Reply to: “A scoring system for biliary atresia: Is this the right one?”
Authors: Mohamed Abdel-Salam El-Guindi, Mostafa Mohamed Sira, Ahmad Mohamed Sira, Tahany Abdel-Hameed Salem
Journal: Journal of Hepatology (2014)

A scoring system for biliary atresia: Is this the right one?Journal of HepatologyVol. 62Issue 4PreviewWe read with interest the recent article by El-Guindi et al. [1]. 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 Sciveres and colleagues 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] and we would like to respond to their letter and help them answer the question they raised. Looking thoroughly into their retrospective study, many limitations can be easily pointed out and may account for their disappointment and poor results. First, the retrospective nature of the study was not the optimal approach for the validation of the biliary atresia (BA) score. Second, the small number of patients (n = 27) in their study carries a very low statistical power of 28.2% at an alpha of 0.05, 2-tailed with 95% CI. This intensifies the need for validation of the BA score on a sample size larger than the one in the original article (n = 75) by El-Guindi et al., as we already suggested. Third, the historical data they collected were not pre-designed to evaluate the score parameters beforehand. For example, the gallbladder length before and after feeding was assessed using two timely-spaced ultrasound reports, which may carry a high fallacy related to different operators with different judgments. Another critical point is the evaluation of hepatic subcapsular flow (HSF), which has the highest value in the BA score (6.735). Sciveres et al. could detect HSF in only one patient among their 27 patients. In our earlier report [[2]El-Guindi M.A. Sira M.M. Konsowa H.A. El-Abd O.L. Salem T.A. Value of hepatic subcapsular flow by color Doppler ultrasonography in the diagnosis of biliary atresia.J Gastroenterol Hepatol. 2013; 28: 867-872Crossref PubMed Scopus (58) Google Scholar], we detected HSF in 96.3% (26/27) of cases in the BA group and in one patient in the non-BA group. In addition, Lee et al. [[3]Lee M.S. Kim M.J. Lee M.J. Yoon C.S. Han S.J. Oh J.T. et al.Biliary atresia: color doppler US findings in neonates and infants.Radiology. 2009; 252: 282-289Crossref PubMed Scopus (83) Google Scholar] detected HSF in 100% (29/29) of cases in the BA group and in 14% (5/35) of the non-BA group. This raises a doubt about HSF results by Sciveres and colleagues. Apart from the minimum level of experience needed for such technique, as we elaborated in our article, this raises the question of whether the Doppler machine parameters were set as reported by Lee et al. [[3]Lee M.S. Kim M.J. Lee M.J. Yoon C.S. Han S.J. Oh J.T. et al.Biliary atresia: color doppler US findings in neonates and infants.Radiology. 2009; 252: 282-289Crossref PubMed Scopus (83) Google Scholar] or El-Guindi et al. [[2]El-Guindi M.A. Sira M.M. Konsowa H.A. El-Abd O.L. Salem T.A. Value of hepatic subcapsular flow by color Doppler ultrasonography in the diagnosis of biliary atresia.J Gastroenterol Hepatol. 2013; 28: 867-872Crossref PubMed Scopus (58) Google Scholar] for the detection of HSF. This is not clear in their study. A further point of concern, although the radiology records were reviewed by a single radiologist, this does not obviate the fact that the procedures were performed by different operators and that the pre-set parameters might have been different or the machine itself used in evaluating the patients over this long period might have varied from one patient to another. All of these may be a source of biased results and interpretation. For that, we advice Sciveres and his colleagues to validate the BA score in a prospective well-designed cohort with pre-set parameters, as reported in the original report [[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]. Furthermore, the use of an appropriate sample size with acceptable statistical power is essential. Sciveres et al. suggested that children with a various combination of normal-colored stool, mild cholestasis, known risk factors for transient cholestasis, normal gamma glutamyl transpeptidase activity, and other features not suggestive of BA might be, in most cases, effectively managed without the need of liver histology. Here, a question enforces itself. Why would patients with such criteria of known risk factors and other features not suggestive of BA be included in a study like ours in the first place? Sciveres and colleagues debated the indication for liver biopsy in the patients in our study. They mentioned that liver biopsy was proposed to all children with "direct hyperbilirubinemia" in our article. This statement can be found nowhere in our article. Therefore, the allegation is not a true one. Herein, we invite Sciveres and his colleagues to review our article with special focus on the different diagnoses of patients in the non-BA group (in all of whom liver biopsy was strongly indicated and justified). It is well understood that liver biopsy is not indicted for all cases of neonatal cholestasis, as some etiologies can be managed without its need. However, the high rate of neonatal cholestasis patients (200–300 case/year) in our center, including BA patients (25–35 case/year) [[4]Sira M.M. Taha M. Sira A.M. Common misdiagnoses of biliary atresia.Eur J Gastroenterol Hepatol. 2014; 26: 1300-1305Crossref PubMed Scopus (17) Google Scholar], makes the number of candidate cases for liver biopsy relatively high, when compared with other centers with lower rates [[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]. In this concern, we need to emphasize, again, that liver biopsy is considered as an integral part of the diagnostic workup of neonatal cholestasis patients and is strongly encouraged according to "The Cholestasis Guideline Committee of NASPGN" [[5]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], unless there is a contraindication or when the parents refuse the procedure. Furthermore, liver biopsy can be performed safely, even in the smallest infants, with sedation and local anesthesia [[6]Rastogi A. Krishnani N. Yachha S.K. Khanna V. Poddar U. Lal R. Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries.J Gastroenterol Hepatol. 2009; 24: 97-102Crossref PubMed Scopus (81) Google Scholar]. The aim of liver biopsy in neonatal cholestasis is, not only to evaluate the features of biliary outflow obstruction, but also essential to reveal the etiology of the liver disease, and assess fibrosis stage, which affects the treatment policy. The authors declared that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.