A Prospective Multicentre Study Evaluating the Performance of the Simple Biliary Atresia Scoring System in Predicting Biliary Atresia

A Prospective Multicentre Study Evaluating the Performance of the Simple Biliary Atresia Scoring System in Predicting Biliary Atresia | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article A Prospective Multicentre Study Evaluating the Performance of the Simple Biliary Atresia Scoring System in Predicting Biliary Atresia Nooraini Mahat, Li Wei Chiang, Yong Chen, Nazrul Hadi, Mohd Yusof Abdullah, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4741373/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 27 Aug, 2024 Read the published version in Pediatric Surgery International → Version 1 posted 7 You are reading this latest preprint version Abstract Purpose: Early diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates and infants. We evaluate the performance of the Simple BA Scoring System (SBASS) in diagnosing BA. Methods: With ethical approval, we performed a prospective, cross-sectional study on all infants with cholestatic jaundice referred to three paediatric surgical tertiary centres from June 2021-December 2022. Diagnosis of BA was confirmed by cholangiography and histological findings. We excluded patients who had excretion of hepatic iminodiacetic acid, and those without all SBASS score parameters available. SBASS scoring was applied to all patients and scores were compared to the eventual diagnosis. The score consists of two ultrasonographic findings (gall bladder length 0.7 score 2, gamma-glutamyl transferase(GGT) > 200 U/L score 2). The maximum score was 6. Results: Of 97 patients referred, 73 were included in this study: 46 (63%) male, 64(87%) term babies. Fifty-two (71%) had BA (age 57 days [range 45–68]) while 21(29%) were non-BA (age 59 days, [range 54–68]). In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15(72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the scoring system showed a sensitivity of 96.2%, specificity of 61.9% positive predictive value of 86.2%, negative predictive value of 86.7%, and overall accuracy of 86.3%. GGT had the highest sensitivity (94.2%) of the four clinical parameters, while triangular cord sign was the most specific (95.2%) in diagnosing BA. Conclusion: Our study shows that the SBASS provides a bedside, non-invasive scoring system to differentiate BA from other cholestatic jaundice causes in infants. Utilizing this scoring system allows for the exclusion of BA using a low risk method and reduces the likelihood of negative surgical explorations. Biliary atresia scoring cholestatic jaundice predictive value Figures Figure 1 Figure 2 Highlights What is currently known about this topic? Distinguishing biliary atresia from other causes of neonatal cholestasis is difficult, and although several scoring systems have been suggested to enhance diagnostic accuracy, none have achieved widespread acceptance in clinical practice. What new information is contained in this article? The Simple BA Scoring System (SBASS) is a non-invasive scoring system that enables low-risk exclusion of biliary atresia, effectively reducing the likelihood of unnecessary surgical explorations. We propose an algorithm for workup to differentiate between biliary atresia and other causes of neonatal cholestatic jaundice. Introduction Early diagnosis of biliary atresia (BA) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver [ 1 ]. Although intraoperative cholangiography (IOC) is required to confirm the diagnosis of BA, it is an invasive procedure with considerable increase morbidity. The lack of a standardized preoperative evaluation for infants with cholestatic jaundice often leads to unnecessary surgical explorations, increasing both morbidity and treatment cost [ 2 ]. Many clinical, imaging, and histological parameters have been reported with various levels of accuracy. The universal screening program in Taiwan, which uses stool color cards, reflects the importance of early diagnosis and treatment of BA. However, it lacks specificity as similar results of pale stool may be observed in other non-BA conditions [ 3 ]. There is no single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of neonatal cholestasis [ 1 ]. Others have noted the association of BA with elevated GGT > 300 U/L giving a specificity of 100% and sensitivity of 67% [ 3 – 5 ]. The presence of the triangular cord sign on ultrasound imaging, though helpful in diagnosis, is not always found in every BA patient. Mittal et al. also found the triangular cord sign is specific (97%) but not sensitive (23%) [ 6 ]. Not finding a gall bladder was a specific finding for BA (100%) but also not sensitive (23%) [ 6 ]. Screening for BA is challenging and the results of previous scoring systems have been suboptimal [ 1 – 4 , 7 ]. The Simple BA Scoring System (SBASS) was introduced by Chiang et al [ 8 ]. They reported a high sensitivity of 97% in diagnosing BA. Compared to other scoring systems, the advantages of using the SBASS are that it consists of a small number of variables and that it is easy to use as a bedside diagnostic score for BA. The aim of this study is to prospectively evaluate the diagnostic value of SBASS in differentiating BA from other infantile cholestasis. Methodology We conducted a prospective cohort study with a cross-sectional design at three paediatric surgical tertiary centres in Malaysia, all of which regularly receive referrals for and manage patients with BA. The three centres were: Hospital Tunku Azizah (Women and Children’s Hospital Kuala Lumpur) - serves as a tertiary referral centre for paediatric surgery cases, accepting referrals from across the country. It also functions as a training centre for doctors and health professionals. Hospital Sultanah Aminah (HSA) - the primary referral centre for paediatric surgical cases in Southern Peninsular Malaysia. University of Malaya Medical Centre (UMMC) - a university-affiliated academic centre serving the mainly urban population in the Klang Valley. The study was conducted from June 2021 to December 2022. All babies with cholestatic jaundice referred to the above three tertiary paediatric surgical centres in Malaysia with a possible diagnosis of BA were identified. A web-based data collection tool (Google Forms) was used to collect data after the first visit to the referral centre, which consisted of demographic data, ultrasonography findings, and liver function tests. All patients were managed according to standard institutional protocols for neonatal cholestatic jaundice and followed up until the diagnosis was known. The diagnosis of BA or non-BA was confirmed by percutaneous cholangiogram or intraoperative cholangiogram, together with the histology result of any liver biopsy performed. The Simple BA Scoring System (SBASS) consists of 4 parameters with a total score of 6 (Table 1 ). The SBASS scoring was calculated for all patients included in this study. Table 1 The Simple Biliary Atresia Scoring System Parameters Score Ultrasound of the hepatobiliary system Gall bladder length 0.7 2 Gamma-glutamyl transferase > 200 (U/L) 2 Maximum score = 6 Score < 3: Likely Not BA Score ≥ 3: Likely BA BA (biliary atresia) Statistical Analysis Data were analyzed using the Statistical Package for the Social Sciences (SPSS) software (IBM SPSS Statistics 23). Descriptive statistics for continuous data were presented as means with standard deviations, and categorical data as numbers and frequencies (%). Mann-Whitney tests were used to assess the homogeneity of the patients. Pearson's Chi-square test was used to evaluate the comparison between categorical data. A p-value of less than 0.05 was considered statistically significant. Diagnostic accuracy of SBASS scoring system was determined by using receiver operating characteristics (ROC) curves. We calculated sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy for each cut-off point (cut off point 3, cut off point 4 and cut off point 5). We also calculated sensitivity, specificity, PPV, NPV of each parameter in the scoring system. T-tests were used to compare the means of individual