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1/11. Homozygous deletion of the CYP21A-TNXA-RP2-C4B gene region conferring C4B deficiency associated with recurrent respiratory infections.

    The central class III region of the human major histocompatibility complex contains highly polymorphic genes that are associated with immune disorders and may serve as susceptibility factors for viral infections. Many HLA haplotype specific rearrangements, duplications, conversions and deletions, occur frequently in the C4 gene region. Genetic deficiencies of complement components are associated with recurrent occurrence of bacterial infections. We have studied the complement profile and the class III genes 5'-RP1-C4A-CYP21A-TNXA-RP2-C4B-CYP21B-TNXB -3' in a 4-year-old Caucasian patient. He has suffered from several pneumonias caused by respiratory viruses, eight acute otitis media, prolonged respiratory infections and urinary tract infection. complement c4 was constantly low, but the other complement components, from C1 to C9, C1INH, factor B and properdin, were within normal limits. Immunological evaluation gave normal lymphocyte numbers and functions with the exception of subnormal T cell response to pokeweed mitogen. Molecular studies of the C4 gene region in the patient revealed homozygous deletion of CYP21A-TNXA-RP2-C4B generating total deficiency of C4B and the flanking 5' region up to C4A, and in the father a missing CYP21A gene. Further investigations are needed to elucidate the relationship between C4B deficiency and susceptibility to infections.
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ranking = 1
keywords = bacterial infection
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2/11. Polymorphic light eruption occurring in common variable hypogammaglobulinaemia, and resolving with intravenous immunoglobulin therapy.

    A 55-year-old woman with a past history of lower respiratory tract infections presented with a photosensitive eruption. Polymorphic light eruption (PLE) was diagnosed on the basis of the temporal relationship to sun exposure and the diagnosis was supported by positive monochromator irradiation tests in the ultraviolet A wavelength spectrum. Investigation of the patient's immune status identified low levels of all immunoglobulin (Ig) subtypes consistent with common variable hypogammaglobulinaemia. Intravenous Ig replacement therapy, instituted to minimize risks from bacterial infections, was commenced and over the ensuing months resulted in a complete resolution of the PLE. PLE is considered to represent a type IV hypersensitivity reaction directed against a cutaneous autoantigen induced by exposure to ultraviolet light. In PLE, nonspecific immunomodulatory mechanisms of intravenous Ig may be active, such as a reduction in the synthesis of cytokines and a blockage of the IgG Fc receptors on macrophages.
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ranking = 1
keywords = bacterial infection
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3/11. Novel bacterium isolated from a lung transplant patient with cystic fibrosis.

    The major clinical problem for patients with cystic fibrosis (CF) is progressive loss of pulmonary function, usually due to chronic bacterial infections. A patient with CF and a lung transplant was severely infected with a previously unidentified gram-negative bacterium. We isolated this organism (strain DS15158) from the patient and characterized it by phylogenetic analysis of the small-subunit rRNA and biochemically by the BIOLOG GN MicroPlate assay, fatty acid analysis, and various standard laboratory tests. No close match to any other organism could be found. Isolate DS15158 represents a new genus-level divergence within the bacterial subdivision alpha-proteobacteria on the basis of the 16S rRNA gene analysis.
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ranking = 1
keywords = bacterial infection
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4/11. Open-label, multicentre, emergency-use study of clinafloxacin (CI-960) in the treatment of patients with serious life-threatening infections.

    In an open-label emergency-use study, 23 patients with bacterial infections caused by multiply drug resistant pathogens were treated with clinafloxacin. Efficacy and safety were evaluated by tabulating investigators' assessments at the end of treatment, treatment discontinuations and adverse event data. Most of the patients were seriously ill and had multi-organ infections, primarily respiratory tract infections such as nosocomial pneumonia and gastrointestinal infections. Eleven patients were successfully treated, two had treatment failure and 10 were not evaluable because the patients died of their underlying disease. Considering that most of the patients had several infections caused by multiply resistant pathogens, clinafloxacin may be useful for the treatment of such life-threatening episodes.
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ranking = 1
keywords = bacterial infection
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5/11. Fatal pulmonary mycobacterium abscessus infection in a patient using etanercept.

