:433-479. [DOI: 10.1007/978-3-030-56802-3_12] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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21
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Hepatic Subcapsular Flow as a Significant Diagnostic Marker for Biliary Atresia: A Meta-Analysis. DISEASE MARKERS 2020; 2020:5262565. [PMID: 32190130 PMCID: PMC7072104 DOI: 10.1155/2020/5262565] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/08/2019] [Accepted: 08/17/2019] [Indexed: 11/18/2022]
Abstract
Aim Increasing evidence indicates that hepatic subcapsular flow (HSF) can serve as a noninvasive ultrasonographic marker for the early diagnosis of biliary atresia (BA). However, results regarding its diagnostic accuracy are inconsistent and inconclusive. We conducted this meta-analysis with an aim to systematically evaluate the diagnostic value of HSF in predicting BA. Methods A comprehensive literature search of four databases was conducted to identify the eligible studies. All analyses were performed using STATA 12.0. Results Nine studies from eight articles containing 368 patients and 469 controls were included in our meta-analysis. Briefly, the values for pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic odds ratio (DOR), and area under the curve (AUC) were 0.95 (95% CI 0.88-0.98), 0.92 (95% CI 0.85-0.96), 11.6 (95% CI 6.3-21.5), 0.06 (95% CI 0.02-0.14), 201 (95% CI 59-689), and 0.98 (95% CI 0.96-0.99), respectively. Additionally, metaregression along with subgroup analysis based on various covariates revealed the potential sources of heterogeneity and the detailed diagnostic value in each subgroup. Conclusion Our meta-analysis showed that HSF assay could provide high accuracy in predicting BA patients and non-BA individuals. However, further studies with better design and larger sample size are required to support the results of the present study.
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22
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Di Serafino M, Severino R, Gioioso M, Rossi E, Vezzali N, Pelliccia P, Caprio MG, Acampora C, Iorio R, Vallone G. Paediatric liver ultrasound: a pictorial essay. J Ultrasound 2020; 23:87-103. [PMID: 30778891 PMCID: PMC7010916 DOI: 10.1007/s40477-018-0352-z] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2018] [Accepted: 12/07/2018] [Indexed: 02/06/2023] Open
Abstract
Ultrasound scan is a painless and radiation-free imaging modality and, therefore, it is widely considered the first-choice diagnostic tool in the setting of hepatopathies in paediatric patients. This article focuses on the normal ultrasound anatomy of the liver in neonatal and paediatric age and reviews the ultrasound appearance of the most common diffuse and focal liver affections.
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Affiliation(s)
- Marco Di Serafino
- Department of Radiology, "Antonio Cardarelli" Hospital, Antonio Cardarelli ST 9, 80131, Naples, Italy.
| | - Rosa Severino
- Department of Radiology, "San Carlo Regional Hospital", Potenza, Italy
| | - Matilde Gioioso
- Department of Radiology, "San Carlo Regional Hospital", Potenza, Italy
| | - Eugenio Rossi
- Department of Radiology, "Santobono-Pausilipon" Children Hospital, Naples, Italy
| | - Norberto Vezzali
- Department of Radiology, "Regional Hospital of Bolzano", Bolzano, Italy
| | | | - Maria Grazia Caprio
- Institute of Biostructure and Bioimaging IBB, Italian National Research Council CNR, Naples, Italy
| | - Ciro Acampora
- Department of Radiology, "Antonio Cardarelli" Hospital, Antonio Cardarelli ST 9, 80131, Naples, Italy
| | - Raffaele Iorio
- Department of Paediatrics, University Hospital "Federico II", Naples, Italy
| | - Gianfrancio Vallone
- Department of Paediatrics, Radiology University Hospital "Federico II", Naples, Italy
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Di Serafino M, Gioioso M, Severino R, Esposito F, Vezzali N, Ferro F, Pelliccia P, Caprio MG, Iorio R, Vallone G. Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. J Ultrasound 2020; 23:1-12. [PMID: 30756259 PMCID: PMC7010886 DOI: 10.1007/s40477-019-00362-9] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2018] [Accepted: 01/24/2019] [Indexed: 02/07/2023] Open
Abstract
Paediatric biliary tract and gallbladder diseases include a variety of entities with a wide range of clinical presentations. Cholestasis represents an impaired secretion of bilirubin by hepatocytes, manifesting with high blood levels of conjugated bilirubin and jaundice. Various causes may be involved, which can be recognised analysing blood tests and hepatobiliary imaging, while sometimes liver biopsy or surgery may be necessary. High-resolution real-time ultrasonography is an important tool for differentiation of obstructive and non-obstructive causes of jaundice in infants and children. In this paper, we briefly review the normal anatomy and the ultrasound aspects of main pathologies affecting gallbladder and biliary tree in neonatal and paediatric age.
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Affiliation(s)
- Marco Di Serafino
- Department of Radiology, "Antonio Cardarelli" Hospital, Antonio Cardarelli st 9, 80131, Naples, Italy.