parameters in the scoring system of patients with and without BA. Ethics Approval This study was approved by the Medical Research and Ethics Committee board, Ministry of Health Malaysia. NMRR.ID: NMRR-21-736-59. Results Ninety-seven infants with cholestatic jaundice were referred to three paediatric surgery tertiary centers in Malaysia for possible BA from June 2021 till December 2022. Twenty-four patients were excluded from the study due to incomplete data. Of the 73 patients included in this study, the majority (n = 46, 63%) were male (Table 2 ). Table 2 Demographic data comparing biliary atresia to non-biliary atresia patients. Total n = 73 n (%) Biliary atresia n = 52 n (%) Non- biliary atresia n = 21 n (%) P-value Gender Male 46 (63) 30 (57.7) 16 (76.2) 0.18 a Female 27 (37) 22 (42.3) 5 (23.8) Gestational age (weeks) Preterm (32-36weeks) 9 (12.3) 5 (9.6) 4 (19.0) 0.27 b Term (37-40weeks) 64 (87.7) 47 (90.4) 17 (81.0) Age at diagnosis (days) Mean (SD) 62.4 (26.6) 60.6 (24.0) 66.7 (32.2) 0.38 c Median (IQR) 58.0 (49.0–68.0) 57 (45.5–68.0) 59 (54.0-68.5) Min - Max 22–200 22–173 42–200 Birth weight (kg) Mean (SD) 2.89 (0.5) 2.98 (0.5) 2.66 (0.5) 0.02 c Weight at time of diagnosis (kg) Mean (SD) 4.22 (0.9) 4.32 (0.7) 3.99 (1.1) 0.22 c Pale stool at first review Yes 59 (72.6) 48 (92.3) 11 (47.6) < 0.001 b No 14(27.4) 4 (7.7) 10 (52.4) Intervention PTC 6 (8.2) 0 (0.0) 6 (28.6) OTC 16 (21.9) 1 (1.0) 15 (71.4) Kasai Procedure 51 (69.9) 51 (98.0) 0 (0.0) SD: standard deviation, IQR: inter-quartile range, PTC: percutaneous cholangiogram), OTC: on table cholangiogram, a- chi square test, b- Fisher exact test, c- T-test We diagnosed 52 (72%) babies with BA, while another 21(28%) were non-BA. The BA group had a higher birth weight compared to the non-BA group babies (P = 0.02). In the BA group, 48 babies (92.3%) had pale stool observed at their first visit to the referral centre, compared to only 11 babies (52.4%) in the non-BA group. This difference was statistically significant, with p-value < 0.001. One infant with BA did not have a Kasai procedure performed but was planned for primary liver transplantation due to the advanced stage of liver cirrhosis present. To assess the applicability of this scoring system, this study was conducted on our sample of 73 patients diagnosed with cholestatic jaundice. The cut-off point was determined using the Receiver Operating Characteristic (ROC) curve, as shown in Fig. 1 . Using a score with cut-off point of 3 and above, the sensitivity was 96.2% and specificity was 61.9% while with a cut-off point of 5, the sensitivity was 61.5% and specificity was 95.2% (Table 3 ). Table 3 Diagnostic value of the Simple Biliary Atresia Scoring System at various cut-off scores. SCORE 3 AND ABOVE 4 AND ABOVE 5 AND ABOVE Sensitivity (95% CI) 96.2 (90.9–101.3) 82.7 (72.4–92.9) 61.5 (48.3–74.7) Specificity (95% CI) 61.9 (41.1–82.6) 81.0 (64.1–97.7) 95.2 (86.1–104.3) Positive Predictive Value (95% CI) 86.2 (77.3–95.0) 91.5 (83.5–99.4) 97.0 (91.1–102.8) Negative Predictive Value (95% CI) 86.7 (69.4–103.8) 65.4 (47.0–83.6) 50.0 (34.5–65.4) Accuracy (95% CI) 86.3 (78.4–94.1) 82.1 (73.3–90.8) 71.2 (60.8–81.5) 95% CI = 95% Confidence interval When we assessed individual parameters in the SBASS, GGT > 200U/L had the highest sensitivity of 94.2% and specificity of 66.7% (Table 4 ). Table 4 Sensitivity, specificity, positive predictive value and negative predictive value of individual parameters in the Simple Biliary Atresia Scoring System (SBASS) for diagnosing biliary atresia. Gall bladder length 0.7 Gamma-glutamyl transferase > 200U/L Sensitivity (%) 63.5 34.6 84.6 94.2 Specificity (%) 76.2 95.2 23.8 66.7 Positive Predictive Value (%) 86.8 94.7 73.3 87.5 Negative Predictive Value (%) 45.7 37.0 38.5 82.4 The mean GGT in the BA group was significantly higher than in the non-BA group, with respective values of 580U/L and 174U/L (P < 0.001) (Table 5 ). In addition, the length of gall bladder was significantly smaller in BA compared to non-BA with a mean of 1.0cm and 1.9cm respectively (P < 0.004). Table 5 Mean and standard deviation of individual parameters in the Simple Biliary Atresia Scoring System (SBASS) comparing biliary atresia and non-biliary atresia groups . PARAMETERS Biliary atresia Non- biliary atresia P-Value Gall bladder length on ultrasound (cm) Mean 1.08 1.87 0.004 SD 1.02 1.04 Min -Max 0–4 0–4 Total bilirubin (umol/L) Mean 163 170 0.690 SD 43 71 Min- Max 103–289 62–371 Direct bilirubin:Total bilirubin ratio Mean 0.76 0.73 0.297 SD 0.09 0.08 Min - Max 0.50–1.00 0.62–3.71 Gamma-glutamyl transferase (U/L) Mean 580 174 < 0.001 SD 346 111 Min - Max 126–1680 30–459 SD: Standard deviation Discussion In our study, we evaluated the performance of the simple biliary atresia scoring system (SBASS), to differentiate between BA and other causes of neonatal cholestatic disorders. We found that this scoring system has a high sensitivity of 96.2%, specificity of 61.9%, positive predictive value of 86.2%, negative predictive value of 86.7% and accuracy of 86.3% using a cut-off of 3 and above, in diagnosis of BA. The AUC of this scoring system was 0.901 (95% CI,0.823–0.978), indicating good discrimination of BA. Our study showed that GGT > 200U/L has a high sensitivity (94.2%) and a high positive predictive value (87.5%). Several studies have also reported higher GGT levels in BA compared to other cholestatic disorders, with a GGT level > 300 U/L having 85% accuracy in diagnosing BA [ 2 , 6 , 9 ]. Additionally, our study showed that, the ratio of direct hyperbilirubinemia to total bilirubin > 70% has sensitivity of 84.6%, specificity of 23% and positive predictive value of 73%. This is in line with a study reported by Harpavat et al. that found elevated serum direct conjugated bilirubin in 34 BA patients within the first 96 hours of age[ 10 ]. In 2016, Hapavat et al. initiated a prospective study using two-step screening with direct bilirubin measurement at 60 hours of life and at 2 weeks of age; the sensitivity, specificity, and positive predictive value were 100%, 99.9%, and 18.2% respectively [ 11 ]. They also postulated that a direct conjugated bilirubin to total bilirubin ratio < 15% has a specificity of 99.3%. Numerous ultrasonographic features have been described as useful pointers to the diagnosis of biliary atresia. In our study, we found that the triangular cord sign is an important ultrasonographic feature in diagnosing BA, which is consistent with findings from other studies. In a meta-analysis of 17 studies from 1996 to 2015, Yoon et al. reported a sensitivity of 85% and specificity of 97% for the diagnosis of BA using the triangular cord sign [ 12 ]. Additionally, in our study, a gall bladder length < 1.6cm showed sensitivity of 63.5% and specificity of 76.2% for diagnosing BA. Yoon et al reported that the most common cut off sizes for gall bladder length (< 1.5cm or < 1.9cm) in 8 studies had a sensitivity range of 50%-100% and specificity range of 68%-99% [ 12 ]. Humphrey and Stinger reported that the features with the greatest individual sensitivity and specificity respectively in diagnosing BA were triangular cord sign (73% and 100%), abnormal gall bladder wall (91% and 95%), abnormal gall bladder shape (70% and 100%), and absence of the common bile duct (93% and 92%) [ 13 ]. Nevertheless, despite the extensive studies on radiological features indicating BA, there is no single feature with sufficient sensitivity and specificity to reliably differentiate BA from other causes of neonatal cholestasis [ 1 ]. An effective bedside diagnostic score for biliary atresia (BA) in cholestatic infants is still lacking, and none of the few proposed in the past has achieved widespread application in clinical practice [ 7 ]. El Guindi and colleagues used a twelve-point diagnostic score which consists of clinical, ultrasound findings, laboratory and histopathological results [ 2 ]. However, the scoring system is not reproducible as reported by Sciveres, and taking a liver biopsy in all children with direct hyperbilirubinemia is not warranted [ 2 , 7 ]. In comparison to other scoring systems, the merits of using the SBASS