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May 16, 2012 6:58 am
With cholestasis in infancy, Bill Balistreri taught me that there were four potential emergencies:
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:
Posted by gutsandgrowth
Categories: biliary atresia, Pediatric Gastroenterology Liver Disease
Tags: biliary atresia, cholestasis, hypoglycemia, intracranial hemorrhage, vitamin k deficiency
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