| | - Matilde Gioioso
- Department of Radiology, "San Carlo Regional Hospital", Potenza, Italy
| | - Rosa Severino
- Department of Radiology, "San Carlo Regional Hospital", Potenza, Italy
| | - Francesco Esposito
- Department of Radiology, "Santobono-Pausilipon" Children Hospital, Naples, Italy
| | - Norberto Vezzali
- Department of Radiology, "Regional Hospital of Bolzano", Bolzano, Italy
| | - Federica Ferro
- Department of Radiology, "Regional Hospital of Bolzano", Bolzano, Italy
| | | | - Maria Grazia Caprio
- Institute of Biostructure and Bioimaging IBB, Italian National Research Council CNR, Rome, Italy
| | - Raffaele Iorio
- Department of Paediatrics, "Federico II" University Hospital, Naples, Italy
| | - Gianfranco Vallone
- Department of Radiology, "Federico II" University Hospital, Naples, Italy
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Lee S, Kim MJ, Lee MJ, Yoon H, Han K, Han SJ, Koh H, Kim S, Shin HJ. Hepatic subcapsular or capsular flow in biliary atresia: is it useful imaging feature after the Kasai operation? Eur Radiol 2020; 30:3161-3167. [PMID: 32048036 DOI: 10.1007/s00330-020-06656-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2019] [Revised: 10/24/2019] [Accepted: 10/29/2019] [Indexed: 12/12/2022]
Abstract
OBJECTIVES To evaluate the implications of hepatic subcapsular and capsular flows using ultrasonography (US) in children after Kasai operation. METHODS Children who underwent liver US including color Doppler US and microvascular imaging (MVI) from May 2017 to October 2017 were retrospectively included. Children who underwent the Kasai operation for biliary atresia were included in the Kasai group and children with normal liver were included in the control group. Using US results, the number of intrahepatic biliary cysts and the maximum diameter of the spleen were evaluated in the Kasai group. Liver stiffness values were included when patients in the Kasai group had transient elastography (TE) or shear wave elastography (SWE) results. Hepatic subcapsular and capsular flows on color Doppler US and MVI were compared between the two groups using the following scores: 0, no flow reaching the hepatic capsule; 1, any flow reaching the hepatic capsule; and 2, contiguous hepatic capsular flow. The logistic regression test was used to identify associations between age, intrahepatic biliary cysts, spleen size, SWV, TE results, and flow scores measured on Doppler US and MVI in the Kasai group using the odds ratio (OR) and 95% confidence interval (CI). RESULT A total of 65 children (mean 7.6 ± 5.3 years), 44 in the Kasai group and 21 in the control group, were included. In the control group, one child had score 1 on MVI and others had score 0 on both Doppler US and MVI. Among the Kasai group, 28 children (63.6%) had score 1, while others had score 0 using Doppler US. Using MVI, 24 children (54.5%) had score 2, 18 children had score 1, and one child had score 0. In the Kasai group, increased liver stiffness on TE was the only factor significantly associated with the presence of subcapsular flow on color Doppler US (OR 1.225, 95% CI 1.020-1.470) and increased spleen size was the only factor significantly associated with increased flow scores on MVI (OR 1.397, 95% CI 1.002-2.724). CONCLUSION Detection of hepatic subcapsular, capsular flows on US would be meaningful for children after receiving the Kasai operation. KEY POINTS • Hepatic subcapsular or capsular flows can be useful not only for the diagnosis but also for the postoperative follow-up in patients with biliary atresia. • Increased liver stiffness and splenomegaly after the Kasai operation were associated with presence of subcapsular or capsular flow on ultrasonography. • Evaluation of hepatic subcapsular and capsular flows could be needed to assess disease progression after receiving the Kasai operation.
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Affiliation(s)
- Suji Lee
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Myung-Joon Kim
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Mi-Jung Lee
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Haesung Yoon
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Kyunghwa Han
- Department of Radiology, Research Institute of Radiological Science, Center for Clinical Imaging Data Science, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Seok Joo Han
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
- Department of Pediatric Surgery, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Hong Koh
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
- Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Seung Kim
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
- Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea
| | - Hyun Joo Shin
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea.
- Severance Pediatric Liver Disease Research Group, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, South Korea.
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Expression of vascular endothelial growth factor A in liver tissues of infants with biliary atresia. Clin Exp Hepatol 2019; 5:308-316. [PMID: 31893243 PMCID: PMC6935852 DOI: 10.5114/ceh.2019.89476] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/22/2019] [Accepted: 08/01/2019] [Indexed: 12/22/2022] Open
Abstract
Aim of the study Assessment of hepatic expression of vascular endothelial growth factor A (VEGF-A) in liver tissues of infants with biliary atresia (BA). Material and methods This retrospective study included 35 infants with BA (BA group), and 38 infants with cholestasis due to causes other than BA (non-BA group). All patients had undergone full history taking, through clinical examination, routine investigations and immunostaining of liver tissue for VEGF-A and cytokeratin 7 (CK7). The diagnosis of BA was confirmed by intraoperative cholangiography. In the non-BA group, other specific laboratory tests according to the expected etiology were done. Results Most of the BA group showed positive VEGF-A expression with variable degrees in both bile ducts (BDs; 80%), and arterial walls (AWs; 77.2%), while most of the non-BA group showed negative staining of VEGF in both BDs and AWs (89.5% and 86.8% respectively) (p < 0.0001). Positive VEGF expression in the portal structures in both BDs and AWs had 84.9% and 82.19% accuracy; respectively. The majority of BA group showed either grade II of positive cytokeratin-7 expression in liver tissues (45.7%) or grade III (34.3%), while most of the non-BA group showed grade I (71.1%) (p < 0.0001). Positive CK7 expression in > 25% of the liver tissues had 80.8% accuracy in discriminating between BA and non-BA. Conclusions VEGF-A expression in the portal structures in liver tissues in both BDs and AWs had very good accuracy in discriminating between BA and non-BA patients.