are that it consists of a few variables that are readily obtained in any initial workup of an infant with cholestatic jaundice [ 1 , 2 , 4 , 5 , 14 , 15 ]. To our knowledge, the SBASS is the simplest scoring system available with high sensitivity and accuracy. When an SBASS score of < 3 is obtained, other diagnostic tests can be used prior to deciding on surgery, such as PTC and liver biopsy. In our study, PTC was used to exclude biliary atresia in 6 (28%) of the cases, and this approach is similar to others such as Jensen et al who reported a sensitivity of 100% and specificity of 86% of PTC in diagnosing BA [ 16 ]. This workflow helps to relieve strain on operating theatre resources and also reduces the risks associated with surgical procedures under general anaesthesia in small infants [ 16 , 17 ]. However, strict selection criteria must be used to avoid failure of this procedure, including ultrasound identification of gallbladder size. Even in institutions where there is a low threshold for surgical exploration for cholestatic jaundice in infants, a low SBASS score (< 3) can still be helpful in guiding preoperative preparations. For example, the anaesthetic team may opt not to insert central venous and intra-arterial lines at the beginning of the procedure, anticipating a low likelihood of BA, thus avoiding the patient safety risks associated with these invasive cannulations. We have proposed an algorithm, as shown in Fig. 2 , based on the findings from this study. HIDA (hepatobiliary iminodiacetic acid), PTC (percutaneous cholangiogram), EHBT (extrahepatic biliary tree), GB (gall bladder), BA (biliary atresia), KPE (Kasai portoenterostomy). Figure 2 : Proposed algorithm for workup to differentiate between biliary atresia and other causes of neonatal cholestatic jaundice. Several limitations of the present study should be acknowledged. Firstly, ultrasound is an operator-dependent procedure, and non-standardised ultrasound protocols across multiple institutions and referral sources can impact reports and interpretations. Hence, establishing a standardized ultrasound protocol for investigation of cholestatic jaundice in infants is required. Secondly, as BA is a progressive illness, biochemical and anatomical morphology can evolve along the course of the disease. It is possible that very early patient referrals at lower gestational age may result in falsely low scores even in the presence of BA and vice versa. Our study was not designed to account for this factor and longer-follow up for these cohorts might be required. We are also mindful of the presence of referral bias, particularly in patients referred by a paediatric gastroenterology team who are likely to have worked up and excluded a number of patients, eventually forwarding only those more likely to have BA. To mitigate this bias in the future, we can apply our scoring method in the primary health care setting to assess whether it yields comparable outcomes. Conclusion Our study demonstrates that the SBASS provides a bedside, non-invasive scoring system for differentiating BA from other causes of cholestatic jaundice in infants. By utilizing this scoring system, it allows for the exclusion of BA using a low-risk method, thereby reducing the likelihood of negative surgical explorations. Declarations Author Contribution Authors' contributions: NM, LWC, YC & SAN conceptualized the study. NM & NHAR collected the data. NM, LWC, YC, HHKS, MYA & SAN performed the data analysis. The first draft of the manuscript was written by NM. LWC, YC, AS, SS & SAN provided critical review of the manuscript. All authors read and approved the final manuscript. Acknowledgement We would also like to express our gratitude to Dr Mohd Fauzi Sharudin, Head Unit and Senior Consultant Paediatric Surgery Hospital Sultanah Aminah, and Dr Muhammad Firdaus Syukri from University of Malaya Medical Centre, Kuala Lumpur for contributing and sharing their pearl of wisdom in this research. References Lee WS, Chai PF (2010) Clinical features differentiating biliary atresia from other causes of neonatal cholestasis. Ann Acad Med Singap 39(8):648–654 El-Guindi MA, Sira MM, Sira AM et al (2014) Design and validation of a diagnostic score for biliary atresia. J Hepatol. 2014;61(1):116 – 23. 10.1016/j.jhep.2014.03.016 Lien TH, Chang MH, Wu JF et al (2011) Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology. 2011;53(1):202-8. 10.1002/hep.24023 Robie DK, Overfelt SR, Xie L (2014) Differentiating biliary atresia from other causes of cholestatic jaundice. Am Surg 80(9):827–831 Gupta DK, Srinivas M, Bajpai M (2001) AIIMS clinical score: a reliable aid to distinguish neonatal hepatitis from extra hepatic biliary atresia. Indian J Pediatr 68(7):605–608. 10.1007/BF02752271 Tang KS, Huang LT, Huang YH et al (2007) Gamma-glutamyl transferase in the diagnosis of biliary atresia. Acta Paediatr Taiwan 48(4):196–200 Sciveres M, Milazzo MP, Maggiore G (2014) A scoring system for biliary atresia: is this the right one? J Hepatol. 2015;62(4):985-6. 10.1016/j.jhep.2014.11.042 Chiang LW et al (September 2019) Unpublished data. ASEAN Society of Peadiatric Surgery Liu CS, Chin TW, Wei CF (1998) Value of gamma-glutamyl transpeptidase for early diagnosis of biliary atresia. Zhonghua Yi Xue Za Zhi (Taipei) 61(12):716–720 Harpavat S, Finegold MJ, Karpen SJ (2011) Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics.128(6):e1428-33. 10.1542/peds.2011-1869 . Epub 2011 Nov 21. PMID: 22106076 Harpavat S, Ramraj R, Finegold MJ et al (2016) Newborn Direct or Conjugated Bilirubin Measurements As a Potential Screen for Biliary Atresia. J Pediatr Gastroenterol Nutr 62(6):799–803. 10.1097/MPG.0000000000001097 Zhou L, Shan Q, Tian W et al (2016) Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis. AJR Am J Roentgenol 206(5):W73–82. 10.2214/AJR.15.15336 Epub 2016 Mar 24 Humphrey TM, Stringer MD (2007) Biliary atresia: US diagnosis. Radiology 244(3):845–851. 10.1148/radiol.2443061051 Chiba T, Kasai M (1975) Differentiation of biliary atresia from neonatal hepatitis by routine clinical examinations. Tohoku J Exp Med 115(4):327–335. 10.1620/tjem.115.327 Hsiao CH, Chang MH, Chen HL et al (2008)Taiwan Infant Stool Color Card Study Group. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 47(4):1233–1240. 10.1002/hep.22182 Jensen MK, Biank VF, Moe DC et al (2012) HIDA, percutaneous transhepatic cholecysto-cholangiography and liver biopsy in infants with persistent jaundice: can a combination of PTCC and liver biopsy reduce unnecessary laparotomy? Pediatr Radiol 42(1):32–39. 10.1007/s00247-011-2202-4 Epub 2011 Jul 24 Ashritha A, Gautam V, Lal BB et al (2022) Percutaneous Cholecystocholangiography-A Tool to Conclusively Exclude Biliary Atresia. Indian J Pediatr 89(11):1144–1147. 10.1007/s12098-022-04354-2 Epub 2022 Sep 20 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 27 Aug, 2024 Read the published version in Pediatric Surgery International → Version 1 posted Editorial decision: Revision requested 31 Jul, 2024 Reviews received at journal 25 Jul, 2024 Reviewers agreed at journal 16 Jul, 2024 Reviewers invited by journal 16 Jul, 2024 Editor assigned by journal 16 Jul, 2024 Submission checks completed at journal 16 Jul, 2024 First submitted to journal 15 Jul, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4741373","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":334357224,"identity":"e5fdc24e-afab-41af-871b-d2b489c6dc6c","order_by":0,"name":"Nooraini Mahat","email":"","orcid":"","institution":"University of Malaya","correspondingAuthor":false,"prefix":"","firstName":"Nooraini","middleName":"","lastName":"Mahat","suffix":""},{"id":334357225,"identity":"9b9d9f3c-b945-4bb4-9da6-c853e70b99ae","order_by":1,"name":"Li Wei Chiang","email":"","orcid":"","institution":"KK Women’s and Children’s 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Kyaw","lastName":"Soe","suffix":""},{"id":334357232,"identity":"567865db-e82e-4d47-b5dc-71300ff506a9","order_by":8,"name":"Shireen Anne Nah","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA40lEQVRIiWNgGAWjYDACCcYGKIuH8QGYPkCCFmYDIrXAWTxsEkRpkZ/d3PbhQ8UdBnP2s8eqeXMY5PhuJLBu5sGjxeDOweaZM848Y7DsyUu7zbuNwVjyRgLbbbxaJBKbmXnbDjMYHMgxA2lJ3EBIi/wMoJa//4Bazr8xKwZqqSeoheEGUAtjA1DLjRwzZqCWBAOCDgNqYew5dpjH4MYbY8m52yQMZ5552HZzDl6HpT9m+FFzWM7gfI7hh7fbbOT5jicfu/EGn8OgAOYSUNQwNjDh9QtWwPiDZC2jYBSMglEwjAEArptQM+PA0g0AAAAASUVORK5CYII=","orcid":"","institution":"University of Malaya","correspondingAuthor":true,"prefix":"","firstName":"Shireen","middleName":"Anne","lastName":"Nah","suffix":""}],"badges":[],"createdAt":"2024-07-15 07:38:39","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4741373/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4741373/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00383-024-05830-w","type":"published","date":"2024-08-27T15:57:30+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":62115423,"identity":"f67fe127-1e1d-415e-a572-520234fd34a8","added_by":"auto","created_at":"2024-08-09 12:47:15","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":17146,"visible":true,"origin":"","legend":"\u003cp\u003eReceiver Operating Characteristics (ROC) curve of the Simple Biliary Atresia Scoring System (SBASS) (area under the curve: 0.901).