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PubMed · 7901547

Screening for biliary atresia.

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C M Arkley. 1993-11-13. Screening for biliary atresia.. https://pubmed.ncbi.nlm.nih.gov/7901547/

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Advanced biliary atresia: is portoenterostomy justified in all infants?

During 1989-98, of the 127 patients with biliary atresia, 23 were seen with advanced biliary atresia (ABA) presenting with (i) at more than 120 days of age (ii) established cirrhosis and (iii) features of portal hypertension. Sixteen of these underwent exploratory laparotomy, dissection at the porta hepatis and hepatico-portoenterostomy (HPE) (group I). The remaining 7 infants underwent portal dissection and sump drainage only (instead of HPE). The drainage if any was evaluated for any change in colour, volume and concentration in the post-operative period (group II). Age and presentation were the same in both the groups. In group-II, HPE was considered only if the bile flow was noted after portal dissection. Bile flow was seen in 3/16 in group I and 0/7 in group II. The fall in serum bilirubin during the first seven post operative days was noted in 2/16 in group I and 0/7 in group II. No drain output was recorded in any of the group II infants. The incidence of complications and the duration of hospital stay was significantly higher in group I patients. The sump drainage as an alternative procedure to HPE not only served the purpose of evaluating the patients with ABA for the possible bile flow in the post operative period but also avoided the need for a major operative procedure like HPE.

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AIIMS clinical score: a reliable aid to distinguish neonatal hepatitis from extra hepatic biliary atresia.

It is important to distinguish neonatal hepatitis (NH) from extra hepatic biliary atresia (EHBA) in a neonate presented with jaundice as the former is purely medical management and the latter requires surgical procedure at the earliest. The observations on the critical evaluation of the neonatal jaundice cases led us to propose AIIMS clinical score (ACS) and this retrospective study was designed to evaluate the reliability of the ACS in all the 120 babies with jaundice referred to the pediatric surgery department during the past 10 year period. The ACS described here is based on 5 clinical parameters--Age, Jaundice, Colour of urine and stool and Clinical examination of liver. The accuracy of the hepatobiliary scan (HBS) with respect to the diagnosis of EHBA in this series, as compared to per operative cholangiography (POC) which is considered as the gold standard to distinguish these conditions was only 68%. However, ACS showed a sensitivity of 91.5%, a specificity of 76.3%, positive predictive value of 89.2%, negative predictive value of 80.5% and an overall diagnostic accuracy of 86.6%. ACS is reliable to distinguish NH from EHBA at bedside.

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