Multiorgan System Dysfunction in the Chylomicronemia Syndrome

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Objective:To describe an extreme presentation of the chylomicronemia syndrome resulting in multiorgan system dysfunction.Patient:A 40-year-old African American male with no past medical history presented with multiorgan system dysfunction manifested by acute respiratory failure and acute kidney injury. He was noted to have very-high triglyceride levels (>5000 mg/dL) at admission.Interventions:An echocardiogram showed normal cardiac function. Amylase and lipase were normal. We confirmed the chylomicronemia syndrome with a triglyceride assay. The associated hyperviscosity was treated with plasmapheresis to reduce the plasma triglyceride level.Results:After 3 sessions of plasmapheresis, his triglyceride levels were significantly reduced, his oxygenation improved, and his acute kidney injury resolved. He was successfully extubated on day 7 of the intensive care unit stay. His diabetes and hypertriglyceridemia were newly diagnosed and drug therapy was instituted with home discharge on day 14.Conclusions:Severe chylomicronemia can cause multiorgan system dysfunction related to hyperviscosity. Early institution of plasmapheresis to reduce the triglyceride-rich lipoproteins can improve tissue perfusion and prevent further organ damage.

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