\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-4741373/v1/4f870b2f48c38945478e938b.png"},{"id":62115424,"identity":"2d016d00-0587-48b8-9f8b-31ec9463fccc","added_by":"auto","created_at":"2024-08-09 12:47:15","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":119612,"visible":true,"origin":"","legend":"\u003cp\u003eProposed algorithm for workup to differentiate between biliary atresia and other causes of neonatal cholestatic jaundice.\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-4741373/v1/2f4e4bdb2c5dbdeb6f429ef4.png"},{"id":63821324,"identity":"d8d61d89-1f9c-4232-b87b-32b4ac1e10d7","added_by":"auto","created_at":"2024-09-02 16:13:11","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":729035,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4741373/v1/a5a26706-c6d5-483b-bba8-f452425fc37a.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eA Prospective Multicentre Study Evaluating the Performance of the Simple Biliary Atresia Scoring System in Predicting Biliary Atresia\u003c/p\u003e","fulltext":[{"header":"Highlights","content":"\u003cp\u003e\u003cstrong\u003eWhat is currently known about this topic?\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eDistinguishing biliary atresia from other causes of neonatal cholestasis is difficult, and although several scoring systems have been suggested to enhance diagnostic accuracy, none have achieved widespread acceptance in clinical practice.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eWhat new information is contained in this article?\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe\u0026nbsp;Simple BA Scoring System (SBASS) is a non-invasive scoring system that enables low-risk exclusion of biliary atresia, effectively reducing the likelihood of unnecessary surgical explorations.\u003c/p\u003e\n\u003cp\u003eWe propose an algorithm for workup to differentiate between biliary atresia and other causes of neonatal cholestatic jaundice.\u003c/p\u003e"},{"header":"Introduction","content":"\u003cp\u003eEarly diagnosis of biliary atresia (BA) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Although intraoperative cholangiography (IOC) is required to confirm the diagnosis of BA, it is an invasive procedure with considerable increase morbidity. The lack of a standardized preoperative evaluation for infants with cholestatic jaundice often leads to unnecessary surgical explorations, increasing both morbidity and treatment cost [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eMany clinical, imaging, and histological parameters have been reported with various levels of accuracy. The universal screening program in Taiwan, which uses stool color cards, reflects the importance of early diagnosis and treatment of BA. However, it lacks specificity as similar results of pale stool may be observed in other non-BA conditions [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. There is no single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of neonatal cholestasis [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Others have noted the association of BA with elevated GGT\u0026thinsp;\u0026gt;\u0026thinsp;300 U/L giving a specificity of 100% and sensitivity of 67% [\u003cspan additionalcitationids=\"CR4\" citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. The presence of the triangular cord sign on ultrasound imaging, though helpful in diagnosis, is not always found in every BA patient. Mittal et al. also found the triangular cord sign is specific (97%) but not sensitive (23%) [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Not finding a gall bladder was a specific finding for BA (100%) but also not sensitive (23%) [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eScreening for BA is challenging and the results of previous scoring systems have been suboptimal [\u003cspan additionalcitationids=\"CR2 CR3\" citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. The Simple BA Scoring System (SBASS) was introduced by Chiang et al [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. They reported a high sensitivity of 97% in diagnosing BA. Compared to other scoring systems, the advantages of using the SBASS are that it consists of a small number of variables and that it is easy to use as a bedside diagnostic score for BA.\u003c/p\u003e \u003cp\u003eThe aim of this study is to prospectively evaluate the diagnostic value of SBASS in differentiating BA from other infantile cholestasis.\u003c/p\u003e"},{"header":"Methodology","content":"\u003cp\u003eWe conducted a prospective cohort study with a cross-sectional design at three paediatric surgical tertiary centres in Malaysia, all of which regularly receive referrals for and manage patients with BA.\u003c/p\u003e \u003cp\u003eThe three centres were:\u003c/p\u003e \u003cp\u003e \u003col\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eHospital Tunku Azizah (Women and Children\u0026rsquo;s Hospital Kuala Lumpur) - serves as a tertiary referral centre for paediatric surgery cases, accepting referrals from across the country. It also functions as a training centre for doctors and health professionals.\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eHospital Sultanah Aminah (HSA) - the primary referral centre for paediatric surgical cases in Southern Peninsular Malaysia.\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eUniversity of Malaya Medical Centre (UMMC) - a university-affiliated academic centre serving the mainly urban population in the Klang Valley.\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003c/ol\u003e \u003c/p\u003e \u003cp\u003eThe study was conducted from June 2021 to December 2022.\u003c/p\u003e \u003cp\u003eAll babies with cholestatic jaundice referred to the above three tertiary paediatric surgical centres in Malaysia with a possible diagnosis of BA were identified. A web-based data collection tool (Google Forms) was used to collect data after the first visit to the referral centre, which consisted of demographic data, ultrasonography findings, and liver function tests. All patients were managed according to standard institutional protocols for neonatal cholestatic jaundice and followed up until the diagnosis was known. The diagnosis of BA or non-BA was confirmed by percutaneous cholangiogram or intraoperative cholangiogram, together with the histology result of any liver biopsy performed.\u003c/p\u003e \u003cp\u003eThe Simple BA Scoring System (SBASS) consists of 4 parameters with a total score of 6 (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). The SBASS scoring was calculated for all patients included in this study.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eThe Simple Biliary Atresia Scoring System\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"3\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eParameters\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eScore\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cb\u003eUltrasound of the hepatobiliary system\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGall bladder length\u0026thinsp;\u0026lt;\u0026thinsp;1.6cm\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePresence of triangular cord sign\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cb\u003eLiver function tests\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eThe ratio of conjugated to total bilirubin\u0026thinsp;\u0026gt;\u0026thinsp;0.