Billiary atresia cases among attendants of Menoufiya National Liver Institute: [a retrospective study]

ABSTRACT

This study included 126 cases of neonatal cholestasis syndrome [NCS] from the attendants of Pediatric Department of the National Liver Institute, Menoufiya University from 1994 up to 2004 . They were divided into two groups First group[1stGr.] included 58 biliary atresia [BA] cases [46%] and the second group [2ndGr.] included 68 [54%] were due to other causes of NCS . The etiology of NCS due to causes other than BA were as follows 28 cases [22.22%] neonatal hepatitis, 11 cases [8.73%] septicemia, 8 cases [6.35%] paucity of intrahepatic bile ducts, 5 cases [3.97%] inspessated bile syndrome, 4 cases [3.17%] choledochal cyst, 2 cases [1.59%] Byler's disease, 2 cases [1.59%] galactosemia, 2 cases ? 1 antitrypsin deficiency [1.59%], 1 case [0.79%] Alagille syndrome, and 5 cases [3.97%] due to unknown causes. Onset of jaundice whether early or late does not differentiate cholestatic cases due to BA from other causes of NCS.Clay coloured [acholic] stools were more frequently detected in BA cases [77.6%] than 2ndGr. [27.9%] [p<0.05]. Triangular cord sign [Tc sign] and absence of and/or gall bladder abnormality were detected in significantly higher proportion of BA group more than the 2ndGr.[p<0.05]. By histopathological examination portal tract fibrosis, bile duct proliferation, bile plugs in portal ductules and preservation of hepatic lobular architecture were detected more frequently among BA cases than the other group, while interface hepatitis and giant cell detection were observed more frequently among 2ndGr. than BA cases [p < 0.05]. The mean of alkaline phosphatase and gamma glutamyl transpeptidase was found to be statistically higher among BA group than that of the 2ndGr. [p<0.05] . Kasai operation was done for only 20 cases of BA cases [34.5%] and the mean of age of patients at time of operation was 75 days +/- 17.8. Complications encountered in BA cases were recurrent cholangitis, ascites, itching, coagulopathy, hematemesis and end-stage liver failure in 48%, 50%, 41.7%, 39.6, 18.8% and 54.2% of cases respectively. In conclution, the results of the present study indicate that clinical evaluation by an experienced pediatric hepatologist and liver biopsy together with careful ultrasonographic evaluation are considered as the most reliable methods for early differentiation of BA from other causes of neonatal cholestasis. Management of BA cases will be improved by public and professional education to encourage early referral of infants with neonatal cholestasis [>14 days] to specilised liver centers for early diagnosis to facilitate initial surgery before 8 weeks of age