    A case of fatal pulmonary mycobacterium abscessus infection in a 56-year-old man is reported. The patient had a longstanding history of seropositive, nodular rheumatoid arthritis with severe joint manifestations that had been treated with a regimen of prednisone, leflunomide, and etanercept. He presented to our facility with complaint of productive cough, persistent fevers, pleuritic chest discomfort, and dyspnea at rest. The patient was admitted to hospital, placed in isolation, a left-sided chest tube was inserted (left pneumothorax identified), and sputum acid-fast bacteria stains and cultures were obtained. Fluorochrome stains demonstrated numerous acid-fast bacteria, and M. abscessus was recovered from the culture media. He was treated with a regimen of amikacin, cefoxitin, and clarithromycin. He initially responded well, and was discharged home with this regimen. He remained afebrile with decreased cough and sputum production until 15 days after discharge when he was again admitted to hospital, with acute onset dyspnea and right-sided chest discomfort (right pneumothorax identified). He ultimately expired, due to overwhelming pulmonary infection, 20 days after readmission to hospital. autopsy revealed acid fast bacilli in the setting of numerous, bilateral, necrotic, granulomatous, cavitary pulmonary lesions. Based on its mechanism of action, we propose an association between the use of etanercept, a tumor necrosis factor alpha (TNF-alpha) inhibitor, and this case of fatal pulmonary mycobacterial infection. We recommend that physicians exercise cautious clinical judgment when initiating etanercept therapy in persons with underlying lung disease, especially in communities in which mycobacterial organisms are highly prevalent. We also advise physicians to maintain a high level of vigilance for late onset granulomatous infection in persons using etanercept.
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ranking = 1
keywords = bacterial infection
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6/11. Primary care challenges in the management of respiratory tract infections.

    Community-acquired respiratory tract infections (RTIs), such as community-acquired pneumonia, acute exacerbations of chronic bronchitis, and acute rhinosinusitis, are among the most common reasons that adults seek care from their primary health care providers in the united states. High levels of pneumococcal resistance have developed, particularly to macrolides, since the 1990s. Empiric management of these infections, the difficulty in differentiating bacterial from nonbacterial infection, and a high level of patient expectation of antimicrobial therapy contribute to the overuse of antibiotics. The primary care clinician must judiciously weigh various factors in order to prescribe appropriate and effective treatment.
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ranking = 1
keywords = bacterial infection
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7/11. Bacterial tracheitis: report of eight new cases and review.

    Bacterial tracheitis, previously referred to as nondiphtheritic laryngitis with marked exudate, was commonly discussed in pediatric textbooks before 1940. It seemed to disappear as a clinical entity after that time, but it has been recorded with increasing frequency in the pediatric literature since 1979. We describe eight new cases and review 110 previously described cases. The clinical course consists of a prodromal upper respiratory illness with stridor, fever, and a variable degree of respiratory distress. Unlike patients with croup, patients with bacterial tracheitis do not respond to aerosolized racemic epinephrine. Most patients require endotracheal intubation; some require tracheostomy. Reported complications include pneumonia, pneumothorax, formation of pseudomembranes, toxic shock syndrome, and cardiopulmonary arrest. Bacterial tracheitis is a secondary bacterial infection following a primary viral respiratory infection. The most common preceding viral infection is parainfluenza. staphylococcus aureus and haemophilus influenzae are the predominant causes of bacterial tracheitis. Secondary bacterial infection may occur as a result of tracheal mucosal injury or impairment of normal phagocytic function due to viral infection.
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ranking = 2
keywords = bacterial infection
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8/11. Functional deficiency of complement factor d in a monozygous twin.

    An adult twin with recurrent bacterial infections was found to have a partial functional deficiency of complement factor d. Full restoration of alternative pathway activity and zymosan- or cobra venom factor-induced consumption of C3 and B was found after reconstitution of patient's serum with purified D. family studies revealed normal D levels in the mother, a brother and another sister. After gel filtration of patient's sera only little D activity could be detected in the fractions, and trypsin activation of the fractions also did not uncover detectable precursor D activity.
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ranking = 1
keywords = bacterial infection
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9/11. Infection, dermatitis, increased IgE, and impaired neutrophil chemotaxis. A possible relationship.

    Eleven patients with recurrent bacterial infections were found to have impaired neutrophil chemotaxis. The infections were primarily staphylococcal abscesses involving the skin and the respiratory tract. All patients had chronic eczematour dermatitis and an elevated level of serum IgE. The peripheral blood eosinophil count was equal to or greater than 10% of the circulating leukocytes in 10 of 11 patients. Associated findings in some patients included urticaria and oral or cutaneous candida infection. These observations suggest a relationship between chronic dermatitis, elevated serum IgE level, eosinophilia, defective neutrophil chemotactic responsiveness, and recurrent pyogenic infections of skin and lungs.
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ranking = 1
keywords = bacterial infection
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10/11. Disseminated strongyloides stercoralis infection.

    A case of disseminated S stercoralis is an immunosuppressed patient manifested with diarrhea, a rash, and progressive respiratory insufficiency. The parasites were eradicated with thiabendazole despite continued steroid therapy, and the patient survived the hospitalization. The characteristics of S stercoralis allow it to be harbored within a host for prolonged periods of time, only to disseminate once cell-mediated immunity is suppressed. A diagnosis of strongyloidiasis should be considered in an immunocompromised patient with a petechial rash. Prompt diagnosis and initiation of thiabendazole therapy provides the greatest opportunity for patient survival. Secondary bacterial infections should be aggressively sought. mortality from disseminated strongyloidiasis approaches 80%.
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ranking = 1
keywords = bacterial infection
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