Implication of early‐onset biliary atresia and extrahepatic congenital anomalies

Background: The aim of the present study was to determine the rate of early‐onset biliary atresia (BA) and its implications, for embryonic‐type BA in Taiwan, a high‐prevalence area for BA. The relationship between the timing of disease onset and congenital extrahepatic anomalies was also identified. Methods: Medical records of 130 infants born in Taiwan with biliary atresia between January 1996 and December 2005 were reviewed retrospectively. The gold standard for the diagnosis of biliary atresia was intraoperative cholangiography. As well as medical records review, abdominal imaging and echocardiograms were performed to determine other structural anomalies. Early‐onset BA was defined as acholic stool and cholestatic jaundice observed before 2 weeks of age. Results: On review of onset of acholic stool and cholestatic jaundice before 2 weeks of age, 31 patients (23.8%) were defined as having early‐onset BA. Twenty patients (15.4%) had major congenital extrahepatic anomalies. One (0.7%) had biliary atresia splenic malformation syndrome (BASM). Both early‐onset and late‐onset BA may be associated with other structural anomalies. Patients with early‐onset BA had a higher probability of having major extrahepatic anomaly (9/31 vs 11/99, P= 0.046). Situs anomalies accompanying major gastrointestinal (GI) tract anomalies occurred only in early‐onset BA patients. Conclusions: After comprehensively investigating the timing of onset and associated congenital extrahepatic anomalies in BA patients in Taiwan, only one BASM with double spleen was detected. A total of 23.8% of patients had early‐onset BA, and this group of patients is prone to extrahepatic anomalies. Situs anomalies accompanying major GI tract anomaly may be indicative of embryonic‐type early‐onset BA.     

Late‐onset bloodstream infections in preterm infants: A 2‐year survey

Background: We determined the prevalence and risk factors for late‐onset bloodstream infections (LO‐BSI), the distribution of pathogens and the outcomes of affected preterm infants. Methods: The records of all preterm infants (<37 weeks gestation) born between 2004 and 2005 and hospitalized in the neonatal intensive care unit for >3 days were retrieved for this retrospective matched case–control study. Results: A total of 108 out of 1459 preterm infants (7.4%) had 142 episodes of LO‐BSI. The highest LO‐BSI rate (44%) was among 198 very‐low‐birthweight infants (<1500 g). The most common causative organisms were Coagulase‐negative staphylococci and Klebsiella (60% and 13%, respectively). The mean hospital stay was 64 days for LO‐BSI preterm infants versus 48 days for non‐LO‐BSI preterm infants. Congenital malformations and peripheral catheters were independent risk factors for LO‐BSI. Crude mortality rates were 6.9% (LO‐BSI) and 3.0% (non‐LO‐BSI), with an LO‐BSI‐attributable mortality of 3.9%. Conclusion: LO‐BSI frequently affect very‐low‐birthweight infants. Strategies to prevent LO‐BSI should target peripheral catheters.