Background: Severe Hypertriglyceridemia (HTG), i.e., plasma triglyceride levels exceeding
1000 mg/dL, is one of the established causes of acute pancreatitis and severe abdominal
pain. There are no established pediatric guidelines regarding treatment of children and adolescents
with severe HTG.
Objective: To review the pathophysiology and etiology of severe HTG in the pediatric age group,
and to discuss management options.
Method and Results: Severe HTG is usually due to deficient or absent Lipoprotein Lipase (LPL)
activity, which can be due to primary genetic etiology or secondary causes triggering HTG in those
with underlying genetic susceptibility. Hospitalization is indicated for patients with severe HTG
who are symptomatic with abdominal pain or pancreatitis, in those with uncontrolled diabetes requiring
insulin, or, in those with substantial elevations of plasma TG. Fasting followed by fat free
diet until plasma TG declines to <1000mg/dL is essential. Subsequently, stringent fat restriction
followed by slowly increasing the dietary fat while maintaining the plasma TG concentration at a
targeted level is recommended. Insulin infusions are helpful in patients who have some LPL activity,
especially in those with diabetes. Plasmapheresis may be considered in those with severe pancreatitis,
shock or multi-organ failure. Medications such as fibrates and omega-3 fatty acids are not
effective if LPL activity is absent or when plasma TG is >1800 mg/dL. Medications only have an
adjunct role in the management. Low fat diet, lifestyle changes, weight loss, control of secondary
causes, and patient education form the mainstay of management once the patient is discharged.