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Pertussis is an acute infection of the respiratory tract caused by Bordetella pertussis. The word pertussis means “violent cough,” which aptly describes the most consistent and prominent feature of the illness. The inspiratory sound made at the end of an episode of paroxysmal coughing gives rise to the common name for the illness, “whooping cough.” However, this feature is variable: it is uncommon among infants ≤6 months of age and is frequently absent in older children and adults. The Chinese name for pertussis is “the 100-day cough,” which accurately describes the clinical course of the illness. The identification of B. pertussis was first reported by Bordet and Gengou in 1906, and vaccines were produced over the following two decades.
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Of the 10 identified species in the genus Bordetella, only four are of major medical significance. B. pertussis infects only humans and is the most important Bordetella species causing human disease. B. parapertussis causes an illness in humans that is similar to pertussis but is typically milder; co-infections with B. parapertussis and B. pertussis have been documented. With improved polymerase chain reaction (PCR) diagnostic methodology, up to 20% of patients with a pertussis-like syndrome have been found to be infected with B. holmesii, formerly thought to be an unusual cause of bacteremia. B. bronchiseptica is an important pathogen of domestic animals that causes kennel cough in dogs, atrophic rhinitis and pneumonia in pigs, and pneumonia in cats. Both respiratory infection and opportunistic infection due to B. bronchiseptica are reported occasionally in humans. B. petrii, B. hinzii, and B. ansorpii have been isolated from patients who are immunocompromised.
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Bordetella species are gram-negative pleomorphic aerobic bacilli that share common genotypic characteristics. B. pertussis and B. parapertussis are the most similar of the species, but B. parapertussis does not express the gene coding for pertussis toxin. B. pertussis is a slow-growing fastidious organism that requires selective medium and forms small, glistening, bifurcated colonies. Suspicious colonies are presumptively identified as B. pertussis by direct fluorescent antibody testing or by agglutination with species-specific antiserum. B. pertussis is further differentiated from other Bordetella species by biochemical and motility characteristics.
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B. pertussis produces a wide array of toxins and biologically active products that are important in its pathogenesis and in immunity. Most of these virulence factors are under the control of a single genetic locus that regulates their production, resulting in antigenic modulation and phase variation. Although these processes occur both in vitro and in vivo, their importance in the pathobiology of the organism is unknown; they may play a role in intracellular persistence and person-to-person spread. The organism’s most important virulence factor is pertussis toxin, which is composed of a B oligomer–binding subunit and an enzymatically active A protomer that ADP-ribosylates a guanine nucleotide–binding regulatory protein (G protein) in target cells, producing a variety of biologic effects. ...