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Chylomicronemia and the Chylomicronemia Syndrome: A Practical Approach to Management

https://doi.org/10.1016/j.amjmed.2007.10.004Get rights and content

Abstract

Chylomicronemia is present when triglyceride levels exceed 1000 mg/dL. Chylomicronemia, when accompanied by eruptive xanthoma, lipemia retinalis, or abdominal symptoms, is referred to as the “chylomicronemia syndrome” and can cause acute pancreatitis. Treatment aimed at reducing triglyceride levels includes lifestyle modifications to promote weight loss with diet and physical activity coupled with medications, including fibrates, n-3 polyunsaturated fatty acids, and nicotinic acid. Chylomicronemic patients with acute pancreatitis require insulinization in an inpatient setting to abolish chylomicronemia.

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Cause of Chylomicronemia

Chylomicronemia results from impaired triglyceride metabolism. The causes of hypertriglyceridemia can be classified as primary and secondary. These result from increased very low-density lipoprotein production, reduced very low-density lipoprotein clearance, or more commonly the dual effect of both.8

Familial hypertriglyceridemia, inherited as an autosomal dominant trait, is the most common primary cause of chylomicronemia in adults. Secondary causes of chylomicronemia are listed in Table 1.

Clinical Evaluation

A laboratory diagnosis of chylomicronemia occurs when triglyceride levels exceed 1000 mg/dL. A 12-hour fast is recommended for optimally assessing triglyceride values. Acute physiologic stress, including infections and the acute coronary syndrome, can increase triglyceride levels by 2-fold in some patients.

Relevant historical information includes a family history of lipid disorders and diabetes mellitus, a personal history of diabetes mellitus and pancreatitis, and the ingestion of medications

Treatment Recommendations

The National Cholesterol Education Program Adult Treatment Panel III treatment guidelines7 recommend the ideal goal of decreasing triglyceride levels to less than 150 mg/dL. Typically this is not realistic. The main goal is to maintain fasting triglyceride levels at less than 500 mg/dL to reduce the risk for acute pancreatitis. Table 2 summarizes the main points in treatment.

Lifestyle Intervention for All Patients

Therapeutic lifestyle changes emphasizing a low-fat diet and physical activity are recommended to all patients. The purpose of this intervention, aimed at promoting weight loss, is to reduce dietary saturated fats (<7% of total calories) and cholesterol (<200 mg/d) intake, and increase physical activity levels. Physical activity can reduce body fat and triglycerides.11 The therapeutic lifestyle change intervention used in the Diabetes Prevention Study, considered to be the “gold standard,” can

Inpatient Management

Chylomicronemic patients with abdominal pain should be considered to have a medical emergency because of the concern for acute pancreatitis. Typically this occurs in the setting of uncontrolled diabetes mellitus. The initial goal of abolishing chylomicronemia is best accomplished in a hospital setting. The patient should receive nothing by mouth and stay hydrated with intravenously administered fluids. Low-dose insulin should be administered (nondiabetic patients will require intravenously

Conclusions

Chylomicronemia will be assuming greater clinical relevance as the incidence of overweight/obesity and diabetes mellitus continues to increase. Practitioners have an important role in evaluating chylomicronemic patients and implementing therapeutic lifestyle and pharmaceutic interventions aimed at reducing the risk for acute pancreatitis.

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