References

Colice. GL. Curtis A. Deslauriers J. et al. Medical and surgical treatment of parapneumonic effusions: An evidence-based guideline. Chest 2000:118:1158-1171. Light RS. Disorders of the pleura, mediastinum and diaphragm. In: Kasper DL, Braunwald E, Fauci AS, et al, eds. Harrison's principles of internal medicine, 16th ed. New York: McGraw-Hill. 2005:1565-1569. Light RW. Pleural effusion. N Engl J Med 2002;346:1971-1977.

A 25-year-old man presents to your clinic for a general checkup and cholesterol screening. He denies having medical problems and takes no medications on a regular basis. He works as a computer programmer, exercises regularly at a gym, and does not smoke or use illicit drugs. He drinks two to three beers on the weekend. His father suffered his first heart attack at age 36 years and eventually died of complications of a heart attack at age 49 years. The patient's older brother recently was diagnosed with "high cholesterol."

The patient's blood pressure is 125/74 mmHg and heart rate 72 bpm. He is 69 inches tall and weighs 165 lb. His physical examination is unremarkable.

Fasting lipid levels are drawn. The next day, you receive the results: total cholesterol 362 mg/dL. triglycerides 300 mg/dL, high-density lipoprotein (HDL) 36 mg/dL, and low-density lipoprotein (LDL) 266 mg/dL.

^ What is the most likely diagnosis?

♦ What are the possible complications if left untreated?

ANSWERS TO CASE 46: Hypercholesterolemia

Summary: A healthy 25-year-old man presents for a physical examination and is found to have markedly elevated total and LDL cholesterol and triglycerides, and low HDL cholesterol. He has an unremarkable physical examination. He is normotensive and is a nonsmoker. but he has a strong family history of hypercholesterolemia and premature atherosclerotic coronary artery disease.

^ Diagnosis: Familial hypercholesterolemia.

♦ Next step: Counsel regarding lifestyle modification with low-fat diet and exercise, and offer treatment with an HMG-CoA (|3-hydroxy-P-methylglutaryl-coenzyme A) reductase inhibitor.

♦ Complications if untreated: Development of atherosclerotic vascular disease, including coronary heart disease (CHD).

Analysis

Objectives

1. Know the risk factors for developing coronary artery disease and know how to estimate the risk for coronary events using the Framingham risk scoring system.

2. Be familiar with the recommendations for cholesterol screening and for the treatment of low-, intermediate-, and high-risk patients.

3. Understand how the different classes of lipid-lowering agents affect lipid levels and the potential side effects of those agents.

4. Know the secondary causes of hyperlipidemia.

Considerations

A young man presents to the clinic for a checkup and is found to have markedly elevated total cholesterol (normal <200 mg/dL) and LDL levels (normal <100 mg/dL), and low HDL levels (normal >45 mg/dL). He does not have any apparent secondary causes of dyslipidemia, and no signs or symptoms of vascular disease. He does have a strong family history of hypercholesterolemia and premature death caused by myocardial infarction. The decisions regarding the method and intensity of lipid-lowering therapy are based on one's estimation of the patient's 10-year risk of major coronary events. Because of his very high lipid levels and his family history, he is a high-risk patient and. thus, should be counseled about lipid-lowering medical therapy. The very high cholesterol levels at a young age in the absence of secondary causes leads one to suspect familial hypercholesterolemia, a condition caused by defective or absent LDL surface receptors and subsequent inability to metabolize LDL particles. Meanwhile, the importance of lifestyle modification cannot be overemphasized.

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