Diphtheria is a potentially fatal, contagious bacterial disease that usually involves the nose, throat, and air passages, but may also infect the skin. Its most striking feature is the formation of a grayish membrane covering the tonsils and upper part of the throat.
Like many other upper respiratory diseases, diphtheria is most likely to break out during the winter months. At one time it was a major childhood killer, but it is now rare in developed countries because of widespread immunization. Since 1988, all confirmed cases in the United States have involved visitors or immigrants. In countries that do not have routine immunization against this infection, the mortality rate varies from 1.5% to 25%.
Persons who have not been immunized may get diphtheria at any age. The disease is spread most often by droplets from the coughing or sneezing of an infected person or carrier. The incubation period is two to seven days, with an average of three days. It is vital to seek medical help at once when diphtheria is suspected, because treatment requires emergency measures for adults as well as children.
The symptoms of diphtheria are caused by toxins produced by the diphtheria bacillus, Corynebacterium diphtheriae (from the Greek for "rubber membrane"). In fact, toxin production is related to infections of the bacillus itself with a particular bacteria virus called a phage (from bacteriophage; a virus that infects bacteria). The intoxication destroys healthy tissue in the upper area of the throat around the tonsils, or in open wounds in the skin. Fluid from the dying cells then coagulates to form the telltale gray or grayish green membrane. Inside the membrane, the bacteria produce an exotoxin, which is a poisonous secretion that causes the life-threatening symptoms of diphtheria. The exotoxin is carried throughout the body in the bloodstream, destroying healthy tissue in other parts of the body.
The most serious complications caused by the exotoxin are inflammations of the heart muscle (myocarditis) and damage to the nervous system. The risk of serious complications is increased as the time between onset of symptoms and the administration of antitoxin increases, and as the size of the membrane formed increases. The myocarditis may cause disturbances in the heart rhythm and may culminate in heart failure. The symptoms of nervous system involvement can include seeing double (diplopia), painful or difficult swallowing, and slurred speech or loss of voice, which are all indications of the exotoxin's effect on nerve functions. The exotoxin may also cause severe swelling in the neck ("bull neck").
The signs and symptoms of diphtheria vary according to the location of the infection. Nasal diphtheria produces few symptoms other than a watery or bloody discharge. On examination, there may be a small visible membrane in the nasal passages. Nasal infection rarely causes complications by itself, but it is a public health problem because it spreads the disease more rapidly than other forms of diphtheria.
Pharyngeal diphtheria gets its name from the pharynx, which is the part of the upper throat that connects the mouth and nasal passages with the larynx. This is the most common form of diphtheria, causing the characteristic throat membrane. The membrane often bleeds if it is scraped or cut. It is important not to try to remove the membrane because the trauma may increase the body's absorption of the exotoxin. Other signs and symptoms of pharyngeal diphtheria include mild sore throat, fever of 10102°F (38.38.9°C), a rapid pulse, and general body weakness.
Laryngeal diphtheria, which involves the voice box or larynx, is the form most likely to produce serious complications. The fever is usually higher in this form of diphtheria (10304°F or 39.40°C) and the patient is very weak. Patients may have a severe cough, have difficulty breathing, or lose their voice completely. The development of a "bull neck" indicates a high level of exotoxin in the bloodstream. Obstruction of the airway may result in respiratory compromise and death.
The skin form of diphtheria, which is sometimes called cutaneous diphtheria, accounts for about 33% of diphtheria cases. It is found chiefly among people with poor hygiene. Any break in the skin can become infected with diphtheria. The infected tissue develops an ulcerated area and a diphtheria membrane may form over the wound but is not always present. The wound or ulcer is slow to heal and may be numb or insensitive when touched.
The diagnosis of diphtheria can be confirmed by the results of a culture obtained from the infected area. Material from the swab is put on a microscope slide and stained using a procedure called Gram's stain. The diphtheria bacillus is called Gram-positive because it holds the dye after the slide is rinsed with alcohol. Under the microscope, diphtheria bacilli look like beaded rod-shaped cells, grouped in patterns that resemble Chinese characters. Another laboratory test involves growing the diphtheria bacillus on Loeffler's medium.
The most important treatment is prompt administration of diphtheria antitoxin. The antitoxin is made from horse serum and works by neutralizing any circulating exotoxin. The physician must first test the patient for sensitivity to animal serum. Patients who are sensitive (about 10%) must be desensitized with diluted antitoxin, since the antitoxin is the only specific substance that will counteract diphtheria exotoxin. No human antitoxin is available for the treatment of diphtheria.
Antibiotics are given to wipe out the bacteria, to prevent the spread of the disease, and to protect the patient from developing pneumonia. They are not a substitute for treatment with antitoxin. Both adults and children may be given penicillin, ampicillin, or erythromycin. Erythromycin appears to be more effective than penicillin in treating people who are carriers because of better penetration into the infected area. Cutaneous diphtheria is usually treated by cleansing the wound thoroughly with soap and water, and giving the patient antibiotics for 10 days.
Universal immunization is the most effective means of preventing diphtheria. The standard course of immunization for healthy children is three doses of DPT (diphtheria-tetanuspertussis) preparation given between two months and six months of age, with booster doses given at 18 months and at entry into school. Adults should be immunized at 10-year intervals with Td (tetanus-diphtheria) toxoid. A toxoid is a bacterial toxin that is treated to make it harmless but still can induce immunity to the disease.
Diphtheria patients must be isolated for one to seven days or until two successive cultures show that they are no longer contagious. Because diphtheria is highly contagious and has a short incubation period, family members and other contacts of diphtheria patients must be watched for symptoms and tested to see if they are carriers. They are usually given antibiotics for seven days and a booster shot of diphtheria/tetanus toxoid.
Reporting is necessary to track potential epidemics, to help doctors identify the specific strain of diphtheria, and to see if resistance to penicillin or erythromycin has developed. In 1990, an outbreak of diphtheria began in Russia and spread within four years to all of the newly independent states of the former Soviet Union. By the time that the epidemic was contained, over 150,000 cases and 5000 deaths were reported. A vast public health immunization campaign largely confined the epidemic by 1999.
See also Bacteria and bacterial infection; Epidemics, bacterial; Public health, current issues
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