Answer: Treatment is aimed at reducing triglyceride levels and may require fasting to lower the triglyceride levels into a safer range.  Reference: Basa ALP, Afsharkharaghan H (Zollo A, ed): Endocrinology in Medical Secrets, 3rd ed. 2001.

Answer: Physical manifestations of this syndrome include lipemia retinalis, eruptive xanthomas, and hepatomegaly. Patients are at increased risk of developing pancreatitis.  Reference: Basa ALP, Afsharkharaghan H (Zollo A, ed): Endocrinology in Medical Secrets, 3rd ed. 2001.

Answer: The chylomicronemia syndrome typically occurs when triglyceride levels are > 1000 mg/dl. Patients develop severe triglyceride elevations when the enzyme lipoprotein lipase (LPL), which is responsible for triglyceride hydrolysis of chylomicrons and very low density lipoprotein (VLDL), becomes saturated and is no longer able to clear chylomicrons from the circulation. When the LPL enzyme [...]

Answer: FH is an autosomal dominant disorder due to a mutation in the LDL receptor (causing a deficient or defective receptor) that leads to altered LDL catabolism and increased cholesterol synthesis. Approximately 1/500 people are heterozygous carriers of a mutation and 1/1,000,000 are homozygous for the disorder. Such people have much higher rates of premature [...]

Answer: Lipoproteins are named based on their density and are composed of nonpolar (and therefore water-insoluble) cholesterol esters and triglycerides surrounded by a layer of polar (and therefore water-soluble) proteins and lipids (unesterified cholesterol and phospholipids). This structure allows the entire particle to remain miscible in serum. LIPOPROTEIN PARTICLES Lipoprotein Particle Location of Origin Composition [...]

Answer: Phentermine, a norepinephrine agonist (15-30 mg/day) Sibutramine, a combination norepinephrine/serotonin reuptake blocker (5, 10, and 15 mg; start with 10 mg and titrate) Orlistat, a pancreatic lipase inhibitor (120 mg 3 times/day before meals) Reference: Larson PR, et al: Williams Textbook of Endocrinology, 10th ed. Philadelphia, W.B. Saunders, 2003.

Answer: Typically, the first approach is diet and exercise with behavioral modifications followed by the addition of pharmacotherapy. Pharmacotherapy should be considered in patients with a BMI over 30, a BMI over 27 with comorbidities, or minimal response after 6 months of lifestyle modifications.  Reference: Larson PR, et al: Williams Textbook of Endocrinology, 10th ed. [...]