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGamma-glutamyl transferase\u0026thinsp;\u0026gt;\u0026thinsp;200 (U/L)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"3\" nameend=\"c3\" namest=\"c1\"\u003e \u003cp\u003e\u003cb\u003eMaximum score\u0026thinsp;=\u0026thinsp;6\u003c/b\u003e\u003c/p\u003e \u003cp\u003e\u003cb\u003eScore\u0026thinsp;\u0026lt;\u0026thinsp;3: Likely Not BA\u003c/b\u003e\u003c/p\u003e \u003cp\u003e\u003cb\u003eScore\u0026thinsp;\u0026ge;\u0026thinsp;3: Likely BA\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"3\"\u003eBA (biliary atresia)\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eStatistical Analysis\u003c/h2\u003e \u003cp\u003eData were analyzed using the Statistical Package for the Social Sciences (SPSS) software (IBM SPSS Statistics 23). Descriptive statistics for continuous data were presented as means with standard deviations, and categorical data as numbers and frequencies (%). Mann-Whitney tests were used to assess the homogeneity of the patients. Pearson's Chi-square test was used to evaluate the comparison between categorical data. A p-value of less than 0.05 was considered statistically significant. Diagnostic accuracy of SBASS scoring system was determined by using receiver operating characteristics (ROC) curves. We calculated sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy for each cut-off point (cut off point 3, cut off point 4 and cut off point 5). We also calculated sensitivity, specificity, PPV, NPV of each parameter in the scoring system. T-tests were used to compare the means of individual parameters in the scoring system of patients with and without BA.\u003c/p\u003e \u003cp\u003e \u003cstrong\u003eEthics Approval\u003c/strong\u003e \u003cp\u003e This study was approved by the Medical Research and Ethics Committee board, Ministry of Health Malaysia. NMRR.ID: NMRR-21-736-59.\u003c/p\u003e \u003c/p\u003e \u003c/div\u003e"},{"header":"Results","content":"\u003cp\u003eNinety-seven infants with cholestatic jaundice were referred to three paediatric surgery tertiary centers in Malaysia for possible BA from June 2021 till December 2022. Twenty-four patients were excluded from the study due to incomplete data. Of the 73 patients included in this study, the majority (n\u0026thinsp;=\u0026thinsp;46, 63%) were male (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eDemographic data comparing biliary atresia to non-biliary atresia patients.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"6\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTotal\u003c/p\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;73\u003c/p\u003e \u003cp\u003en (%)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eBiliary atresia\u003c/p\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;52\u003c/p\u003e \u003cp\u003en (%)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNon- biliary atresia\u003c/p\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;21\u003c/p\u003e \u003cp\u003en (%)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eP-value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eGender\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e46 (63)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e30 (57.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e16 (76.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.18\u003csup\u003ea\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eFemale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27 (37)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22 (42.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e5 (23.8)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003eGestational age (weeks)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePreterm (32-36weeks)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e9 (12.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e5 (9.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e4 (19.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.27\u003csup\u003eb\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTerm (37-40weeks)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e64 (87.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e47 (90.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e17 (81.0)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eAge at diagnosis (days)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean (SD)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e62.4 (26.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e60.6 (24.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e66.7 (32.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e0.38\u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMedian (IQR)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e58.0 (49.0\u0026ndash;68.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e57 (45.5\u0026ndash;68.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e59 (54.0-68.5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMin - Max\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e22\u0026ndash;200\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22\u0026ndash;173\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e42\u0026ndash;200\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBirth weight (kg)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean (SD)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2.89 (0.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2.98 (0.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e2.66 (0.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e\u003cb\u003e0.02\u003c/b\u003e\u003csup\u003e\u003cb\u003ec\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eWeight at time of diagnosis (kg)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean (SD)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4.22 (0.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4.32 (0.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e3.99 (1.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.22\u003csup\u003ec\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003ePale stool at first review\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eYes\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e59 (72.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e48 (92.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e11 (47.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cb\u003e\u0026lt;\u0026thinsp;0.001\u003c/b\u003e\u003csup\u003e\u003cb\u003eb\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNo\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e14(27.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (7.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e10 (52.4)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eIntervention\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePTC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 (8.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e6 (28.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\" morerows=\"2\" rowspan=\"3\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOTC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e16 (21.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (1.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e15 (71.4)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eKasai Procedure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e51 (69.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e51 (98.