Haydek et al evaluated patients with sickle cell disease (SCD) who developed extreme hyperbilirubinemia. This is associated with increased morbidity and mortality. The authors are from Emory University.
Patient selection: sickle cell disease (SS)
Extreme hyperbilirubinemia: serum total bilirubin > 13 mg/dL
Associations:
(1) elevated liver function tests
(2) two or more organ failures
(3) need for blood transfusion and/or exchange transfusion
Contributory factors may include:
(1) hepatic sequestration
(2) SCD-related hepatopathy, including intrahepatic cholestasis
(3) hemolysis
(4) infectious hepatitis
Cause of death:
(1) sepsis
(2) acute liver failure
(3) other organ failure
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