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Hyperlipoproteinemia type V

Hyperlipoproteinemia is the presence of elevated levels of lipoprotein in the blood. Lipids (fatty molecules) are transported in a protein capsule, and the density of the lipids and type of protein determines the fate of the particle and its influence on metabolism. more...

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Although the terms hyperlipoproteinemia and hypercholesterolemia are often used interchangeably, the former is more specific. The term "hyperchylomicronemia" is used for an excess of chylomicrons.

Hyperlipoproteinemias are classified according to the Fredrickson/WHO classification (Fredrickson et al 1967), which is based on the pattern of lipoproteins on electrophoresis or ultracentrifugation.

Hyperlipoproteinemia type I

This very rare form (also known as "Buerger-Gruetz syndrome", "Primary hyperlipoproteinaemia", or "familial hyperchylomicronemia"), is due to high chylomicrons, the particles that transfer fatty acids from the digestive tract to the liver.

Hyperlipoproteinemia type II

Hyperlipoproteinemia Type II is hyperlipidemia (hypercholesterolemia) in the Fredrickson classification, which is determined by lipoprotein electrophoresis.

Hyperlipoproteinemia type II is further classified into:

  • Type IIa (elevated LDL only)
    • Polygenic hypercholesterolaemia
    • Familial hypercholesterolemia (FH)
  • Type IIb - combined hyperlipidemia (elevated LDL and VLDL, leading to high triglycerides levels)
    • Familial combined hyperlipoproteinemia
    • Secondary combined hyperlipoproteinemia

Hyperlipoproteinemia type III

This form is due to high chylomicrons and IDL (intermediate density lipoprotein).

Hyperlipoproteinemia type IV

This form is due to high triglycerides. It is also known as "hyperglyceridemia" (or "pure hyperglyceridemia".

Hyperlipoproteinemia type V

This type is very similar to Type I, but with high VLDL.

Unclassified forms

Non-classified forms are extremely rare:

  • Hypo-alpha lipoproteinemia
  • Hypo-beta lipoproteinemia

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Xerostomia
From Drug Store News, 4/26/99 by Carol Dickson McKee

Introduction

Xerostomia, often referred to as dry mouth, is a common problem that not only impairs health, but also may dramatically affect quality of life. An understanding of normal saliva formation and function and common causes of, complications from and methods to treat xerostomia will provide the pharmacist with valuable insights to help improve patient function and quality of life.

Xerostomia refers to the patient's perception of oral dryness, usually due to the lack of normal secretions. It is most commonly due to decreased salivary flow; less commonly, there may be a complete cessation of salivary flow. Dry mouth may also be caused or worsened by dry air, breathing through the mouth and cigarette smoking, among other factors.

What is xerostomia?

What is [saliva?.sup.(37)(37)(40)]

Saliva is the clear, usually alkaline, somewhat viscid secretion from the parotid, submaxillary, sublingual and smaller mucous glands of the mouth. Saliva consists primarily of water, but also contains enzymes and other proteins, small organic molecules, electrolytes and constituents of nonsalivary origin (Table 1). The details of salivary secretion are outlined in Figures 1 and 2.

What does saliva [do?.sup.37-40]

Saliva aids in speech, taste and the preparation of food for digestion. Saliva also often provides a first defense against chemical, mechanical and infectious attacks. Its many protective functions include lubrication, antimicrobial activity, remineralization, cleansing, buffering and helping to maintain mucosal integrity. Functions of saliva are detailed in Table 2.

Common signs and symptoms of [xerostomia.sup.37-40]

Signs and symptoms of xerostomia include complaints of dry mouth, fissures or sores at lip corners (angular cheilosis/cheilitis), halitosis, difficulty with speech and swallowing and the constant need for fluids. Chewing some types of food, particularly dry foods, is also difficult. The tongue is frequently described as "burning" or "tingling," and changes in taste are common.

The oral mucosa loses its usual moistness and glistening properties and becomes dehydrated and fissured. It is frequently inflamed and erythremic, though it may also appear pale and thin. Tissue may be cracked and bleeding and there may be fissuring and lobulation, especially on the dorsum of the tongue.

Patients with dentures or prostheses are likely to have difficulty wearing them as long as usual. Xerostomia decreases the oral pH and significantly increases the development of plaque and dental cavities or caries; these are often located at sites not generally susceptible to decay. Xerostomia may alter the normal mouth flora, increasing especially the concentrations of Streptococcus mutans and Lactobacillus, two microorganisms which have been associated with dental decay. Candidiasis is common, especially on the tongue and palate. Gingivitis and periodontal disease may also occur.

