Reply to: “A diagnostic score for biliary atresia”
Authors: Mohamed Abdel-Salam El-Guindi, Mostafa Mohamed Sira, Ahmad Mohamed Sira, Tahany Abdel-Hameed Salem
Journal: Journal of Hepatology (2014)

A diagnostic score for biliary atresiaJournal of HepatologyVol. 61Issue 6PreviewWe read with great interest the paper by El-Guindi et al. [1], evaluating a new diagnostic score for biliary atresia (BA). BA is a destructive inflammatory obliterative cholangiopathy of unknown origin that affects intra- and extrahepatic bile ducts. Success of surgical correction, in form of a Kasai portoenterostomy amongst other factors, depends on early diagnosis and timely intervention. Presently, there are no non-invasive diagnostic methods that can clearly identify infants with BA from other causes of neonatal cholestasis (NC). 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 Pfister et al. 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 (85) Google Scholar] and would like to comment on the issues raised in their letter. Although the concerns that were raised by Pfister et al. are interesting, none is related to the applicability of the BA score as the recruitment in our study was consecutive for all cases with neonatal cholestasis except for those with contraindications to liver biopsy. For that reason, we did not go through such recruitment details in our article. None of the patients was preterm and diagnoses were as described in the article. Causes like alpha-1 antitrypsin, cystic fibrosis and hypopituitarism were not proven in our study population. In the validation group, diagnoses like galactosemia, tyrosinemia, cytomegalovirus induced hepatitis, biliary paucity and progressive familial intrahepatic cholestasis (PFIC) were confirmed by completing the workup after being allocated earlier by the score as non-BA. The PFIC patients, diagnosed in our study, were of type 3 with high gammaglutamyl transpeptidase (GGT) levels and negative hepatic immunostaining for multidrug resistance protein 3 and none were type PFIC1 or 2. In accordance with our recent reports [2El-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 (54) Google Scholar, 3Ghoneim E.M. Sira M.M. Abd Elaziz A.M. Khalil F.O. Sultan M.M. Mahmoud A.B. Diagnostic value of hepatic intercellular adhesion molecule-1 expression in Egyptian infants with biliary atresia and other forms of neonatal cholestasis.Hepatol Res. 2011; 41: 763-775Crossref PubMed Scopus (15) Google Scholar, 4Sira M.M. El-Guindi M.A. Saber M.A. Ehsan N.A. Rizk M.S. Differential hepatic expression of CD56 can discriminate biliary atresia from other neonatal cholestatic disorders.Eur J Gastroenterol Hepatol. 2012; 24: 1227-1233Crossref PubMed Scopus (15) Google Scholar], earlier studies [5Liu C.S. Chin T.W. Wei C.F. Value of gamma-glutamyl transpeptidase for early diagnosis of biliary atresia.Zhonghua Yi Xue Za Zhi. 1998; 61: 716-720PubMed Google Scholar, 6Sinatra F.R. The role of gamma-glutamyl transpeptidase in the preoperative diagnosis of biliary atresia.J Pediatr Gastroenterol Nutr. 1985; 4: 167-168Crossref PubMed Scopus (5) Google Scholar, 7Vajaradul C. Vanprapar N. Chuenmeechow T. Ongajyooth S. Use of serum gamma glutamyl transpeptidase to differentiate between extrahepatic biliary atresia and neonatal hepatitis.J Med Assoc Thai. 1989; 72: 395-399PubMed Google Scholar, 8Wright K. Christie D.L. Use of gamma-glutamyl transpeptidase in the diagnosis of biliary atresia.Am J Dis Child. 1981; 135: 134-136PubMed Google Scholar] also reported that GGT levels differ significantly and can be used in discriminating BA from non-BA cases. So, we agree with Pfister et al. that such difference should not be surprising. The authors declare that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.