Control of dietary intake is an important facet of disease control for a patient with familial lipoprotein lipase deficiency.


Goal: To keep plasma triglyceride concentrations < 2,000 mg/dL and to minimize chylomicronemia.


Dietary restrictions:

(1) Dietary fat intake should be restricted to < 20 grams per day or < 15% of total energy intake (with fat providing 9 kcal per gram).

(2) Fish oil supplements are contraindicated.

(3) Use medium-chain triglycerides for cooking (these are not incorporated into chylomicrons).

(4) Avoid parenteral nutrition using a lipid emulsion or containing excess calories during an episode of acute pancreatitis.


Clinical findings suggesting inadequate dietary restrictions:

(1) continued symptoms

(2) a plasma trigylceride > 2,000 mg/dL

(3) recurrent or persistent eruptive xanthomas

(4) persistent hepatosplenomegaly


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