Pediatric emergency medicine trisk 2074 2074
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weeks of age. Persistent jaundice beyond 2 weeks of age should be evaluated
for possible BA with a measurement of serum conjugated (direct) bilirubin.
Triage Considerations. In most patients, there are no specific triage
considerations for newborns with BA, as they are typically well appearing. If
unrecognized, patients will have persistent jaundice and over time, develop
liver failure and its related complications.
Clinical Assessment. Patients are typically well appearing and present with
persistent jaundice. Acholic stools, dark brown urine, and failure to thrive may
also be present, but are generally late findings and often present outside of the
goal age range for early identification. On examination, there may not be any
physical findings other than skin discoloration and icterus, yet splenomegaly
may be present if a patient has developed portal hypertension. Dysmorphic
features may be identified that likely suggest another disorder, such as
Alagille syndrome (AS) (see section below).
Initial laboratory evaluation of any patient with persistent jaundice should
include a serum conjugated bilirubin level. If the newborn has a conjugated
(direct) hyperbilirubinemia, BA should be suspected and the infant should be
referred immediately to an appropriate center. Other laboratory abnormalities
may demonstrate a cholestatic disease process with an elevation in liver
function enzymes (AST, ALT), serum GGT, and alkaline phosphatase.
Synthetic liver function is generally normal early in the course of the disease,
and most patients are not hypoalbuminemic or coagulopathic.
Ultrasound is an important initial step in evaluation of a patient with
possible BA. Findings may include hepatomegaly, an absent or atretic
gallbladder, or the “triangular cord sign” which is a hyperechoic area that
results from a fibrous hepatic duct. This finding is operator dependent and has
reported sensitivities of 49% to 80%, but if found, may be 98% specific. Bile
duct dilation is never present. If inconclusive, additional diagnostic testing
may be performed in consultation with Gastroenterology and Pediatric
Surgery to differentiate BA from other cholestatic disease processes.
In patients who have already been diagnosed and undergone surgical repair,
the most common complication is cholangitis, occurring in up to 55% of
patients in the first 2 years after surgery. Many patients also develop portal
hypertension (see section on portal hypertension), hepatic malignancy, or
hepatopulmonary syndrome which results in increased pulmonary vascular