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eSD: standard deviation, IQR: inter-quartile range, PTC: percutaneous cholangiogram), OTC: on table cholangiogram, a- chi square test, b- Fisher exact test, c- T-test\u003c/p\u003e \u003cp\u003eWe diagnosed 52 (72%) babies with BA, while another 21(28%) were non-BA. The BA group had a higher birth weight compared to the non-BA group babies (P\u0026thinsp;=\u0026thinsp;0.02). In the BA group, 48 babies (92.3%) had pale stool observed at their first visit to the referral centre, compared to only 11 babies (52.4%) in the non-BA group. This difference was statistically significant, with p-value\u0026thinsp;\u0026lt;\u0026thinsp;0.001. One infant with BA did not have a Kasai procedure performed but was planned for primary liver transplantation due to the advanced stage of liver cirrhosis present.\u003c/p\u003e \u003cp\u003eTo assess the applicability of this scoring system, this study was conducted on our sample of 73 patients diagnosed with cholestatic jaundice. The cut-off point was determined using the Receiver Operating Characteristic (ROC) curve, as shown in Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e1\u003c/span\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eUsing a score with cut-off point of 3 and above, the sensitivity was 96.2% and specificity was 61.9% while with a cut-off point of 5, the sensitivity was 61.5% and specificity was 95.2% (Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eDiagnostic value of the Simple Biliary Atresia Scoring System at various cut-off scores.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"4\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colspan=\"3\" nameend=\"c4\" namest=\"c2\"\u003e \u003cp\u003eSCORE\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 AND ABOVE\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4 AND ABOVE\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e5 AND ABOVE\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSensitivity\u003c/p\u003e \u003cp\u003e(95% CI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e96.2\u003c/p\u003e \u003cp\u003e(90.9\u0026ndash;101.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e82.7\u003c/p\u003e \u003cp\u003e(72.4\u0026ndash;92.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e61.5\u003c/p\u003e \u003cp\u003e(48.3\u0026ndash;74.7)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSpecificity\u003c/p\u003e \u003cp\u003e(95% CI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e61.9\u003c/p\u003e \u003cp\u003e(41.1\u0026ndash;82.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e81.0\u003c/p\u003e \u003cp\u003e(64.1\u0026ndash;97.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e95.2\u003c/p\u003e \u003cp\u003e(86.1\u0026ndash;104.3)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePositive Predictive Value\u003c/p\u003e \u003cp\u003e(95% CI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e86.2\u003c/p\u003e \u003cp\u003e(77.3\u0026ndash;95.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e91.5\u003c/p\u003e \u003cp\u003e(83.5\u0026ndash;99.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e97.0\u003c/p\u003e \u003cp\u003e(91.1\u0026ndash;102.8)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNegative Predictive Value\u003c/p\u003e \u003cp\u003e(95% CI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e86.7\u003c/p\u003e \u003cp\u003e(69.4\u0026ndash;103.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e65.4\u003c/p\u003e \u003cp\u003e(47.0\u0026ndash;83.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e50.0\u003c/p\u003e \u003cp\u003e(34.5\u0026ndash;65.4)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAccuracy\u003c/p\u003e \u003cp\u003e(95% CI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e86.3\u003c/p\u003e \u003cp\u003e(78.4\u0026ndash;94.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e82.1\u003c/p\u003e \u003cp\u003e(73.3\u0026ndash;90.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e71.2\u003c/p\u003e \u003cp\u003e(60.8\u0026ndash;81.5)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"4\"\u003e95% CI\u0026thinsp;=\u0026thinsp;95% Confidence interval\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eWhen we assessed individual parameters in the SBASS, GGT\u0026thinsp;\u0026gt;\u0026thinsp;200U/L had the highest sensitivity of 94.2% and specificity of 66.7% (Table\u0026nbsp;\u003cspan refid=\"Tab4\" class=\"InternalRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab4\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 4\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSensitivity, specificity, positive predictive value and negative predictive value of individual parameters in the Simple Biliary Atresia Scoring System (SBASS) for diagnosing biliary atresia.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGall bladder length\u0026thinsp;\u0026lt;\u0026thinsp;1.6cm\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eTriangular cord sign present\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDirect Bilirubin:Total bilirubin ratio\u0026thinsp;\u0026gt;\u0026thinsp;0.7\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eGamma-glutamyl transferase\u0026thinsp;\u0026gt;\u0026thinsp;200U/L\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSensitivity (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e63.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e34.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e84.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e94.2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSpecificity (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e76.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e95.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e23.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e66.7\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePositive Predictive Value (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e86.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e94.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e73.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e87.5\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNegative Predictive Value (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e45.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e37.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e38.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e82.4\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThe mean GGT in the BA group was significantly higher than in the non-BA group, with respective values of 580U/L and 174U/L (P\u0026thinsp;\u0026lt;\u0026thinsp;0.001) (Table\u0026nbsp;\u003cspan refid=\"Tab5\" class=\"InternalRef\"\u003e5\u003c/span\u003e). In addition, the length of gall bladder was significantly smaller in BA compared to non-BA with a mean of 1.0cm and 1.9cm respectively (P\u0026thinsp;\u0026lt;\u0026thinsp;0.004).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab5\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 5\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003e\u003cb\u003eMean and standard deviation of individual parameters in the Simple Biliary Atresia Scoring System (SBASS) comparing biliary atresia and non-biliary atresia groups\u003c/b\u003e.