During my fellowship, one of the faculty presented an abstract indicating that 4 out of 40 preterm infants with cholestasis had significant underlying liver disease in addition to parenteral nutrition associated cholestasis (PNAC). One of these patients had biliary atresia. The obvious point was not to assume that the cholestasis was due to the usual suspects found with premature infants.
A recent study indicates that biliary atresia (BA) in Taiwan is more common in preterm infants than in term infants (J Pediatr 2013; 163: 100-3). The authors identified 197 cases (166 term infants) of BA between 2004-2010. This retrospective study used a nationwide screening for BA (the national stool card registry center database) along with reports from surgeons of the Taiwan Biliary Atresia Study Group.
- Annual incidence of BA per 10,000 live births was 1.43 and 2.37 for term and preterm infants respectively.
- Kasai operation before 60 days occurred in 68.7% of term and 44.4% of preterm infants. Mean age of Kasai was 52.9 days for term infants and 71.8 for preterm infants.
- Major congenital anomalies along with BA were more common among preterm (18.5%) than term (4.1%).
- Mean onset of clay-colored stools among preterm infants was 33.6 days compared with 29.6 for term infants.
- Stool cards had good sensitivity in detecting BA in both preterm and term infants: 96.3% and 92.8% respectively.
- Jaundice-free at 3 months following Kasai was 62% of term infants and 37% of preterm infants.
- 18-month survival with native liver was 72.7% in term infants and 50% in preterm infants.
While the authors point out several studies that have shown prematurity is an independent risk factor associated with BA, nevertheless this idea is counter to conventional wisdom that BA patients are typically well-appearing term infants at the time of diagnosis. The authors also note that despite delayed diagnosis in preterm infants, this was not correlated with an impact on jaundice-free status 3 months following surgery. This finding, in particular, should be cautiously interpreted as there were only 27 infants in the preterm group.
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