Category Archives: biliary atresia

Diagnosing biliary atresia earlier

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Biliary atresia (BA) is often suspected among infants with prolonged jaundice. In fact, efforts have been underway for a long time to encourage fractionation of the bilirubin values to look for conjugated hyperbilirubinemia, especially in infants that remain jaundiced at three weeks of life.  While this is still good advice, given the lack of success in implementation, there is good evidence that obtaining a fractionated bilirubin at any time point can help identify cholestasis associated with BA.

A recent article by Karpen et al (Pediatrics 2011; 128:. e1428 -e1433) indicates that direct bilirubin values are elevated beginning within the first one to two days in patients with BA.  In their cohort of 61 BA subjects, 56% had newborn fractionated bilirubin values.  Every BA patient had elevated direct bilirubin, on average 1.4 ± 0.43 mg/dL (normal <0.5) (compared with control patients:  0.19 ± 0.075 mg/dL, P < .0001).  Also, another important finding was that early on the ratio of direct bilirubin to total bilirubin was normal in 79%; normally this ratios is ≤0.2.  As such, all patients with increased direct bilirubin need to be followed closely.

Related blog entries:

Outcomes of Biliary Atresia

MicroRNAs and biliary atresia

Bleeding due to vitamin K deficiency

Bleeding due to vitamin K deficiency

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With cholestasis in infancy, Bill Balistreri taught me that there were four potential emergencies:

  • Bleeding due to vitamin K deficiency or coagulopathy
  • Hypoglycemia
  • Sepsis
  • Metabolic poisoning with formula (in patients with galactosemia)

Once these issues have been considered, then it is appropriate to start investigating the etiology of the cholestasis.

One of the more dramatic complications is intracranial hemorrhage (ICH). While ICH is a well-recognized complication of cholestasis in infancy, the long-term outcomes are not well-characterized. A report from Japan adds some insight (JPGN 2012; 54: 552-57).

Among a retrospective review of 83 infants with biliary atresia (BA) between 1979 to 2009, ICH occurred in 8% despite oral vitamin K prophylaxis (2 mg).  The onset of ICH was between 47-76 days after birth and was prior to surgery.  Coagulopathy was noted in all cases, which improved with vitamin K intravenously.  Two infants required craniotomy.  In 5 of 7 cases, neurologic sequelae were noted including developmental delay in three, epilepsy in one, and mild hemiparesis in two.

Additional references:

  • Blood Rev 2009; 23: 49-59.  Review of vitamin K deficiency.
  • Pediatrics 2008; 121:e857.  Vitamin K deficiency common in cholestatic breastfed babies.  Can be prevented with 1mg po each week or single IM dose of 2mg.
  • Eur J Pediatr Surg 2005; 15: 295-9.  Bleeding disorder as 1st symptom of BA.
  • Pediatr Neurosurg 2006; 42: 362-7.  ICH due to vitamin K deficiency.
  • Pediatrics 2006; 118: e1657.  dose of 0.2mg effective for median of 25days (w/o toxicity/accumulation of K1O) in infants <32weeks gestation.

The heart connection

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Cardiac changes with biliary atresia (BA) are surprisingly common– Gastoenterology 2011; 141: 1264-72.  In this study, 48 patients with BA, listed for liver transplantation, underwent echocardiography between 2004-2010.  The median age was 8 months.  Significant increases in left ventricle wall thickness (23% increase) and mass (51% increase) were noted; in addition, functional changes were noted as there was an increase in LV shortening fraction (8% increase).  Features of ‘cirrhotic cardiomyopathy’ were evident in 72% of infants (29/40).  The authors conclude that these heart changes likely contribute to prolongation of posttransplant hospitalization.

Additional References:

  • -J Am Coll Cardiol 2010; 56: 539-49
  • -J Hepatol 2010; 53: 179-90.