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26
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Carollo V, Milazzo M, Miraglia R. The "hepatic subcapsular flow sign" in early diagnosis of biliary atresia. Abdom Radiol (NY) 2019; 44:3200-3202. [PMID: 31236607 DOI: 10.1007/s00261-019-02115-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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Choochuen P, Kritsaneepaiboon S, Charoonratana V, Sangkhathat S. Is "gallbladder length-to-width ratio" useful in diagnosing biliary atresia? J Pediatr Surg 2019; 54:1946-1952. [PMID: 30765154 DOI: 10.1016/j.jpedsurg.2019.01.008] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2018] [Revised: 11/13/2018] [Accepted: 01/09/2019] [Indexed: 10/27/2022]
Abstract
BACKGROUND The accurate assessment of gallbladder shape and wall abnormalities by ultrasound (US) in diagnosing biliary atresia (BA) remains a subjective determination. The objective of this study was to examine the reliability of gallbladder length-to-width ratio (LTWR) by US measurement for diagnosis of BA. METHODS One hundred infants with conjugated hyperbilirubinemia and unknown cause of jaundice who underwent transabdominal US from February 2009 to February 2017 were enrolled. The gallbladder classification and other detailed US findings were reviewed. RESULTS There were statistical differences in gallbladder lumen, classification, length, width and LTWR of gallbladder (all P < 0.05) between BA and non-BA groups. The gallbladder LTWR with a cutoff at 4.1 had the highest sensitivity of 71.7%, while the fibrotic cord thickness had the highest specificity of 95.9%. The combination of portal vein (PV) diameter > 4.4 mm, hepatic artery (HA) diameter > 1.2 mm, and gallbladder LTWR >4.1, provided much higher specificity (98%), odds ratio (11), and positive likelihood ratio (LR+) (10.6). CONCLUSION The gallbladder LTWR by US could be a suggestive US parameter for BA screening. The triad of PV diameter, HA diameter, and gallbladder LTWR yielded the highest specificity, odds ratio, and LR+ for diagnosing BA. LEVEL OF EVIDENCE Level III study of diagnostic test.
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Affiliation(s)
- Panjai Choochuen
- Department of Radiology, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand
| | - Supika Kritsaneepaiboon
- Department of Radiology, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand.
| | - Vorawan Charoonratana
- Department of Radiology, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand
| | - Surasak Sangkhathat
- Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand
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Enlarged hepatic hilar lymph node: an additional ultrasonographic feature that may be helpful in the diagnosis of biliary atresia. Eur Radiol 2019; 29:6699-6707. [PMID: 31297631 DOI: 10.1007/s00330-019-06339-w] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2019] [Revised: 04/17/2019] [Accepted: 06/25/2019] [Indexed: 12/12/2022]
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Does Supersonic Shear Wave Elastography Help Differentiate Biliary Atresia from Other Causes of Cholestatic Hepatitis in Infants Less than 90 Days Old? Compared with Grey-Scale US. BIOMED RESEARCH INTERNATIONAL 2019; 2019:9036362. [PMID: 31275991 PMCID: PMC6582890 DOI: 10.1155/2019/9036362] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/05/2019] [Revised: 04/12/2019] [Accepted: 04/30/2019] [Indexed: 12/24/2022]
Abstract
Purpose To investigate the diagnostic performance of shear wave elastography (SWE) for measuring liver stiffness to identify and differentiate biliary atresia (BA) from cholestatic hepatitis in infants younger than 90 days. Methods A total of 138 infants younger than 90 days with cholestatic hepatitis were examined by SWE. The infants were subclassified into BA and nonbiliary atresia (non-BA) groups. Receiver operating characteristic (ROC) analysis was used to determine the sensitivity and specificity of hepatic Young's modulus measurements, the ultrasonic findings in the differential diagnosis of suspected BA, and the cut-off value to diagnose BA. Results In all infants with cholestatic hepatitis, the cut-off value of hepatic Young's modulus to differentiate the BA group from the non-BA group was 12.35 kPa and the area under the ROC curve (AUC) was 0.937, with a sensitivity of 84.3% and a specificity of 89.7%; nevertheless the AUC of the abnormal gallbladder (AbGB) was 0.940, with a sensitivity of 96.1% and a specificity of 92.0%. In the parallel test, triangular cord (TC) sign combined with AbGB had the best diagnostic performance and the AUC was 0.960, with a sensitivity of 100% and a specificity of 92.0%. In the serial test, SWE combined with AbGB achieved the best diagnostic performance; the AUC was 0.902, the sensitivity and specificity were 80.4% and 100%, respectively. Conclusions SWE could not only help differentiate BA from cholestatic hepatic diseases but also increase the diagnostic specificity when combined with grey-scale ultrasound in the serial test.
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30
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Behairy BES, Konswa HAA, Ahmed HT, El-Azab DS, Adawy NM, Sira AM. Serum ferritin in neonatal cholestasis: A specific and active molecule or a non-specific bystander marker? Hepatobiliary Pancreat Dis Int 2019; 18:173-180. [PMID: 30833173 DOI: 10.1016/j.hbpd.2019.02.006] [Citation(s) in RCA: 6] [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: 07/06/2018] [Accepted: 02/16/2019] [Indexed: 02/05/2023]
Abstract
BACKGROUND Serum ferritin (SF) and consequently hepatic iron have long been considered important in liver fibrosis progression. They have been studied in different liver diseases with no previous reports in neonatal cholestasis (NC). This study aimed to measure SF in different etiologies of NC and investigate its relation to hepatic iron and fibrosis. METHODS SF was measured in 75 infants, including 50 with NC and 25 with sepsis. SF was compared between these two groups. Biochemical parameters, hepatic iron grades, and liver fibrosis and other histopathological characteristics and correlated with SF were assessed in NC group. Finally, a comparison between intrahepatic cholestasis and obstructive etiology was performed. RESULTS SF was elevated in NC (1598 ± 2405 ng/mL) with no significant difference from those with sepsis (P = 0.445). NC and sepsis constituted augmenting factors leading to more elevation of SF (2589 ± 3511 ng/mL). SF was significantly correlated with hepatic iron grades (r = 0.536, P < 0.0001) and a cut-off value of 803.5 ng/mL can predict higher grades (≥ grade 3) of iron deposition with sensitivity of 100%, specificity of 70% and accuracy of 85%. Moreover, SF was significantly higher (P < 0.0001) in those with intrahepatic cholestasis (2602 ± 3154 ng/mL) and their prevalent pathological findings of giant cell transformation (P = 0.009) and hepatocyte swelling (P = 0.023) than those with obstructive etiology (672 ± 566 ng/mL) and their prevalent pathological findings of ductular proliferation (P = 0.003) and bile plugs (P = 0.002). SF was unrelated to the grade of liver fibrosis (P = 0.058). CONCLUSIONS SF is non-specifically elevated in NC, with positive correlation to hepatic iron grades. SF ≥ 803.5 ng/mL can predict higher grades (≥ grade 3) of hepatic iron. However, an active role of increased SF and hepatic iron in disease progression remains questionable.
