Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two children with congenital heart disease developed persistent fever, anemia, and hepatosplenomegaly. Both were shown to have intracardiac vegetations and evidence of infection with Coxiella burnetti. Thus, the same clinical manifestations of Q fever may develop in both children and adults.
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PMID:Chronic Q fever endocarditis with massive splenomegaly in childhood. 395 26

Antimyolemmal antibodies can be demonstrated in sera of patients with coxsackie B, influenza, mumps and Q-fever perimyocarditis, in sera of patients with postpericardiotomy and postinfarction syndromes, in part of the sera of patients with endocarditis and in some patients with dilated heart disease most likely due to secondary immunopathogenesis after perimyocarditis. Antimyolemmal antibodies in titres greater than 1: 40 are complement fixing and cytolytic when added to cultures of vital myocytes. In vitro cardiocytolysis indicates that humoral effector mechanisms could also play a pathogenetic role in vivo. In vitro antibody dependent and independent cellular cytotoxicity of patients lymphocytes against isolated cardiocytes could not be observed in perimyocarditis and postmyocarditic cardiomyopathy. It could be demonstrated, however, in patients with postpericardiotomy syndrome and in some patients with dilated cardiomyopathies. Immunological investigations are therefore not only of diagnostic significance but have widened our knowledge of the etiology and pathogenesis of perimyocardial diseases. Furthermore they are helpful in the follow-up and prognosis of patients with protracted perimyocardial affections.
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PMID:[Secondary immunopathogenesis of cardiac diseases]. 637

Sixteen patients with serologically proven Q-fever infection are reviewed. Fifteen had significantly raised antibody titres to both phase I and phase II Coxiella burneti antigens, indicating persistent or chronic infection. One patient, a premature infant who died, had raised phase II titres only, but is included together with the mother who had chronic Q-fever and was the presumed source of infection. Chronic Q-fever infection has previously been regarded as virtually synonymous with Q-fever endocarditis, but only seven of the patients in this survey had evidence of valvular endocarditis. In those who did, the infection had arisen on prosthetic valves or those affected by rheumatic or syphilitic heart disease. One patient had inexorably progressive destruction of an infected congenitally bicuspid aortic valve. Eight patients had infections associated with extra-valvular sites and several of these associations have not been previously described. These include extreme prematurity with perinatal death, possibly following transplacental infection, the sudden infant death syndrome (SIDS), multiple lower limb emboli from endocarditis of an abdominal aortic dacron graft, and colonization of ventricular endocardium following left ventricular myotomy/sub-aortic diaphragm resection. The current concept that chronic Q-fever is invariably associated with endocarditis is therefore untenable and the indications for phase I antibody screening should be extended to include patients other than those under investigation for 'culture-negative' endocarditis, for example those with unusual osteomyelitis of vertebrae.
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PMID:Chronic or fatal Q-fever infection: a review of 16 patients seen in North-East Scotland (1967-80). 687 20

Q fever is a widespread disease caused by the rickettsia Coxiella burnetii, an obligate intracellular bacteria which man usually acquires through the inhalation of infected dust from subclinically infected animals. Q fever may be acute or chronic. The chronic form mostly presents as endocarditis, which is difficult to diagnose and may ultimately be fatal. Immunocompromised conditions and underlying heart disease are the most important risk factors to consider in cases of Q fever endocarditis. The ultimate diagnosis is based on specific diagnostic tests which include serology, demonstration of C. burnetii in valvular material, isolation of C. burnetii from blood and tissue samples by cell-culture techniques as well as amplification and detection of the bacterial DNA by polymerase chain reaction. Treatment of chronic Q fever endocarditis is complex and requires long-term antibiotic therapy, sometimes associated with heart valve replacement. At the present time neither an optimal antibiotic combination nor the duration of treatment is known and patients with Q fever endocarditis require prolonged follow-up because of the possibility of later relapses.
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PMID:Q fever endocarditis. 924 58

Q fever is an important zoonosis caused by the rickettsial organism Coxiella burnetii, which can result in life threatening illness, especially in those with an underlying cardiac defect. C. burnetii infections in England and Wales reported to the PHLS Communicable Disease Surveillance Centre between 1984 and 1994 were reviewed. A total of 1117 cases were reported, a third of which came from the South Western region. The annual totals fell over this period. The mean age of cases was 45 years, and 74% were men. Reports peaked in the month of May. Contact with animals, mainly cattle and sheep, was reported in 60 cases. Occupationally acquired infection was reported for 24 cases including abattoir workers, farmers, veterinary surgeons, hide handlers, and butchers. Forty-seven per cent of cases presented with respiratory symptoms, 7% with heart disease, and 5% with hepatitis. Seven per cent of cases reported travel abroad before becoming ill. Joint veterinary and medical investigations should be undertaken to establish the natural history of C. burnetii infection in England and Wales and formulate policies to prevent acute and chronic infections.
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PMID:Epidemiological features of Coxiella burnetii infection in England and Wales: 1984 to 1994. 881 Jan 19