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e \u003cp\u003ePARAMETERS\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eBiliary atresia\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eNon- biliary atresia\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eP-Value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eGall bladder length on ultrasound (cm)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.08\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1.87\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e\u003cb\u003e0.004\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSD\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.02\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1.04\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMin -Max\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u0026ndash;4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0\u0026ndash;4\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eTotal bilirubin (umol/L)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e163\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e170\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e0.690\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSD\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e43\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e71\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMin- Max\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e103\u0026ndash;289\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e62\u0026ndash;371\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eDirect bilirubin:Total bilirubin ratio\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0.76\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.73\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e0.297\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSD\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0.09\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.08\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMin - Max\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0.50\u0026ndash;1.00\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.62\u0026ndash;3.71\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003eGamma-glutamyl transferase (U/L)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMean\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e580\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e174\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e\u003cb\u003e\u0026lt;\u0026thinsp;0.001\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSD\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e346\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e111\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMin - Max\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e126\u0026ndash;1680\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e30\u0026ndash;459\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003eSD: Standard deviation\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eIn our study, we evaluated the performance of the simple biliary atresia scoring system (SBASS), to differentiate between BA and other causes of neonatal cholestatic disorders. We found that this scoring system has a high sensitivity of 96.2%, specificity of 61.9%, positive predictive value of 86.2%, negative predictive value of 86.7% and accuracy of 86.3% using a cut-off of 3 and above, in diagnosis of BA. The AUC of this scoring system was 0.901 (95% CI,0.823\u0026ndash;0.978), indicating good discrimination of BA.\u003c/p\u003e \u003cp\u003eOur study showed that GGT\u0026thinsp;\u0026gt;\u0026thinsp;200U/L has a high sensitivity (94.2%) and a high positive predictive value (87.5%). Several studies have also reported higher GGT levels in BA compared to other cholestatic disorders, with a GGT level\u0026thinsp;\u0026gt;\u0026thinsp;300 U/L having 85% accuracy in diagnosing BA [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Additionally, our study showed that, the ratio of direct hyperbilirubinemia to total bilirubin\u0026thinsp;\u0026gt;\u0026thinsp;70% has sensitivity of 84.6%, specificity of 23% and positive predictive value of 73%. This is in line with a study reported by Harpavat et al. that found elevated serum direct conjugated bilirubin in 34 BA patients within the first 96 hours of age[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. In 2016, Hapavat et al. initiated a prospective study using two-step screening with direct bilirubin measurement at 60 hours of life and at 2 weeks of age; the sensitivity, specificity, and positive predictive value were 100%, 99.9%, and 18.2% respectively [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. They also postulated that a direct conjugated bilirubin to total bilirubin ratio\u0026thinsp;\u0026lt;\u0026thinsp;15% has a specificity of 99.3%.\u003c/p\u003e \u003cp\u003eNumerous ultrasonographic features have been described as useful pointers to the diagnosis of biliary atresia. In our study, we found that the triangular cord sign is an important ultrasonographic feature in diagnosing BA, which is consistent with findings from other studies. In a meta-analysis of 17 studies from 1996 to 2015, Yoon et al. reported a sensitivity of 85% and specificity of 97% for the diagnosis of BA using the triangular cord sign [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. Additionally, in our study, a gall bladder length\u0026thinsp;\u0026lt;\u0026thinsp;1.6cm showed sensitivity of 63.5% and specificity of 76.2% for diagnosing BA. Yoon et al reported that the most common cut off sizes for gall bladder length (\u0026lt;\u0026thinsp;1.5cm or \u0026lt;\u0026thinsp;1.9cm) in 8 studies had a sensitivity range of 50%-100% and specificity range of 68%-99% [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. Humphrey and Stinger reported that the features with the greatest individual sensitivity and specificity respectively in diagnosing BA were triangular cord sign (73% and 100%), abnormal gall bladder wall (91% and 95%), abnormal gall bladder shape (70% and 100%), and absence of the common bile duct (93% and 92%) [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Nevertheless, despite the extensive studies on radiological features indicating BA, there is no single feature with sufficient sensitivity and specificity to reliably differentiate BA from other causes of neonatal cholestasis [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAn effective bedside diagnostic score for biliary atresia (BA) in cholestatic infants is still lacking, and none of the few proposed in the past has achieved widespread application in clinical practice [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. El Guindi and colleagues used a twelve-point diagnostic score which consists of clinical, ultrasound findings, laboratory and histopathological results [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. However, the scoring system is not reproducible as reported by Sciveres, and taking a liver biopsy in all children with direct hyperbilirubinemia is not warranted [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. In comparison to other scoring systems, the merits of using the SBASS are that it consists of a few variables that are readily obtained in any initial workup of an infant with cholestatic jaundice [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]. To our knowledge, the SBASS is the simplest scoring system available with high sensitivity and accuracy.\u003c/p\u003e \u003cp\u003eWhen an SBASS score of \u0026lt;\u0026thinsp;3 is obtained, other diagnostic tests can be used prior to deciding on surgery, such as PTC and liver biopsy. In our study, PTC was used to exclude biliary atresia in 6 (28%) of the cases, and this approach is similar to others such as Jensen et al who reported a sensitivity of 100% and specificity of 86% of PTC in diagnosing BA [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. This workflow helps to relieve strain on operating theatre resources and also reduces the risks associated with surgical procedures under general anaesthesia in small infants [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. However, strict selection criteria must be used to avoid failure of this procedure, including ultrasound identification of gallbladder size.\u003c/p\u003e \u003cp\u003eEven in institutions where there is a low threshold for surgical exploration for cholestatic jaundice in infants, a low SBASS score (\u0026lt;\u0026thinsp;3) can still be helpful in guiding preoperative preparations. For example, the anaesthetic team may opt not to insert central venous and intra-arterial lines at the beginning of the procedure, anticipating a low likelihood of BA, thus avoiding the patient safety risks associated with these invasive cannulations. We have proposed an algorithm, as shown in Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e2\u003c/span\u003e, based on the findings from this study.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eHIDA (hepatobiliary iminodiacetic acid), PTC (percutaneous cholangiogram), EHBT (extrahepatic biliary tree), GB (gall bladder), BA (biliary atresia), KPE (Kasai portoenterostomy).