Saliva, which is present, is thicker and more stringy than usual and there is difficulty milking saliva from the ducts of the major salivary glands.

Additional problems caused by [xerostomia.sup.38]

Nutritional problems, both with respect to quantity and quality of food selected, may occur in patients with xerostomia, especially in those who experience alterations in taste. Sleep may be disrupted both by thirst and by the necessity of making frequent nocturnal visits to the bathroom secondary to fluid ingestion prior to bedtime. Xerostomia may make speaking difficult, and patients may make smacking sounds because the tongue tends to stick to the hard palate. Patients may develop social phobias and avoid public speaking, talking on the telephone and other types of socializing. All of these factors decrease a patient's quality of life.

Of particular note to the pharmacist is the decrease in compliance possible secondary to difficulty patients may have swallowing their medications. Dry mouth may also delay the dissolution of sublingual tablets, such as nitroglycerin.

Common causes of [xerostomia.sup.37-40]

Common causes of xerostomia include medications, irradiation to the head and neck and organic and psychogenic diseases. Classes of medications that commonly cause xerostomia include anorectics, anticholinergics, antidepressants, antihistamines, antihypertensives, antiparkinson medications, antipsychotics, antispasmodics, decongestants or other sympathomimetics, diuretics, sedative/hypnotics and possibly narcotic analgesics. The potential to cause xerostomia generally increases as a medication's anticholinergic properties increase, and within each category of drugs there is often a wide range of anticholinergic activity. It is often possible, therefore, to decrease xerostomia by changing from one medication to another (Tables 3 and 4). Some cancer chemotherapeutic agents, including methotrexate and fluorouracil, may also cause xerostomia, usually by their direct action on the salivary glands.

Xerostomia is one of the most frequent side effects of irradiation to the head and neck and is due to changes in the salivary glands. It tends to worsen as the dose of irradiation increases and may or may not be reversible upon cessation of irradiation. Irradiation-induced xerostomia may also be exacerbated by the use of some cancer chemotherapeutic agents.

Rheumatoid (connective tissue, collagen, autoimmune) conditions that may cause xerostomia include Sjorgren's syndrome, rheumatoid arthritis, systemic lupus erythematosus and scleroderma. Other organic diseases that may cause xerostomia include diabetes mellitus, hypertension, cystic fibrosis and neurological diseases, such as Bell's palsy, cerebral palsy and trauma. Hyposecretory conditions, including primary biliary cirrhosis, atrophic gastritis, graft vs. host disease, pancreatic insufficiency, type V hyperlipoproteinemia and immunodeficiency diseases, such as acquired immunodeficiency disease, may also cause xerostomia.

Any condition which contributes to dehydration will also tend to produce xerostomia; these include impaired water intake; loss of water through the skin secondary to burns, fever or excessive sweating; emesis; diarrhea; polyuria; osmotic diuresis and blood loss. Psychogenic diseases, such as depression and anxiety, may also cause xerostomia; it may be difficult in these cases especially to separate the xerostomia-inducing contribution from the disease state and the medication(s) used to treat it.

Xerostomia is often exacerbated by activities such as rapid breathing, breathing through the mouth, smoking or drinking alcohol. Decreased mastication may also contribute to dry mouth since chewing also influences salivary secretion. It is not clear whether the incidence of xerostomia increases in the elderly, or if this is more a function of an increased likelihood of chronic diseases and the use of multiple medications.

Helping patients who complain of dry [mouth.sup.(38)(39)]

Pharmacists are in an ideal position to counsel patients on ways to decrease xerostomia or at least lessen its impact on their quality of life. When a pharmacist is speaking with a patient who complains of dry mouth, common transitory problems, such as breathing through the mouth while congested from a cold, should be considered. Dry air is also a common problem, especially during the winter months. Social history, particularly alcohol consumption and whether a person smokes cigarettes, should be considered.

Patients should he encouraged to conduct a daily mouth exam, checking the inside of the cheeks, the roof of the mouth, the gums and the tongue for red, white or dark patches or ulcers. If anything unusual is found, it should be reported to the physician. Patients should also he encouraged to maintain meticulous oral hygiene and visit their dentists regularly for prophylactic maintenance.

The pharmacist should review the patient's complete medication profile, including prescription and nonprescription drugs, herbal and related products, for medications that may cause or worsen xerostomia. If it is drug-induced or drug-exacerbated, then contacting the prescriber to change medications may alleviate the symptoms.

