Pediatric emergency medicine trisk 0882 0882
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45 days of life. After 90 days of life, success rate of the Kasai HPE drops to
<25%. Even if successful, 70% of children will continue to develop fibrosis,
portal hypertension, and cirrhosis, and require liver transplantation.
Metabolic Abnormalities
The metabolic and hepatic disorders have variable symptoms at onset. Infants
with galactosemia, tyrosinemia, fatty acid oxidation disorders, and fructose
intolerance can present with cholestasis and may appear ill in the emergency
department due to metabolic derangement or secondary infection. Some cases
present acutely; however, many children can have an antecedent history of failure
to thrive, developmental delay, and inconstant jaundice. A family history of
unexplained mortality in childhood or unexplained pulmonary, gastrointestinal,
neurologic, or psychiatric disturbance in other family members may provoke
diagnostic consideration.
Obstructive Etiologies: Gallstones, Cysts
Biliary calculi and acute inflammation of the gallbladder are less common causes
of conjugated hyperbilirubinemia in the pediatric population except among
patients with specific underlying conditions. For example, cholelithiasis may
complicate any of the hemolytic anemias, particularly in patients with sickle
hemoglobinopathies. Other risk factors for gallstones include obesity, Crohn
disease, chronic parenteral nutrition, and cystic fibrosis. Cholecystitis may also
accompany a variety of acute focal infections, such as pneumonia or peritonitis,
and may occur in the course of bacterial sepsis. In less severe presentations of
acute cholecystitis, fever, nausea, vomiting, abdominal distension, and right upper
quadrant pain are prominent features.
Other causes of obstruction include choledochal cysts, which can present with
triad of right upper quadrant abdominal mass, pain, and jaundice. Acute hydrops
of the gall bladder, from Kawasaki disease or systemic streptococcal infection,
can also appear with acute, painful right upper quadrant mass associated with
jaundice. When cholelithiasis or other obstruction from stones or cysts is
suspected, ultrasound of the right upper quadrant should be performed as it is
highly sensitive and specific for stones in the gallbladder. Endoscopic retrograde
cholangiopancreatography (ERCP) is more helpful to identify stones and
abnormalities in the extrahepatic biliary tree and common bile duct. If a
choledochal cyst is not seen by ultrasound but there is a high index of suspicion
for this diagnosis, magnetic resonance cholangiopancreatography (MRCP) is the
diagnostic test of choice.