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Affiliation(s)
- Behairy El-Sayed Behairy
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Hatem Abd-Alsattar Konswa
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Hanaa Talaat Ahmed
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Dina Shehata El-Azab
- Department of Pathology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Nermin Mohamed Adawy
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Ahmad Mohamed Sira
- Department of Pediatric Hepatology, Gastroenterology, and Nutrition, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
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Wang L, Yang Y, Chen Y, Zhan J. Early differential diagnosis methods of biliary atresia: a meta-analysis. Pediatr Surg Int 2018; 34:363-380. [PMID: 29397405 DOI: 10.1007/s00383-018-4229-1] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/11/2018] [Indexed: 11/25/2022]
Abstract
PURPOSE To evaluate the accuracy of early differential diagnosis methods of biliary atresia in patients with infantile cholestasis. METHODS We searched PubMed, EMBASE and the Web of Science databases for articles evaluated the early differential diagnosis methods of biliary atresia. The methodological quality of each study was assessed with version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Two reviewers extracted data independently. Pooled sensitivity, specificity, positive likelihood ratio (LR +), negative likelihood ratio (LR -), diagnostic odds ratio (DOR) with 95% CIs were calculated to assess each diagnosis method. RESULTS A total of 38 articles were included. Summary sensitivity and specificity were 77% (95% CI 74-80%) and 93% (95% CI 91-94%), respectively, for B-US in 23 studies; 96% (95% CI 92-98%) and 58% (95% CI 51-65%), respectively, for MRCP in five studies; 87% (95% CI 82-91%) and 78% (95% CI 74-82%), respectively, for acholic stool in seven studies; 84% (95% CI 78-89%) and 97% (95% CI 97-98%), respectively, for serum liver function test in seven studies; 96% (95% CI 94-97%) and 73% (95% CI 70-76%), respectively, for hepatobiliary scintigraphy in 18 studies; 98% (95% CI 96-99%) and 93% (95% CI 89-95%), respectively, for percutaneous liver biopsy in 11 studies. CONCLUSION The accuracy rate of percutaneous liver biopsy is better than all of the noninvasive methods. Take into consideration the advantages and disadvantages of the six methods, combination of multidisciplinary noninvasive diagnosis methods is the first choice for differential diagnosis of BA from other causes of neonatal cholestasis.
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Affiliation(s)
- Li Wang
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China
| | - Yuan Yang
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China
| | - Yang Chen
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China
| | - Jianghua Zhan
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China.
- Tianjin Pediatrics Research Institute, Tianjin, 300134, China.
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Ghazy RM, Adawy NM, Khedr MA, Tahoun MM. Biliary atresia recent insight. EGYPTIAN PEDIATRIC ASSOCIATION GAZETTE 2018. [DOI: 10.1016/j.epag.2017.12.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
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Kim YH, Kim MJ, Shin HJ, Yoon H, Han SJ, Koh H, Roh YH, Lee MJ. MRI-based decision tree model for diagnosis of biliary atresia. Eur Radiol 2018; 28:3422-3431. [PMID: 29476221 DOI: 10.1007/s00330-018-5327-0] [Citation(s) in RCA: 36] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2017] [Revised: 01/08/2018] [Accepted: 01/12/2018] [Indexed: 12/16/2022]
Abstract
OBJECTIVES To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. METHODS We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB). Hepatic subcapsular flow was reviewed on Doppler US. We performed conditional inference tree analysis using MRI findings to generate a decision tree model. RESULTS A total of 208 infants were included, 112 in the BA group and 96 in the non-BA group. Mean age at the time of MRI was 58.7 ± 36.6 days. Visibility of CBD, abnormality of GB and MR-TCT were good discriminators for the diagnosis of BA and the MRI-based decision tree using these findings with MR-TCT cut-off 5.1 mm showed 97.3 % sensitivity, 94.8 % specificity and 96.2 % accuracy. CONCLUSIONS MRI-based decision tree model reliably differentiates BA in infants with jaundice. MRI can be an objective imaging modality for the diagnosis of BA. KEY POINTS • MRI-based decision tree model reliably differentiates biliary atresia in neonatal cholestasis. • Common bile duct, gallbladder and periportal signal changes are the discriminators. • MRI has comparable performance to ultrasonography for diagnosis of biliary atresia.
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Affiliation(s)
- Yong Hee Kim
- Department of Radiology and Research Institute of Radiological Science, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Myung-Joon Kim
- Department of Radiology and Research Institute of Radiological Science, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.,Severance Pediatric Liver Disease Research Group, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Hyun Joo Shin
- Department of Radiology and Research Institute of Radiological Science, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.,Severance Pediatric Liver Disease Research Group, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Haesung Yoon
- Department of Radiology and Research Institute of Radiological Science, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.,Severance Pediatric Liver Disease Research Group, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Seok Joo Han
- Severance Pediatric Liver Disease Research Group, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.,Department of Pediatric Surgery, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Hong Koh
- Severance Pediatric Liver Disease Research Group, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.,Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Yun Ho Roh
- Biostatistics Collaboration Unit, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea
| | - Mi-Jung Lee
- Department of Radiology and Research Institute of Radiological Science, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea. .,Severance Pediatric Liver Disease Research Group, Severance Children's Hospital, Yonsei University College of Medicine, 50-1Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea.
