Hyper-lipoproteinemia
About one in five persons with elevated plasma lipid and lipoprotein levels has hyperlipoproteinemia, an inherited disorder marked by increased plasma concentrations of one or more lipoproteins. Hyperlipoproteinemia may also occur secondary to other conditions, such as diabetes, pancreatitis, hypothyroidism, and renal disease.
This disorder affects lipid transport in serum and produces varied clinical changes, from relatively mild symptoms that can be corrected by dietary management to potentially fatal pancreatitis.
Hyperlipoproteinemia occurs as five distinct metabolic disorders. Types I and III are transmitted as autosomal recessive traits; types II, IV, and V are transmitted as autosomal dominant traits.
Causes
Each type of hyperlipoproteinemia has distinct causes and incidence. (See Types of hyperlipoproteinemia, page 414.)
Signs and symptoms
Clinical features of hyperlipoproteinemia vary according to the type of disorder:
❑ Type I: recurrent attacks of severe abdominal pain similar to pancreatitis, usually preceded by fat intake; abdominal spasm, rigidity, or rebound tenderness; hepatosplenomegaly with liver or spleen tenderness; papular or eruptive xanthomas (pinkish yellow cutaneous deposits of fat) over pressure points and extensor surfaces; lipemia retinalis (reddish white retinal vessels); malaise; anorexia; and fever
❑ Type II: tendinous xanthomas (firm masses) on the Achilles tendons and tendons of the hands and feet, tuberous xanthomas, xanthelasma, juvenile corneal arcus (opaque ring surrounding the corneal periphery), accelerated atherosclerosis and premature coronary artery disease (CAD), and recurrent polyarthritis and tenosynovitis
❑ Type III: peripheral vascular disease manifested by claudication or tuberoeruptive xanthomas (soft, inflamed, pedunculated lesions) over the elbows and knees; palmar xanthomas on the hands, particularly fingertips; premature atherosclerosis
❑ Type IV: predisposition to atherosclerosis and early CAD, exacerbated by excessive calorie intake, obesity, diabetes, and hypertension
❑ Type V: abdominal pain (most common), pancreatitis, peripheral neuropathy, eruptive xanthomas on extensor surfaces of the arms and legs, lipemia retinalis, and hepatosplenomegaly.
Diagnosis
Diagnostic findings vary among the five types of hyperlipoproteinemia.
Treatment
The first goal is to identify and treat any underlying problem such as diabetes. If no underlying problem exists, the primary treatment for types II, III, and IV is dietary management, especially restriction of cholesterol intake, simple sugars, and refined carbohydrates that will elevate triglyceride levels. Omega-3 essential fatty acids (fish oils) have proved useful in reducing serum triglycerides. Drug therapy (cholestyramine, clofibrate, or pharmaceutical non-flush niacin) may also be used to lower the plasma triglyceride or cholesterol level when diet alone is ineffective.
Type I
In type I hyperlipoproteinemia, treatment requires long-term weight reduction, with fat intake restricted to less than 20 g/day. A 20- to 40-g/day medium-chain triglyceride diet may be ordered to supplement caloric intake. The patient should also avoid alcoholic beverages to decrease plasma triglycerides. The prognosis is good with treatment; without treatment, death can result from pancreatitis.
Type II
For type II, dietary management to restore normal lipid levels and decrease the risk of atherosclerosis includes restriction of cholesterol intake to less than 300 mg/day for adults and less than 150 mg/day for children; triglyceride levels must be restricted to less than 100 mg/day for children and adults. The diet should also be high in polyunsaturated fats.
In familial hypercholesterolemia, nicotinic acid with a bile acid usually normalizes low-density lipoprotein levels. For severely affected children, portacaval shunt is a last resort to reduce plasma cholesterol levels.
The prognosis remains poor regardless of treatment; in homozygotes, myocardial infarction usually causes death before age 30.
Type III
For type III, dietary management includes restriction of cholesterol intake to less than 300 mg/day; carbohydrates must also be restricted, and polyunsaturated fats are increased. Clofibrate and niacin help lower blood lipid levels. Weight reduction is helpful. With strict adherence to the prescribed diet, the prognosis is good.
Type IV
For type IV, weight reduction may normalize blood lipid levels without additional treatment. Long-term dietary management includes restricted cholesterol intake, increased polyunsaturated fats, and avoidance of alcoholic beverages. Clofibrate and niacin may lower plasma lipid levels.
The prognosis remains uncertain because of predisposition to premature CAD.
Type V
The most effective treatment for type V is weight reduction and long-term maintenance of a low-fat diet. Alcoholic beverages must be avoided. Niacin, clofibrate, gemfibrozil, and a 20- to 40-g/day medium-chain triglyceride diet may prove helpful.
The prognosis is uncertain because of the risk of pancreatitis. Increased fat intake may cause recurrent bouts of illness, possibly leading to pseudocyst formation, hemorrhage, and death.
Special considerations
❑ Care for patients with hyperlipoproteinemia emphasizes careful monitoring for adverse reactions to drugs and teaching the importance of long-term dietary management.
❑ Administer cholestyramine before meals or before bedtime. This drug must not be given with other medications. Watch for adverse reactions, such as nausea, vomiting, constipation, steatorrhea, rashes, and hyperchloremic acidosis. Also watch for malabsorption of other medications and fat-soluble vitamins.
❑ Give clofibrate as prescribed. Watch for adverse reactions, such as choelithiasis, cardiac arrhythmias, intermittent claudication, thromboembolism, nausea, weight gain (from fluid retention), and myositis.
Clinical tip Don’t administer niacin to patients with active peptic ulcers or liver disease. Use with caution in patients with diabetes. In other patients, watch for adverse reactions, such as flushing, pruritus, hyperpigmentation, and exacerbation of inactive peptic ulcers.
❑ Urge the patient to adhere to his diet (usually 1,000 to 1,500 calories/day), to avoid excess sugar and alcoholic beverages, to minimize intake of saturated fats (higher in meats, coconut oil), and to increase intake of polyunsaturated fats (vegetable oils).
❑ Instruct the patient, for the 2 weeks preceding serum cholesterol and serum triglyceride tests, to maintain a steady weight and to adhere strictly to the prescribed diet. He should also fast for 12 hours before the test.
❑ Instruct women with elevated serum lipid levels to avoid hormonal contraceptives or drugs that contain estrogen.
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Book Source Details
- Book Title: Handbook of Diseases
- Author(s): Springhouse
- Year of Publication: 2003
- Copyright Details: Handbook of Diseases, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright Details: Handbook of Diseases, Copyright © 2008 Williams & Wilkins.
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Title: Handbook of Diseases
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2003
ISBN: 1-58255-266-5
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