Multiorgan system dysfunction in the chylomicronemia syndrome

J Intensive Care Med. 2014 May-Jun;29(3):175-8. doi: 10.1177/0885066613475425. Epub 2013 Jan 29.

Abstract

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.

Keywords: acute respiratory failure; chylomicronemia; hypertriglyceridemia; hyperviscosity; multiorgan system dysfunction; plasmapheresis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Blood Viscosity
  • Humans
  • Hyperlipoproteinemia Type I / complications*
  • Male
  • Multiple Organ Failure / etiology*
  • Plasmapheresis
  • Triglycerides / blood

Substances

  • Triglycerides