Pediatric emergency medicine trisk 2072 2072
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BILIARY TRACT DISEASE
Biliary Atresia
Goals of Treatment
BA is uncommon but is associated with very high morbidity and mortality,
and is a leading cause of liver transplant in young children. Early detection,
before 30 to 45 days of life, can significantly improve patient outcomes and
potentially avoid transplantation. In the United States, the average age of
diagnosis unfortunately remains at approximately 60 days of life. The goal of
an ED provider is to suspect BA and facilitate its diagnosis and subsequent
definitive surgical care to improve patient outcomes. In patients who have
undergone surgical correction, an ED provider must recognize high-risk
complications, including ascending cholangitis, as well as progression of
underlying disease leading to cirrhosis, portal hypertension, and liver failure.
CLINICAL PEARLS AND PITFALLS
Patients with BA have an elevated conjugated (direct) bilirubin
shortly after birth, even within the first 24 to 48 hours of life.
According to the American Academy of Pediatrics, all infants with
persistent jaundice beyond 2 to 3 weeks of age should have their
conjugated bilirubin measured to evaluate for cholestasis.
BA should be considered in any young infant with even minimal
conjugated hyperbilirubinemia (greater than normal) regardless of
the ratio to unconjugated bilirubin.
Identification within the first 30 to 45 days of life leads to improved
outcomes.
Patients with acholic stools should be investigated for biliary
disease, however acholic stools and hyperpigmented urine are
relatively late findings.
Cholangitis is the most common complication after a
hepatoportoenterostomy (Kasai) procedure.
Current Evidence