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Early US findings of biliary atresia in infants younger than 30 days. Eur Radiol 2017; 28:1771-1777. [PMID: 29063249 DOI: 10.1007/s00330-017-5092-5] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2017] [Revised: 09/07/2017] [Accepted: 09/22/2017] [Indexed: 10/18/2022]
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Zhou Y, Jiang M, Tang ST, Yang L, Zhang X, Yang DH, Xiong M, Li S, Cao GQ, Wang Y. Laparoscopic finding of a hepatic subcapsular spider-like telangiectasis sign in biliary atresia. World J Gastroenterol 2017; 23:7119-7128. [PMID: 29093620 PMCID: PMC5656459 DOI: 10.3748/wjg.v23.i39.7119] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2017] [Revised: 08/23/2017] [Accepted: 09/13/2017] [Indexed: 02/06/2023] Open
Abstract
AIM To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia.
METHODS A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with non-hepatic conditions. Analysis was first made by two observers separately and finally, a consensus conclusion was achieved. Then, the diagnostic value of the HSST sign was validated in an independent cohort including 45 BA and 45 non-BA patients.
RESULTS In the retrospective investigation, an amplified HSST sign was found in all BA patients, while we were unable to detect the HSST sign in 98.1% of the 54 non-BA patients. There was no HSST sign in any of the control subjects. In the first review, the sensitivity and specificity from one reviewer were 100% and 98.1%, respectively, and the results from the other reviewer were both 100%. The consensus sensitivity and specificity were 100% and 98.1%, respectively. The HSST sign was defined as being composed of several enlarged tortuous spider-like vascular plexuses with two to eight branches distributed on all over the liver surface, which presented as either a concentrated type or a dispersed type. In the independent validation group, the sensitivity, specificity, positive predictive value and negative predictive value of the HSST sign were 100%, 97.8%, 97.8% and 100%, respectively.
CONCLUSION The HSST sign is characteristic in BA, and laparoscopic exploration for the HSST sign is valuable in the diagnosis of BA.
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Affiliation(s)
- Ying Zhou
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Meng Jiang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Shao-Tao Tang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Li Yang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Xi Zhang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - De-Hua Yang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Meng Xiong
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Shuai Li
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Guo-Qing Cao
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Yong Wang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
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Yoon HM, Suh CH, Kim JR, Lee JS, Jung AY, Cho YA. Diagnostic Performance of Sonographic Features in Patients With Biliary Atresia: A Systematic Review and Meta-analysis. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2017; 36:2027-2038. [PMID: 28556285 DOI: 10.1002/jum.14234] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/14/2016] [Accepted: 12/30/2016] [Indexed: 06/07/2023]
Abstract
OBJECTIVES To evaluate the diagnostic performance of sonographic features of biliary atresia in patients with infantile cholestasis. METHODS The Ovid-MEDLINE and EMBASE databases were searched for studies of the diagnostic performance of sonographic features of biliary atresia in patients with infantile cholestasis. A meta-analysis was performed to evaluate the diagnostic performance of the triangular cord sign and other sonographic features in patients with biliary atresia. RESULTS Seventeen eligible studies with 1444 patients were included. The triangular cord sign had high accuracy for diagnosing biliary atresia: the meta-analytic summary sensitivity and specificity were 85% (95% confidence interval, 77%-90%) and 97% (95% confidence interval, 94%-99%), respectively. The area under the hierarchical summary receiver operating characteristic curve was 0.97. Meta-regression analysis revealed that the cutoff thickness of the triangular cord sign (3 or 4 mm) was a significant factor affecting study heterogeneity (P < .01). The proportions of nonidentification of the gallbladder (GB) ranged from 0% to 53%. Abnormal GB morphologic characteristics, nonvisualization of the common bile duct, and the presence of hepatic subcapsular flow showed relatively high sensitivities and specificities. CONCLUSIONS The triangular cord sign and other sonographic features, including abnormal GB morphologic characteristics, nonvisualization of the common bile duct, and the presence of hepatic subcapsular flow, had high diagnostic performance for the diagnosis of biliary atresia.
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Affiliation(s)
- Hee Mang Yoon
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Chong Hyun Suh
- Department of Radiology, Namwon Medical Center, Namwon, Korea
| | - Jeong Rye Kim
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Jin Seong Lee
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Ah Young Jung
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Young Ah Cho
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
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Govindarajan KK. Biliary atresia: Where do we stand now? World J Hepatol 2016; 8:1593-1601. [PMID: 28083081 PMCID: PMC5192550 DOI: 10.4254/wjh.v8.i36.1593] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2016] [Revised: 09/09/2016] [Accepted: 11/01/2016] [Indexed: 02/06/2023] Open
Abstract
The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia.