The worldwide epidemiology and population-based incidence of Q fever endocarditis (QFE) have been less well studied than those for uncomplicated Q fever. An exhaustive literature review revealed 408 patients with QFE reported between 1949 and 1994, mostly from 3 large geographic areas. Underlying valvular heart disease was almost invariably present, and 38% had prosthetic valves. The most common clinical manifestations were fever and congestive heart failure. The mortality rate dropped over the years from 65% to 25%, but a meta-analysis of published data showed the death rate to be significantly lower among patients receiving combination therapy (12/65, 18%), as compared to patients treated with tetracycline alone (18/41, 44%, p = 0.005). A 10-year (1983-1992) retrospective nationwide survey of QFE in Israel revealed 35 patients with QFE, representing an annual incidence of 0.75 cases per 1 million population. Underlying heart disease, clinical manifestations and outcome in the Israeli group were not substantially different from those described in the world literature. The current state-of-the-art clinical approach includes early diagnosis, prompt initiation of combination therapy for at least 3 years, and long-term clinical and serologic follow-up. Adherence to these rules might have contributed to the improved prognosis in recent years.
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PMID:Q fever endocarditis in Israel and a worldwide review. 911 97

Q fever is caused by the rickettsia Coxiella burnetti, an obligate intracellular bacterium acquired by inhalation of infected dust from subclinically infected animals. Q fever may be acute or chronic; the chronic form mostly presents as endocarditis. Immunocompromised states and underlying heart disease are the most important risk factors. Usually the symptoms of Q fever endocarditis are nonspecific and diagnosis is often established very late. New criteria for diagnosis include a single blood culture positive for Coxiella burnetti, positive Q fever serology and characteristic echocardiographic studies. We describe a 49-year-old man with bicuspid aortic valve admitted with fever, weight loss and a new heart murmur. The diagnosis of Q fever endocarditis was established by positive Q fever serology, and an echocardiogram showing vegetations and valvular dysfunction. This case suggests that Q fever endocarditis should be considered in patients with "sterile" endocarditis.
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PMID:[Q fever endocarditis and bicuspid aortic valve]. 941 57

The first case of Q fever endocarditis that has been diagnosed in Mexico is presented. A 10-year-old girl with discrete subaortic stenosis (SAS) and patent ductus arteriosus (PDA) was seen in December of 1996 with fever, hepatomegaly and splenomegaly. She presented also anemia, leukopenia, hypergammaglobulinemia, positive rheumatoid factor, cryoglobulinemia, antinuclear and anticytoplasmic antibodies (anti-RNA-proteins and anti-DNA). An aortic valve vegetation was seen by echocardiogram. Blood-cultures were negative. Antibody test for Coxiella burnetii was positive. Treatment with doxicyclin was initiated as soon the diagnosis was done. PDA was closed, SAS was liberated and two aortic vegetations were resected. Endocarditis in Q fever occurs when there is predisposing heart disease and/or immunodeficiency. Effective therapy has not yet been established. The diagnosis of Q fever endocarditis is difficult; it should be considered, in case of clinical suspicion of endocarditis with negative blood-cultures.
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PMID:[Coxiella burnetii endocarditis. A report of the first case diagnosed in Mexico]. 981 Mar 69

Infections due to Coxiella burnetii, the causative agent of Q fever, are uncommon in the United States. Cases of chronic Q fever are extremely rare and most often manifest as culture-negative endocarditis in patients with underlying valvular heart disease. We describe a 31-year-old farmer from West Virginia with a history of congenital heart disease and recurrent fevers for 14 months who was diagnosed with Q fever endocarditis based on an extremely high antibody titer against Coxiella burnetii phase I antigen. Despite treatment with doxycycline, he continued to have markedly elevated Coxiella burnetii phase I antibody titers for 10 years after the initial diagnosis. To our knowledge, this case represents the longest follow-up period for a patient with chronic Q fever in the United States. We review all cases of chronic Q fever reported in the United States and discuss important issues pertaining to epidemiology, diagnosis, and management of this disease.
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PMID:Chronic Q fever in the United States. 1675 41

Coxiella burnetii endocarditis is a rare diagnosis in children. We present a case of Q fever endocarditis due to a new genotype, MST 54, and review recent literature on Q fever infections in children. Practitioners should consider Q fever in culture-negative endocarditis, particularly in children with congenital heart disease and history of travel or residence in endemic regions.
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PMID:Coxiella burnetii Endocarditis in a Child Caused by a New Genotype. 2653 79


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