Easy-to-take dosage formulations, such as liquids, may be preferred. Sublingual dosage forms should be avoided. It should be made clear to patients which medications can and which should not be crushed. Tablets and capsules are easier to swallow if the mouth and throat are first lubricated with water or a similar substance; medications should be followed by at least a half glass of water.

The ability to obtain a complete medication profile coupled with consistently excellent patient consultations by all pharmacists at one site is another reason patients should be encouraged to purchase all of their healthcare products at one pharmacy. Other ideas to help minimize dry mouth are listed in Table 5.

Products and treatments that increase comfort and prevent complications from [xerostomia.sup.(37)(39)(41)]

Treatment of xerostomia is directed toward the control of dental decay, relief of symptoms and increasing the flow of saliva, if possible.

[Fluoride.sup.(40)(41)]

Fluoride is available as rinses and gels and acts topically to increase tooth resistance to acid dissolution by promoting remineralization and inhibiting cariogenic or cavity-causing microorganisms. Acidulation provides greater topical fluoride uptake by dental enamel than neutral solutions. Acidulated fluorides are, therefore, more effective and should be used whenever possible, although neutral fluorides may become necessary temporarily in patients with mucositis, such as that secondary to irradiation or cancer chemotherapy. Acidulated fluorides may dull porcelain and composite restorations.

The use of stannous fluoride gels 0.4 percent or sodium fluoride gel 1.1 percent as toothbrushing agents may also be recommended. Patients should also use a soft or very soft bristled toothbrush to minimize irritation.

Rinses and gels are most effective immediately after brushing and flossing and just prior to sleep. Patients should usually vigorously swish 5 ml to 10 ml of a fluoride rinse for one minute and then expectorate or spit it out. The fluoride rinse should not be swallowed. Patients should not eat or drink for at least 30 minutes after rinsing. Fluoride rinses are usually used at bedtime.

Meticulous attention to oral hygiene and regular dental check-ups are essential to minimize the development of caries; patients should also avoid sucrose and other sugars, particularly in forms that have prolonged contact with the teeth, such as sweetened hard candy or lollipops.

Patients who are likely to experience oral complications, such as those undergoing head and neck irradiation or receiving xerostomia-inducing chemotherapy, should have a complete dental consult before therapy is initiated. Potential sources of irritation and infection can be eliminated, and preventative measures can improve and maintain oral health dramatically.

Patients with a history of susceptibility to dental caries should use a professionally-designed topical fluoride program in addition to artificial saliva products. These patients should also use very soft toothbrushes and avoid mouth rinses with high alcoholic content, since alcohol can cause pain and contribute to xerostomia.

Artificial saliva and related [products.sup.38-40]

Commercially available artificial salivas relieve soft tissue discomfort and are more effective and longer lasting than simple rinses. Artificial saliva preparations are designed to mimic natural saliva, both chemically and physically. They do not stimulate natural salivary gland production, however, so they are a replacement therapy rather than a cure for xerostomia.

Artificial salivas are available in a variety of products and formulations, including solutions, sprays, gels and lozenges; patients should try several until they find one that works best for them. One advantage of the gel formulation is that it promotes prolonged contact with the oral mucosa. Common saliva substitutes are listed in Table [6.sup.41]. In general, they contain:

An agent to increase viscosity: Carboxymethylcellulose, hydroxyethylcellulose

(Glycerin should generally be avoided since it absorbs water and actually dries the oral cavity.)

Minerals: All products contain calcium and phosphate ions, and some also contain fluoride.

Preservatives: Salivart does not contain preservatives because it is packaged as a sterile aerosol. Other products contain preservatives, including methyl- or propylparaben, which may cause hypersensitivity reactions in some patients.

Flavoring and related agents: Flavorings such as mint or lemon (caution: acidic) and/or sweeteners, such as sorbitol and xylitol, are commonly used.

Artificial salivas have few side effects and can generally be used on an "as needed" basis. The main limitations to their use are their limited duration of activity and cost to the patient. Artificial salivas can also be applied to dentures or prostheses prior to insertion.