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Affiliation(s)
- Krishna Kumar Govindarajan
- Krishna Kumar Govindarajan, Department of Pediatric Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry 605006, India
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Monti L, Soglia G, Tomà P. Imaging in pediatric liver transplantation. Radiol Med 2016; 121:378-90. [PMID: 26909515 DOI: 10.1007/s11547-016-0628-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2016] [Accepted: 01/31/2016] [Indexed: 12/11/2022]
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El-Guindi MAS, El-Said HH, Hussein MH, Nassar RES, Sira AM. Urinary urobilinogen in biliary atresia: A missed, simple and cheap diagnostic test. Hepatol Res 2016; 46:174-182. [PMID: 26190184 DOI: 10.1111/hepr.12558] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/08/2015] [Revised: 07/01/2015] [Accepted: 07/14/2015] [Indexed: 02/08/2023]
Abstract
AIM Early diagnosis of biliary atresia (BA) is of utmost importance for good outcome; however, it is sometimes difficult due to the overlapping diagnostic test results with other causes of neonatal cholestasis. Moreover, many diagnostic tests are costly, invasive and not available in all centers, especially in developing countries. So, we aimed to investigate the diagnostic performance of urinary urobilinogen; an easy, cheap test that was not tested before in BA. METHODS Seventy-five infants divided into three age- and sex-matched groups (BA, non-BA cholestasis and healthy control group) were recruited for the study. Each group comprised 25 infants. Urinary urobilinogen was measured for all infants using the modified Ehrlich's method. RESULTS Urinary urobilinogen was significantly lower in the BA group (0.31 ± 0.25 mg/dL) than both of the non-BA cholestasis (2.08 ± 3.48 mg/dL) and healthy control (0.53 ± 0.64 mg/dL) groups at P < 0.0001 and P < 0.001, respectively. Urinary urobilinogen at a cut-off value of 0.32 mg/dL or less can differentiate BA from other non-BA cholestasis with a sensitivity of 88% and a specificity of 72%. When this cut-off value was combined with γ-glutamyltransferase (γ-GT) at a cut-off value of 363 U/L or more, BA could be differentiated from other cholestatic disorders with a sensitivity of 80% and specificity of 100%. On the other hand, dipstick test could not differentiate between BA and non-BA cholestasis (P = 0.396). CONCLUSION Urinary urobilinogen is a simple, non-invasive, cheap, sensitive and specific marker, especially if combined with γ-GT, which can be used in diagnosis of BA, especially in developing countries.
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Affiliation(s)
| | - Hala Hany El-Said
- Clinical Biochemistry, National Liver Institute, Menofiya University, Shebin El-koom, Egypt
| | - Mohsen Hassan Hussein
- Departments of Pediatric Hepatology, National Liver Institute, Menofiya University, Shebin El-koom, Egypt
| | - Rana El-Sayed Nassar
- Departments of Pediatric Hepatology, National Liver Institute, Menofiya University, Shebin El-koom, Egypt
| | - Ahmad Mohamed Sira
- Departments of Pediatric Hepatology, National Liver Institute, Menofiya University, Shebin El-koom, Egypt
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Monti L, Infante A, Salsano M. Biliary Atresia and Choledochal Cyst. IMAGING NON-TRAUMATIC ABDOMINAL EMERGENCIES IN PEDIATRIC PATIENTS 2016:95-113. [DOI: 10.1007/978-3-319-41866-7_7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Sira MM, Sira AM, Ehsan NA, Mosbeh A. P-Selectin (CD62P) Expression in Liver Tissue of Biliary Atresia: A New Perspective in Etiopathogenesis. J Pediatr Gastroenterol Nutr 2015; 61:561-567. [PMID: 26102172 DOI: 10.1097/mpg.0000000000000875] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVES The etiology of biliary atresia (BA) is still elusive. Inflammation plays a key role in bile duct and liver injury. The recruitment and accumulation of inflammatory cells is largely dependent on adhesion molecules. We aimed to investigate P-selectin (CD62P) expression in liver tissue in patients with BA compared with other neonatal cholestatic disorders. METHODS The study included 63 infants with neonatal cholestasis in 2 groups: BA group (n = 32) and non-BA group (n = 31) with non-BA cholestatic disorders as controls. Demographic, clinical, laboratory, ultrasonographic, and histopathological parameters were collected. P-selectin immunostaining was performed. Immunostaining in bile duct epithelium, cellular infiltrate, and vascular endothelial cells were scored as positive or negative. RESULTS The frequency of P-selectin-positive endothelium, platelets, and bile duct epithelium was significantly higher in the BA group (72%, 72%, and 63%, respectively) than in the non-BA group (32%, 16%, and 13%, respectively) with P of 0.002, <0.0001, and <0.0001, respectively. Few mononuclear cellular infiltrates in portal tract expressed P-selectin and were comparable in both groups (P = 0.932). Of interest, the platelet count was significantly higher in the BA group (532 ± 172) than in the non-BA group (406 ± 158), and 68.8% of the BA group had thrombocytosis versus 25% in the controls (P = 0.001 for both). CONCLUSIONS The significant expression of P-selectin in endothelium, platelets, and bile duct epithelium in patients with BA suggests a potential role for this adhesion molecule in the pathogenesis of this devastating neonatal hepatic disorder. It further suggests that platelets in BA are activated and may have a role in the inflammatory process in BA.
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Affiliation(s)
- Mostafa M Sira
- *Department of Pediatric Hepatology †Department of Pathology, National Liver Institute, Menofiya University, Shebin El-koom, Menofiya, Egypt
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Lee SM, Cheon JE, Choi YH, Kim WS, Cho HH, Cho HH, Kim IO, You SK. Ultrasonographic Diagnosis of Biliary Atresia Based on a Decision-Making Tree Model. Korean J Radiol 2015; 16:1364-72. [PMID: 26576128 PMCID: PMC4644760 DOI: 10.3348/kjr.2015.16.6.1364] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2015] [Accepted: 09/19/2015] [Indexed: 01/11/2023] Open
Abstract
Objective To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). Materials and Methods From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Results Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Conclusion Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.
