Abstract
Q fever is a zoonosis with a worldwide distribution that is caused by the intracellular bacterium Coxiella burnetii. Although most infections in children are asymptomatic and self-limiting, some experience severe or chronic manifestations. Its manifestations in patients with sickle cell disease are unknown, as there are no reports currently. We report the case of a 4-year-old child with sickle cell disease who was admitted to the intensive care unit with fever, septic shock and fulminant hepatic failure secondary to hepatic sequestration crisis and intrahepatic cholestasis. Coxiella burnetii infection was confirmed by molecular and serologic assays. Empiric therapy with doxycycline had a significant impact on his course, and he made an excellent recovery despite requiring extensive life-supportive measures initially. This is the first report of Q fever in a patient with sickle cell disease, demonstrating its capability to manifest as acute sickle hepatopathy with critical illness.
Published Version
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