Related [products.sup.(37)(38)]

Chewing sugarless gum stimulates salivary flow and forces saliva between the teeth, which helps prevent the formation of caries. Biotene chewing gum is sugarless, sweetened with the non-cariogenic xylitol and does not stick to dentures. It addition, it contains the enzymes glucose oxidase and lactoperoxidase. These enzymes combine with potassium thiocyanate, which is found in saliva, to produce the hypothiocyanate ion. This ion normally occurs in saliva and inhibits the growth and acid production of plaqueforming bacteria. These same anticariogenic enzymes are found in Biotene toothpaste, mouthwash and Oralbalance gel, which are listed in Table 6. More information about these products may be obtained online at www.laclede.com.

[Pilocarpine.sup.(38)(40)(41)]

Relatively low doses of pilocarpine (Salagen) (5 mg to 10 mg TID) may stimulate salivary flow and produce clinically significant benefits in some patients. Pilocarpine is a cholinergic parasympathomimetic agent with predominantly muscarinic action. In addition to increasing salivary secretions, it can also increase those of the sweat, lacrimal, gastric, pancreatic and intestinal glands, and in the mucous cells of the respiratory tract. Since pilocarpine and artificial salivas work by different mechanisms, concomitant use may provide additive relief.

Pilocarpine is contraindicated in patients with uncontrolled asthma, in conditions in which miosis is undesirable, such as narrow angle or angle-closure glaucoma and acute iritis, and in patients with a known hypersensitivity to the drug. Pilocarpine should be used with caution in patients with significant cardiovascular disease, and in those with controlled asthma, chronic bronchitis or chronic obstructive pulmonary disease. It should also be used with caution in patients with known or suspected cholelithiasis or biliary tract disease, nephrolithiasis or psychiatric disorders, since cholinergic agents produce dose-related central nervous system effects.

Pilocarpine is a cholinergic parasympathomimetic agent, so it may have additive adverse effects with other parasympathomimetic drugs, such as carbachol and neostigmine. It may antagonize anticholinergics. Pilocarpine may increase the risk of conduction disturbances in patients taking beta blockers.

Dose-related adverse effects from pilocarpine include sweating, rhinitis, headache, nausea, chills, dizziness, flushing, asthenia, urinary frequency, increased lacrimation, visual disturbances and abdominal cramps. Cardiovascular and pulmonary adverse effects are also possible.

Pilocarpine may cause decreased visual acuity, so caution should be used when driving, especially at night, and when performing hazardous activities in reduced lighting. Patients who sweat excessively should be certain to take in adequate fluids to avoid dehydration.

Conclusion

Pharmacists are usually the healthcare professionals patients encounter most often and are in an ideal position to counsel patients. By understanding xerostomia and its common causes, complications and pharmacological and non-pharmacological treatments, pharmacists can help patients minimize xerostomia or at least lessen its impact on their quality of life.

Some ideas to minimize dry [mouth.sup.37-40]

* Do not smoke; drink in moderation, if at all

* Drink plenty of water. Many people carry a squeeze bottle or sports bottle to sip from throughout the day. It may be useful to keep one at the bedside, also.

* Use lip lubricants or balms as needed.

* Drink liquids with meals and use gravies and sauces to make food easier to swallow.

* Soft foods, such as macaroni and cheese and scrambled eggs, may be easier to eat; foods may also be pureed.

* Avoid dry foods, such as crackers, cookies and toast, or soften them with liquids before eating.

* Eating smaller, more frequent meals may be easier than eating two or three larger meals.

* Avoid or minimize intake of carbonated, citrus and caffeinated drinks.

* If acidic juices cause irritation, fruit-flavored drinks or milk may be used.

* Avoid overly salty food.

* Avoid chewable vitamin C tablets and acidic, sugared lozenges.

* Suck on sugar-free hard candy. Sour flavors may especially stimulate salivation; those with citric acid should be avoided.

* Chew sugar-free gum.

* Suck on ice chips or sugar-free popsicles.

* If air is dry, try a humidifier or vaporizer, especially at night.

* Coat the oral cavity with small amounts of vegetable oil two or three times daily and before bedtime; be certain not to aspirate the oil.

* Use mouth rinses or washes that do not contain alcohol or peroxide.

* Maintain meticulous mouth care. Use a low abrasive toothpaste with fluoride. Sodium lauryl sulfate may contribute to the formation of apthous ulcers or canker sores and should be avoided by susceptible individuals. Waxed floss may be easier than unwaxed to use. A soft or super-soft toothbrush should be used.

Goal

This article will provide the pharmacist with an understanding of xerostomia and its common causes, complications and pharmacological and non-pharmacological treatments.

Objectives

After reading this article, the pharmacist should be able to:

* Discuss normal saliva formation and function.