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Affiliation(s)
- So Mi Lee
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Jung-Eun Cheon
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Young Hun Choi
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Woo Sun Kim
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | | | - Hyun-Hye Cho
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - In-One Kim
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Sun Kyoung You
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
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Hanquinet S, Courvoisier DS, Rougemont AL, Dhouib A, Rubbia-Brandt L, Wildhaber BE, Merlini L, McLin VA, Anooshiravani M. Contribution of acoustic radiation force impulse (ARFI) elastography to the ultrasound diagnosis of biliary atresia. Pediatr Radiol 2015; 45:1489-95. [PMID: 25943691 DOI: 10.1007/s00247-015-3352-6] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2014] [Accepted: 04/01/2015] [Indexed: 02/08/2023]
Abstract
BACKGROUND Children with biliary atresia rapidly develop liver fibrosis secondary to inflammatory destruction of the biliary tract. Noninvasive detection of liver fibrosis in neonatal/infantile cholestasis is an additional criterion for the diagnosis of biliary atresia, leading to prompt surgical exploration. OBJECTIVE To assess the value of US with acoustic radiation force impulse (ARFI) elastography to detect biliary atresia in the workup of neonatal/infantile cholestasis. MATERIALS AND METHODS In this retrospective study, 20 children with cholestasis suspected of having biliary atresia were investigated by US and ARFI. We evaluated the association between US findings and the diagnosis of biliary atresia and with two scores of liver fibrosis obtained from liver biopsy. RESULTS In univariate analyses, gallbladder size, triangular cord sign, spleen size and ARFI values were found to be associated with biliary atresia, though only the triangular cord sign remained significant when elevated gamma glutamyltransferase (GGT) was included as a predictor. In contrast, spleen size and ARFI correlated with the degree of liver fibrosis on biopsy (r > 0.70, P < 0.001), which remained significant when gamma glutamyltransferase elevation was included as a predictor. CONCLUSION The addition of ARFI to a standard abdominal US in the initial workup of the neonate with possible infantile cholestasis can provide reliable information on liver fibrosis and help in the diagnosis of biliary atresia.
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Affiliation(s)
- Sylviane Hanquinet
- Department of Pediatric Radiology, University Children's Hospital, 6 rue Willy Donzé, Ch 1211, Geneva 14, Switzerland,
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Abstract
To date, the etiology and pathogenic underpinning of the progression of the most prevalent serious neonatal liver disease, biliary atresia, remains elusive. This disease presents as an aggressive form of neonatal cholestasis characterized by the destruction and obliteration of the extrahepatic bile ducts within the first few weeks of life and a rapid progression of biliary fibrosis, likely due to unremitting cholestasis and retention of biliary constituents including bile acids. In ∼5% of patients, biliary atresia is associated with laterality features, suggesting a genetic underpinning to a disease that begins soon after birth. However, biliary atresia does not occur within families and twins are discordant, indicating an absence of strict mendelian inheritance. Despite this, genes related to bile duct dysmorphogenesis/ciliopathies overlapping with features of biliary atresia in both humans and nonhuman model systems have been proposed. Taken together, strict genetic etiologies leading to a common pathway of a neonatal cholangiopathy resulting in biliary atresia remain elusive. Contributions from fibrogenesis- and inflammation-based studies suggest that early engagement of these pathways contributes to disease progression, but a recent double-blind study did not suggest any benefit from early use of corticosteroids. However, there are genetic contributions to the adaptation and response to cholangiopathies and cholestasis that may be present in certain populations that likely impact upon the response to hepatoportoenterostomy and subsequent biliary tract function. Studies utilizing next generation sequencing technologies (e.g., exome analysis) are ongoing in several laboratories around the world; they are expected to provide insights into genetic contributions to biliary atresia outcomes. Altogether, combinations of exome sequencing and large population studies are expected to reveal causative and modifying genes relevant to patients with biliary atresia as a means to provide therapeutic targets and potential opportunities for genetic screening.
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Affiliation(s)
- Anya Mezina
- Emory University School of Medicine, Atlanta, Ga., USA
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El-Guindi MAS, Sira MM, Sira AM, Salem TAH. Reply to: "A scoring system for biliary atresia: Is this the right one?". J Hepatol 2015; 62:986-987. [PMID: 25500722 DOI: 10.1016/j.jhep.2014.12.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2014] [Accepted: 12/05/2014] [Indexed: 02/07/2023]
Affiliation(s)
- Mohamed Abdel-Salam El-Guindi
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Mostafa Mohamed Sira
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
| | - Ahmad Mohamed Sira
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Tahany Abdel-Hameed Salem
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
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Götze T, Blessing H, Grillhösl C, Gerner P, Hoerning A. Neonatal Cholestasis - Differential Diagnoses, Current Diagnostic Procedures, and Treatment. Front Pediatr 2015; 3:43. [PMID: 26137452 PMCID: PMC4470262 DOI: 10.3389/fped.2015.00043] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/20/2014] [Accepted: 04/29/2015] [Indexed: 12/18/2022] Open
Abstract
Cholestatic jaundice in early infancy is a complex diagnostic problem. Misdiagnosis of cholestasis as physiologic jaundice delays the identification of severe liver diseases. In the majority of infants, prolonged physiologic jaundice represent benign cases of breast milk jaundice, but few among them are masked and caused by neonatal cholestasis (NC) that requires a prompt diagnosis and treatment. Therefore, a prolonged neonatal jaundice, longer than 2 weeks after birth, must always be investigated because an early diagnosis is essential for appropriate management. To rapidly identify the cases with cholestatic jaundice, the conjugated bilirubin needs to be determined in any infant presenting with prolonged jaundice at 14 days of age with or without depigmented stool. Once NC is confirmed, a systematic approach is the key to reliably achieve the diagnosis in order to promptly initiate the specific, and in many cases, life-saving therapy. This strategy is most important to promptly identify and treat infants with biliary atresia, the most common cause of NC, as this requires a hepatoportoenterostomy as soon as possible. Here, we provide a detailed work-up approach including initial treatment recommendations and a clinically oriented overview of possible differential diagnoses in order to facilitate the early recognition and a timely diagnosis of cholestasis. This approach warrants a broad spectrum of diagnostic procedures and investigations including new methods that are described in this review.
