Pediatric emergency medicine trisk 2075 2075
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blood flow and hypoxia. Patients may also present with worsening cirrhosis
and liver failure as a result of progression of their underlying disease.
Management. The most important role for the emergency provider in
managing a patient with BA is early identification of the disorder. Once the
diagnosis is made, management is primarily surgical. In patients with a
conjugated hyperbilirubinemia, ED providers should further evaluate for
cholestasis (total and fractionated bilirubin, GGT, alkaline phosphatase),
aminotransferase elevation (AST, ALT), and liver function (albumin, PT/INR,
PTT) (see Fig. 44.1 for further information on the workup of Conjugated
Hyperbilirubinemia). Patients should also receive an abdominal ultrasound
with Doppler to assess for other anatomic causes as well as obstruction and
chest x-ray to assess for possible vertebral body changes or rib cage changes
suggestive of Alagille’s. The patient should be urgently referred to
Gastroenterology and Pediatric Surgery.
CHOLELITHIASIS/CHOLECYSTITIS
Gallstones or cholelithiasis occasionally occur in adolescents. Often the
condition remains asymptomatic until a complication develops, such as
cholecystitis. It is primarily seen in patients with hemolytic anemias (pigment
stones) such as sickle cell disease and hereditary spherocytosis, however it
becoming increasingly common in otherwise healthy children, likely as a
result of childhood obesity and diabetes. Other risk factors for developing
gallstones includes the use of total parenteral nutrition (TPN), systemic
infection, antibiotic use, biliary anatomic abnormalities, cystic fibrosis,
increased estrogen, and family history. While not nearly as common in adults,
children do account for 4% of all cholecystectomies, and two-thirds are
female.
Biliary colic results from acute transient obstruction of the cystic duct or
common bile duct by gallstone(s). Cholecystitis is an aseptic inflammatory
process that develops as a reaction to chemical injury triggered by obstruction
to the cystic duct by a gallstone. Cholangitis results from secondary bacterial
infection by enteric organisms in the face of biliary tract obstruction or after
surgical manipulation of the biliary tract. Acute cholangitis may be mild and
superficial, producing only short-lived symptoms, or it may be severe, causing
suppurative cholangitis with septic shock and formation of hepatic abscesses.