* Identify common causes of xerostomia and discuss the impact of xerostomia on quality of life.

* Discuss drug groups that commonly cause xerostomia and potential therapeutic alternatives that possess fewer anticholinergic effects.

* Describe the sequelae of untreated xerostomia and methods to help prevent these complications.

* List methods of improving a patient's ability to function with dry mouth.

* Discuss the pharmacology, contraindications and adverse effects of pilocarpine.

Questions on "Xerostomia," published April 1999 (lesson 401- -000-99018-HOl), are worth two hours of credit. Mail completed answer sheet to DrSN/CP CE, P.O. Box 31180, Tampa, Fla., 33631-3180. For faster service, fax to (813) 626-7203.

1. Which of the following is the primary component of saliva?

a. Water

b. Proteins

c. Small organic molecules

d. Electrolytes

2. Which of the following constituents of non-salivary origin may be found in saliva?

a. Blood cells

b. Bronchial secretions

c. Desquamated epithelial cells

d. All of the above

3. Which of the following is/are protective functions provided by saliva?

a. Lubrication

b. Antimicrobial activity

c. Helping to maintain mucosal integrity

d. All of the above

4. Which of the following functions of saliva would be helped most by the presence of amylase, lipase and proteases?

a. Speech

b. Digestion

c. Lubrication

d. Buffering

5. Which of the following is least likely to be found in a patient with xerostomia?

a. Halitosis

b. Difficulty with speech

c. Moist, glistening oral mucosa

d. Difficulty with swallowing

6. Which of the following is least likely to occur in patients with xerostomia?

a. An increased incidence of plaque

b. A decreased incidence of dental caries

c. An increased incidence of oral Candida infections

d. A decrease in oral pH

7. Which of the following classes of medications is least likely to cause xerostomia?

a. Anorectics

b. Anticholinergics

c. Analgesics (non-narcotic)

d. Antihistamines

8. Which of the following antidepressants is most likely to cause xerostomia?

a. Amitriptyline

b. Desipramine

c. Fluoxetine

d. Nefazadone

9. Which of the following antipsychotics is most likely to cause xerostomia?

a. Fluphenazine

b. Perphenazine

c. Promazine

d. Prochlorperazine

10. Which of the following medications or treatments used in some patients with cancer is least likley to cause xerostomia?

a. Irradiation to the head and neck

b. Corticosteroids

c. Methotrexate

d. Fluorouracil

11. Which of the following autoimmune or rheumatoid conditions may cause xerostomia?

a. Sjorgren's syndrome

b. Rheumatoid arthritis

c. Scleroderma

d. All of the above

12. Which of the following is least likely to worsen xerostomia?

a. Chewing gum

b. Smoking cigarettes

c. Drinking alcohol

d. Breathing rapidly

13. Which of the following interventions or consultations would not be appropriate for a pharmacist to make with respect to a patient experiencing dry mouth?

a. Selecting an easy-to-swallow dosage formulation

b. Choosing a medication with a high degree of anticholinergic activity

c. Telling which medications should and should not be crushed

d. Advising the patient to lubricate the mouth and throat with water prior to swallowing tablets or capsules.

14. Which of the following beverages would be most useful in patients with xerostomia?

a. Coffee

b. Carbonated soda pop

c. Water

d. Homemade lemonade

15. Which of the following are desirable characteristics in a toothpaste for patients with dry mouth?

a. Is low abrasive

b. Does not contain sodium lauryl sulfate

c. Contains fluoride

d. All of the above

16. Which of the following is NOT an appropriate consultation for patients using fluoride rinses?

a. Use 5-10 ml of fluoride rinse

b. Vigorously swish fluoride rinse in mouth for about a minute

c. Swallow the fluoride rinse

d. Do not eat or drink for 30 minutes after using fluoride rinse

17. Which of the following is least likely to be found in artificial saliva products?

a. Sucrose

b. Carboxymethylcellulose

c. Calcium

d. Parabens

18. Which of the following saliva substitutes is not a spray?

a. Entertainer's Secret

b. Glandosane

c. Mouthkote

d. Salivart

19. Which of the following saliva substitutes contains fluoride?

a. Moi-Stir Swabsticks

b. Optimoist

c. Salivart

d. Salix

20. Which of the following saliva substitutes is available as a gel?

a. Entertainer's Secret

b. Glandosane

c. Mouthkote

d. Oralbalance

COPYRIGHT 1999 Lebhar-Friedman, Inc.
COPYRIGHT 2000 Gale Group

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