Hypertriglyceridemia

How is Hypertriglyceridemia diagnosed?

Lipid screening in all children ages 9–11 and 17–21 years or checking of lipid levels in the high-risk patients helps diagnose hypertriglyceridemia. Those with triglyceride >130 mg/dL should have two additional fasting lipid profiles measured 2 weeks to 3 months apart and averaged. Once the diagnosis is established, it is important to work up to look for the cause of high triglyceride and assess need for treatment. Diagnosis of Familial Chylomicronemia syndrome (FCS) can be fully confirmed by performing specific genetic testing, and any patient with triglycerides >500 mg/dL without any secondary causes should be referred to lipid specialist.

What are the causes of Hypertriglyceridemia?

• Overweight and Obesity
• Uncontrolled Diabetes mellitus- Type 1 or Type 2
• Lipodystrophy
• Hypothyroidism
• Medications (oral contraceptives, steroids, isotretinoin, L-asparaginase).
• Post-solid organ transplant ( on systemic steroids, tacrolimus, sirolimus)
• Type 1 hyperlipoproteinemia (T1HLP) or Familial Chylomicronemia syndrome (FCS): Children with familial chylomicronemia syndrome or FCS can present at any age with extremely elevated triglycerides >1000 mg/dL and have very high risk of pancreatitis. It is caused by mutation or impaired function of an enzymes called lipoprotein lipase or its associated factors.

How is Hypertriglyceridemia treated?

Treatment of hypertriglyceridemia is primarily driven toward lifestyle changes. In the setting of obesity, management is primarily aimed at weight control. Reduced caloric intake and increased exercise are associated with a significant reduction and stabilization of triglycerides. The American Academy of Pediatrics recommends that children get at least 1 hour of moderate to vigorous activity daily and screen time of < 2 hours a day. Children with triglyceride levels > 500 mg/dL are at risk for pancreatitis. Triglyceride lowering medications could be considered in consultation with a lipid specialist with severe hypertriglyceridemia. Children with FCS do not respond to standard lipid lowering therapy. They need to be on extremely low fat diet consisting of <15% of total daily calories from fat and should be referred to lipid specialist.