[size=6]A 50-day-old infant has conjugated hyperbilirubinemia, mild transaminitis, and acholic stool. Ultrasonography demonstrated an absent gallbladder. A blood sample was sent out for MMP-7 yesterday and results are pending. What is the next best step in management?

 

a Await MMP-7 results

 

b Operative cholangiogram

 

c Liver transplant

 

d Duodenal intubation[/size]

B

[size=6]A 50-day-old infant has conjugated hyperbilirubinemia, mild transaminitis, and acholic stool. Ultrasonography demonstrated an absent gallbladder. A blood sample was sent out for MMP-7 yesterday and results are pending. What is the next best step in management?

 

a Await MMP-7 results

 

b Operative cholangiogram

 

c Liver transplant

 

d Duodenal intubation[/size]

A

B

B

A

[size=6]A 50-day-old infant has conjugated hyperbilirubinemia, mild transaminitis, and acholic stool. Ultrasonography demonstrated an absent gallbladder. A blood sample was sent out for MMP-7 yesterday and results are pending. What is the next best step in management?

 

a Await MMP-7 results

 

b Operative cholangiogram

 

c Liver transplant

 

d Duodenal intubation[/size]

B

B

correct answer

b Operative cholangiogram

 

This 50-day old infant has persistent conjugated hyperbilirubinemia with acholic stool. The ultrasound suggests biliary atresia as the gallbladder is absent. At this age, further delay waiting for the results of the MMP-7 testing would potentially compromise bile clearance after portoenterostomy, therefore it would be prudent to proceed with an operative cholangiogram. MMP-7 remains experimental, but its most appropriate current application is in younger babies with conjugated hyperbilirubinemia in whom there is not a high suspicion for biliary atresia—for example, green stool or a visible gallbladder on ultrasound-as it obviates more invasive evaluations and can facilitate ruling out biliary atresia. Duodenal intubation would be unlikely to clarify the diagnosis and liver transplantation would be premature at this point.