\u003c/p\u003e \u003cp\u003eFigure \u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e2\u003c/span\u003e: \u003cb\u003eProposed algorithm for workup to differentiate between biliary atresia and other causes of neonatal cholestatic jaundice.\u003c/b\u003e\u003c/p\u003e \u003cp\u003eSeveral limitations of the present study should be acknowledged. Firstly, ultrasound is an operator-dependent procedure, and non-standardised ultrasound protocols across multiple institutions and referral sources can impact reports and interpretations. Hence, establishing a standardized ultrasound protocol for investigation of cholestatic jaundice in infants is required. Secondly, as BA is a progressive illness, biochemical and anatomical morphology can evolve along the course of the disease. It is possible that very early patient referrals at lower gestational age may result in falsely low scores even in the presence of BA and vice versa. Our study was not designed to account for this factor and longer-follow up for these cohorts might be required. We are also mindful of the presence of referral bias, particularly in patients referred by a paediatric gastroenterology team who are likely to have worked up and excluded a number of patients, eventually forwarding only those more likely to have BA. To mitigate this bias in the future, we can apply our scoring method in the primary health care setting to assess whether it yields comparable outcomes.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eOur study demonstrates that the SBASS provides a bedside, non-invasive scoring system for differentiating BA from other causes of cholestatic jaundice in infants. By utilizing this scoring system, it allows for the exclusion of BA using a low-risk method, thereby reducing the likelihood of negative surgical explorations.\u003c/p\u003e"},{"header":"Declarations","content":"\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eAuthors' contributions: NM, LWC, YC \u0026amp; SAN conceptualized the study. NM \u0026amp; NHAR collected the data. NM, LWC, YC, HHKS, MYA \u0026amp; SAN performed the data analysis. The first draft of the manuscript was written by NM. LWC, YC, AS, SS \u0026amp; SAN provided critical review of the manuscript. All authors read and approved the final manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgement\u003c/h2\u003e\u003cp\u003eWe would also like to express our gratitude to Dr Mohd Fauzi Sharudin, Head Unit and Senior Consultant Paediatric Surgery Hospital Sultanah Aminah, and Dr Muhammad Firdaus Syukri from University of Malaya Medical Centre, Kuala Lumpur for contributing and sharing their pearl of wisdom in this research.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eLee WS, Chai PF (2010) Clinical features differentiating biliary atresia from other causes of neonatal cholestasis. Ann Acad Med Singap 39(8):648\u0026ndash;654\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eEl-Guindi MA, Sira MM, Sira AM et al (2014) Design and validation of a diagnostic score for biliary atresia. 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Pediatr Radiol 42(1):32\u0026ndash;39. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s00247-011-2202-4\u003c/span\u003e\u003cspan address=\"10.1007/s00247-011-2202-4\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003eEpub 2011 Jul 24\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAshritha A, Gautam V, Lal BB et al (2022) Percutaneous Cholecystocholangiography-A Tool to Conclusively Exclude Biliary Atresia. Indian J Pediatr 89(11):1144\u0026ndash;1147. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s12098-022-04354-2\u003c/span\u003e\u003cspan address=\"10.1007/s12098-022-04354-2\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003eEpub 2022 Sep 20\u003c/span\u003e\u003c/li\u003e \u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Biliary atresia, scoring, cholestatic jaundice, predictive value","lastPublishedDoi":"10.21203/rs.3.rs-4741373/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4741373/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003ePurpose:\u003c/h2\u003e \u003cp\u003eEarly diagnosis of biliary atresia (BA) is critical for best outcomes, but is challenged by overlapping clinical manifestations with other causes of obstructive jaundice in neonates and infants. We evaluate the performance of the Simple BA Scoring System (SBASS) in diagnosing BA.\u003c/p\u003e\u003ch2\u003eMethods:\u003c/h2\u003e \u003cp\u003e With ethical approval, we performed a prospective, cross-sectional study on all infants with cholestatic jaundice referred to three paediatric surgical tertiary centres from June 2021-December 2022. Diagnosis of BA was confirmed by cholangiography and histological findings. We excluded patients who had excretion of hepatic iminodiacetic acid, and those without all SBASS score parameters available. SBASS scoring was applied to all patients and scores were compared to the eventual diagnosis. The score consists of two ultrasonographic findings (gall bladder length\u0026thinsp;\u0026lt;\u0026thinsp;1.6cm score 1, presence of triangular cord sign score 1), and two laboratory variables (conjugated bilirubin:total bilirubin ratio\u0026thinsp;\u0026gt;\u0026thinsp;0.7 score 2, gamma-glutamyl transferase(GGT)\u0026thinsp;\u0026gt;\u0026thinsp;200 U/L score 2). The maximum score was 6.\u003c/p\u003e\u003ch2\u003eResults:\u003c/h2\u003e \u003cp\u003eOf 97 patients referred, 73 were included in this study: 46 (63%) male, 64(87%) term babies. Fifty-two (71%) had BA (age 57 days [range 45\u0026ndash;68]) while 21(29%) were non-BA (age 59 days, [range 54\u0026ndash;68]). In the non-BA group, 6 (28%) had percutaneous cholangiography (PTC) while 15(72%) had intraoperative cholangiogram (IOC). At a cut-off of 3, the scoring system showed a sensitivity of 96.2%, specificity of 61.9% positive predictive value of 86.2%, negative predictive value of 86.7%, and overall accuracy of 86.3%. GGT had the highest sensitivity (94.2%) of the four clinical parameters, while triangular cord sign was the most specific (95.2%) in diagnosing BA.\u003c/p\u003e\u003ch2\u003eConclusion:\u003c/h2\u003e \u003cp\u003eOur study shows that the SBASS provides a bedside, non-invasive scoring system to differentiate BA from other cholestatic jaundice causes in infants. Utilizing this scoring system allows for the exclusion of BA using a low risk method and reduces the likelihood of negative surgical explorations.\u003c/p\u003e","manuscriptTitle":"A Prospective Multicentre Study Evaluating the Performance of the Simple Biliary Atresia Scoring System in Predicting Biliary Atresia","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-08-09 12:47:10","doi":"10.21203/rs.3.rs-4741373/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-07-31T16:01:18+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-07-25T21:20:37+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"233184211345270713944379144326789296745","date":"2024-07-17T02:21:32+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-07-16T11:41:04+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-07-16T08:46:44+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-07-16T07:45:20+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Surgery International","date":"2024-07-15T07:37:22+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"bac6149b-5c8d-4fed-b019-620031942c1e","owner":[],"postedDate":"August 9th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2024-09-02T16:07:14+00:00","versionOfRecord":{"articleIdentity":"rs-4741373","link":"https://doi.org/10.1007/s00383-024-05830-w","journal":{"identity":"pediatric-surgery-international","isVorOnly":false,"title":"Pediatric Surgery International"},"publishedOn":"2024-08-27 15:57:30","publishedOnDateReadable":"August 27th, 2024"},"versionCreatedAt":"2024-08-09 12:47:10","video":"","vorDoi":"10.1007/s00383-024-05830-w","vorDoiUrl":"https://doi.org/10.1007/s00383-024-05830-w","workflowStages":[]},"version":"v1","identity":"rs-4741373","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4741373","identity":"rs-4741373","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}