Reply to: “Scoring system in diagnosing biliary atresia”
Authors: Mohamed Abdel-Salam El-Guindi, Mostafa Mohamed Sira, Ahmad Mohamed Sira, Tahany Abdel-Hameed Salem
Journal: Journal of Hepatology (2014)

Scoring system in diagnosing biliary atresiaJournal of HepatologyVol. 61Issue 6PreviewWe read with great interest the article by El-Guindi et al. [1] who propose a diagnostic scoring system for biliary atresia (BA), including clinical, laboratory, ultrasonographic, and histopathological parameters. They conclude that the scoring system could discriminate BA from other causes of neonatal cholestasis with a sensitivity, specificity and accuracy of 100%, 97.67%, and 98.83%, respectively, in the validation set. Thus, unnecessary intraoperative cholangiography can be avoided in non-BA patients. Full-Text PDF Open AccessDesign and validation of a diagnostic score for biliary atresiaJournal of HepatologyVol. 61Issue 1PreviewThe 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. Full-Text PDF We appreciate the interest of Dong and Zheng in our recent study [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar]. Although hepatobiliary scintigraphy (HBS) is one of the most sensitive methods for the diagnosis of biliary atresia (BA), it has a low specificity (70.4%) as reported in a meta-analysis of 81 studies [[2]Kianifar H.R. Tehranian S. Shojaei P. Adinehpoor Z. Sadeghi R. Kakhki V.R. et al.Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature.Pediatr Radiol. 2013; 43: 905-919Crossref PubMed Scopus (72) Google Scholar]. Sun et al. [[3]Sun S. Chen G. Zheng S. Xiao X. Xu M. Yu H. et al.Analysis of clinical parameters that contribute to the misdiagnosis of biliary atresia.J Pediatr Surg. 2013; 48: 1490-1494Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar] reported a 13.3% (66/498) false positive rate of HBS. They concluded that the excessive dependence on HBS may contribute to the misdiagnosis of BA. Although HBS, showing biliary excretion, excludes BA, non-excretion neither confirms the diagnosis of BA nor rules out the diagnosis of non-BA aetiologies [[4]Yachha S.K. Cholestatic jaundice during infancy.Indian J Gastroenterol. 2005; 24: 47-48PubMed Google Scholar]. Oral phenobarbital can enhance the diagnostic performance of HBS by re-examination one week later [[5]Liu S.X. Huang Z.H. The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice.Front Med China. 2010; 4: 342-345Crossref PubMed Scopus (7) Google Scholar]; however, for the potential delay in diagnosis it is not routinely practiced [[6]Jain A.K. Approach to cholestatic Jaundice in newborn.in: Sachdeva A. Dutta A.K. Jain M.P. Satya P.Y. Goyal R.K. Arora A. Advances in pediatrics. Jaypee brothers Medical publishers (P) Ltd., Daryaganj, New Delhi, India2012: 126-132Google Scholar]. Moreover, the reported BA score specificity (97.67%) [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar] cannot be enhanced by HBS, which has a much lower specificity [[2]Kianifar H.R. Tehranian S. Shojaei P. Adinehpoor Z. Sadeghi R. Kakhki V.R. et al.Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature.Pediatr Radiol. 2013; 43: 905-919Crossref PubMed Scopus (72) Google Scholar]. Liu and Huang [[5]Liu S.X. Huang Z.H. The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice.Front Med China. 2010; 4: 342-345Crossref PubMed Scopus (7) Google Scholar] reported that if no bile was found in the duodenal tube test (DTT), the drainage tube would be kept for up to 72 h. In some cases, the duodenal fluid changed from white to yellow after several days or weeks. Therefore, persistent examination of the duodenal fluid is very important. This also prolongs the time of diagnosis and is not routinely performed. However, DTT may have a special importance in situations where other tests are not available [[6]Jain A.K. Approach to cholestatic Jaundice in newborn.in: Sachdeva A. Dutta A.K. Jain M.P. Satya P.Y. Goyal R.K. Arora A. Advances in pediatrics. Jaypee brothers Medical publishers (P) Ltd., Daryaganj, New Delhi, India2012: 126-132Google Scholar]. An important concept of our BA score [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar] is that it depends on parameters that are an integral part of the workup and are routinely performed for patients suffering neonatal cholestasis (NC) whether suspected to be BA or non-BA. Contrarily, HBS and DTT cannot be considered as such. Dong and Zheng were encouraged by the article of Liu and Huang [[5]Liu S.X. Huang Z.H. The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice.Front Med China. 