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ESSENTIALS OF DIAGNOSIS
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Exposure to sheep, goats, cattle, or their products is common; some infections are laboratory acquired An acute or chronic febrile illness with severe headache, cough, prostration, and abdominal pain Extensive pneumonitis, hepatitis, or encephalopathy; rarely, endocarditis or chronic fatigue syndrome
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Coxiella burnetii is unique among rickettsiae in that it is usually transmitted to humans not by arthropods but by inhalation or ingestion It is distributed worldwide with few exceptions Coxiella infections occur mostly in cattle, sheep, and goats, in which they cause mild or subclinical disease (Table 32 3) Transmission by cows and goats is principally through the milk and placenta and by sheep through feces, placenta, and milk Dry feces and milk, dust contaminated with them, and the tissues of these animals contain large numbers of infectious organisms that are spread by the airborne route Inhalation of contaminated dust and of droplets from infected animal tissues is the main source of human infection Outbreaks have been described in association with parturient cats There is an occupational risk for animal handlers, slaughterhouse workers, veterinarians, laboratory workers, and other workers exposed to animal products The route of acquisition appears to determine the main clinical syndrome Endocarditis is an uncommon but serious form of Coxiella infection and has been linked with preexisting valvular conditions, immunocompromise, urban residence, and raw milk ingestion Coxiella is resistant to heat and drying, perhaps because the organism forms endospore-like structures Thus, it survives in dust, on the fleece of infected animals, or in inadequately pasteurized milk Spread from one human to another does not seem to occur even in the presence of florid pneumonitis, but maternal fetal infection can occur
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Diagnosis is made by the history of tick exposure followed by a clinical illness with the characteristic symptoms and signs An indirect fluorescent antibody assay is available through the CDC and requires acute and convalescent sera A PCR assay applied to whole blood samples is a rapid diagnostic tool, if available Treatment for both forms of ehrlichiosis is with doxycycline, 200 mg orally or intravenously for at least 10 days or until 3 days of defervescence Treatment should not be withheld while awaiting confirmatory serology when suspicion is high Lack of clinical improvement and defervescence 48 hours after doxycycline initiation suggests an alternate diagnosis
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Demma LJ et al Epidemiology of human ehrlichiosis and anaplasmosis in the United States, 2001 2002 Am J Trop Med Hyg 2005 Aug;73(2):400 9 [PMID: 16103612] Stone JH et al Human monocytic ehrlichiosis JAMA 2004 Nov 10;292(18):2263 70 [PMID: 15536115]
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A Symptoms and Signs
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After an incubation period of 1 3 weeks, a febrile illness develops with headache, prostration, and muscle pains, often with a nonproductive cough Pneumonia is the predominant manifestation of acute Q fever, although granulomatous hepatitis and CNS manifestations may occur The most common manifestation of chronic Q fever is culture-negative endocarditis, which occurs in less than 1% of infected individuals It is
Viral & Rickettsial Infections
found mainly in the setting of preexisting valve disease Uncommon manifestations of Coxiella infection include myocarditis, encephalitis, aortic aneurysm, hemolytic anemia, orchitis, acute renal failure, and mediastinal lymphadenopathy Reactivation of Q fever in pregnant women may cause abortions The clinical course may be acute or may be chronic and relapsing A Q fever chronic fatigue syndrome is thought by some experts to involve bacteremic shedding from bone marrow reservoirs and to be immunogenetically determined
CMDT 2008
B Laboratory Findings
Laboratory examination during the acute phase may show elevated liver function tests and occasionally leukocytosis Patients with acute Q fever usually produce antibodies to C burnetii phase II antigen A fourfold rise between acute and convalescent sera by indirect immunofluorescence is diagnostic The diagnosis of Q fever endocarditis is made serologically The IgG titer is usually 1:200 or greater and is directed against phase I antigen Sometimes the diagnosis is not made until the time of valve replacement, with PCR of tissue samples Isolation of C burnetii from affected valves is also possible using the shell-vial technique, but the organism is highly transmissible to laboratory workers
untreated patients, the mortality rate is usually low, except when endocarditis develops (see below) Although the optimal regimen for the treatment of endocarditis is not known, most experts recommend a combination of oral doxycycline (200 mg/d) plus oral hydroxychloroquine (600 mg/d), usually for at least 2 years Serologic responses can be monitored to determine cessation of therapy Doxycycline levels correlate with declines in phase 1 antibody levels Heart valve replacement may be necessary in refractory disease Given the difficulty in treating endocarditis, the same combination therapy for 1 year is recommended for patients with acute disease and underlying valvular heart disease
Arashima Y et al Improvement of chronic nonspecific symptoms by long-term minocycline treatment in Japanese patients with Coxiella burnetii infection considered to have post-Q fever fatigue syndrome Intern Med 2004 Jan;43(1):49 54 [PMID: 14964579] Mcquiston JH et al National surveillance and the epidemiology of human Q fever in the United States, 1978 2004 Am J Trop Med Hyg 2006 Jul;75(1):36 40 [PMID: 16837706] Marmion BP et al Long-term persistence of Coxiella burnetii after acute primary Q fever QJM 2005 Jan;98(1):7 20 [PMID: 15625349] Raoult D et al Natural history and pathophysiology of Q fever Lancet Infect Dis 2005 Apr;5(4):219 26 [PMID: 15792739] Tissot-Dupont H et al Wind in November, Q fever in December Emerg Infect Dis 2004 Jul;10(7):1264 9 [PMID: 15324547]
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