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Affiliation(s)
- Thomas Götze
- Department for Pediatric and Adolescent Medicine, Friedrich-Alexander University of Erlangen-Nuremberg , Erlangen , Germany
| | - Holger Blessing
- Department for Pediatric and Adolescent Medicine, Friedrich-Alexander University of Erlangen-Nuremberg , Erlangen , Germany
| | - Christian Grillhösl
- Department for Pediatric and Adolescent Medicine, Friedrich-Alexander University of Erlangen-Nuremberg , Erlangen , Germany
| | - Patrick Gerner
- Department for Pediatric and Adolescent Medicine, Albert-Ludwigs-University Freiburg , Freiburg , Germany
| | - André Hoerning
- Department for Pediatric and Adolescent Medicine, Friedrich-Alexander University of Erlangen-Nuremberg , Erlangen , Germany
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El-Guindi MAS, Sira MM, Sira AM, Salem TAH. Reply to: "A diagnostic score for biliary atresia". J Hepatol 2014; 61:1440-1441. [PMID: 25219885 DOI: 10.1016/j.jhep.2014.09.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/26/2014] [Accepted: 09/02/2014] [Indexed: 02/07/2023]
Affiliation(s)
| | - Mostafa Mohamed Sira
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, Shebin El-koom, Menofiya, Egypt.
| | - Ahmad Mohamed Sira
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, Shebin El-koom, Menofiya, Egypt
| | - Tahany Abdel-Hameed Salem
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, Shebin El-koom, Menofiya, Egypt
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Abstract
OBJECTIVES Discrimination of biliary atresia (BA) from other causes of neonatal cholestasis (NC) is challenging. We aimed to analyze the clinicopathological findings in cholestatic infants who were provisionally diagnosed with BA and then excluded by intraoperative cholangiography compared with those with a definitive diagnosis of BA and to shed light on common misdiagnoses of BA. METHODS We retrospectively analyzed the data of infants diagnosed preoperatively with BA and referred to surgery between the years 2009 and 2013. On the basis of intraoperative cholangiography results, infants were divided into those with a definitive diagnosis of BA and those misdiagnosed with BA. RESULTS Out of 147 infants, there was a misdiagnosis of BA in 10 (6.8%) infants. Alanine transaminase was significantly higher in the non-BA group, whereas other clinical and laboratory findings were comparable in both groups. Hepatomegaly and abnormal gallbladder in ultrasound, and ductular proliferation and advanced grades of portal fibrosis in liver biopsy were significantly higher in infants with BA. However, giant cells were more common in the non-BA infants. Nonetheless, the frequency of clay stool, hepatomegaly, abnormal gallbladder, ductular proliferation, and advanced portal fibrosis was remarkable (100, 70, 40, 70, and 50%, respectively) in the misdiagnosed infants. The misdiagnoses were idiopathic neonatal hepatitis, progressive familial intrahepatic cholestasis type 3, cytomegalovirus hepatitis, Alagille syndrome, and a cholangitic form of congenital hepatic fibrosis. CONCLUSION A meticulous preoperative workup should be performed to exclude other causes of NC even if signs of BA are present, especially if features such as giant cells in histopathology are present. This involves completing the NC workup in parallel involving all common causes of NC rather than performing them in series to avoid loss of valuable time and efforts.
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Affiliation(s)
- Mostafa M Sira
- Departments of aPediatric Hepatology bHepatobiliary Surgery, National Liver Institute, Menofiya University, 32511, Shebin El-koom, Menofiya, Egypt
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El-Guindi MAS, Sira MM, Sira AM, Salem TAH, El-Abd OL, Konsowa HAS, El-Azab DS, Allam AAH. Design and validation of a diagnostic score for biliary atresia. J Hepatol 2014; 61:116-123. [PMID: 24657403 DOI: 10.1016/j.jhep.2014.03.016] [Citation(s) in RCA: 92] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2013] [Revised: 02/22/2014] [Accepted: 03/10/2014] [Indexed: 02/06/2023]
Abstract
BACKGROUND & AIMS The dilemma of early diagnosis of biliary Atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis is challenging. The aim was to design and validate a scoring system for early discrimination of BA from other causes of neonatal cholestasis. METHODS A twelve-point scoring system was proposed according to clinical, laboratory, ultrasonographic, and histopathological parameters. A total of 135 patients with neonatal cholestasis in two sets were recruited to design (n=60) and validate (n=75) a scoring system. Parameters with significant statistical difference between BA (n=30) and non-BA (n=30) patients in the design set were analyzed by logistic regression to predict the presence or absence of BA then a scoring system was designed and validated. RESULTS The total score ranged from 0 to 37.18 and a cut-off value of >23.927 could discriminate BA from other causes of neonatal cholestasis with sensitivity and specificity of 100% each. By applying this score in the validation set, the accuracy was 98.83% in predicting BA. The diagnosis of BA was proposed correctly in 100% and the diagnosis of non-BA was proposed correctly in 97.67% of patients. By applying this model, unnecessary intraoperative cholangiography would be avoided in non-BA patients. CONCLUSIONS This scoring system accurately separates infants with BA and those with non-BA, rendering intraoperative cholangiography for confirming or excluding BA unnecessary in a substantial proportion of patients.
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Affiliation(s)
- Mohamed Abdel-Salam El-Guindi
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Mostafa Mohamed Sira
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
| | - Ahmad Mohamed Sira
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Tahany Abdel-Hameed Salem
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Osama Lotfy El-Abd
- Department of Radiology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Hatem Abdel-Sattar Konsowa
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Dina Shehata El-Azab
- Department of Pathology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
| | - Alif Abdel-Hakim Allam
- Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt
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