2010; 4: 342-345Crossref PubMed Scopus (7) Google Scholar] that reports a performance of combined HBS and DTT of 100% sensitivity and 100% specificity. We read the article with great interest but we found two major concerns. First, recruitment of patients was selective as patients with certain aetiologies were excluded from the study. This selectivity may render the calculated specificity inaccurately a representative one. Furthermore, it supports the concept that these procedures are needed for selected patients. Second, the method of calculating the combined performance is not clear. According to their results, 3 groups of patients can be discriminated: (1) Patients having HBS with no excretion and DTT with no bile; (26 patients with BA). (2) Patients having HBS with excretion and DTT with bile; (41 patients with non-BA). (3) Patients having HBS with no excretion and DTT with bile; (17 patients; 3 were BA and 14 were non-BA). These results can never, in any way, lead to a sensitivity and specificity of 100%. It seems that Liu and Huang [[5]Liu S.X. Huang Z.H. The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice.Front Med China. 2010; 4: 342-345Crossref PubMed Scopus (7) Google Scholar] simply combined the sensitivity of one test (HBS) with the specificity of the other (DTT) which is unreasonable. Dong and Zheng, suggested that liver biopsy should be avoided whenever possible. We disagree with this concept as we believe that liver biopsy should be performed unless there is a contraindication or when parents refuse the procedure. For that, liver biopsy is considered as an integral part of the diagnostic workup of NC patients and is strongly encouraged according to “The Cholestasis Guideline Committee” of the NASPGN [[7]Moyer V. Freese D.K. Whitington P.F. Olson A.D. Brewer F. Colletti R.B. et al.Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.J Pediatr Gastroenterol Nutr. 2004; 39: 115-128Crossref PubMed Scopus (317) Google Scholar]. The aim of liver biopsy in NC is not only to evaluate the features of biliary outflow obstruction but is also an essential tool in revealing the aetiology of the liver disease, and in assessing the fibrosis stage which affects treatment policy. Such targets are irreplaceable by HBS and DTT. For that, liver biopsy is generally considered the “gold standard” compared to other diagnostic tools [[8]Yeh M.M. Pathologic diagnosis of biliary atresia on liver biopsy: is tissue the issue?.J Gastroenterol Hepatol. 2009; 24: 936-938Crossref PubMed Scopus (9) Google Scholar]. Recently, Boskovic et al. [[9]Boskovic A. Kitic I. Prokic D. Stankovic I. Grujic B. Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in Serbian infants.Turk J Gastroenterol. 2014; 25: 170-174Crossref PubMed Scopus (13) Google Scholar] reported that liver biopsy was superior to DTT in the diagnosis of BA. As for the safety of liver biopsies, Pietrobattista et al. [[10]Pietrobattista A. Fruwirth R. Natali G. Monti L. Devito R. Nobili V. Is juvenile liver biopsy unsafe? Putting an end to a common misapprehension.Pediatr Radiol. 2009; 39: 959-961Crossref PubMed Scopus (25) Google Scholar] raised the issue asking “Is juvenile liver biopsy unsafe?” They concluded that ultrasound-guided biopsy, performed in a specialized tertiary care paediatric centre by an experienced physician, is considered safe; which is the same in our experience. Although the study population in our study was relatively small, yet, when compared to the studies (discussed in our article) of Chiba and Kasai as well as Gupta et al., evaluating different scores for discriminating BA, our study population was still relatively high [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar] (population size was 123, 120 and 135, respectively). Nonetheless, we suggest that the score needs to be validated in different populations with different investigators. The numbers in Fig. 1C and D [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar] represent the values of the odds ratio (OR) model. For a parameter like hepatic subcapsular flow, which was found in 29/30 of BA and in 1/30 of the non-BA (Table 3), the OR equals 841. The regression coefficient for the same parameter was 6.735 and the relative risk was 29. This clarifies the higher values in the OR model compared to other models. So, we affirm that there is no mistake. The authors declare that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.