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1/552. risk factors for meningococcal disease in Cape Town.

    OBJECTIVE: To determine the risk factors associated with meningococcal disease among children living in Cape Town. DESIGN: A case-control study was conducted from October 1993 to January 1995. SETTING: The study population consisted of all children under the age of 14 years who were resident in the Cape Town metropolitan region. Cases and controls were selected from red cross war Memorial Children's Hospital. RESULTS: A total of 70 cases and 210 controls were interviewed. Significant risk factors for meningococcal disease included being breast-fed for less than 3 months (adjusted odds ratio (OR) 2.4); overcrowding (adjusted OR 2.3); and age less than 4 years (adjusted OR 2.3). Exposure to two or more household members who smoked was also a risk factor, but only in the presence of a recent upper respiratory tract infection (adjusted OR 5.0). CONCLUSION: This is the first case-control study in south africa examining risk factors for meningococcal disease. It provides further evidence for reduction of smoking, reduction of overcrowding and promotion of breast-feeding as important public health measures. ( info)

2/552. inhalation anthrax in a home craftsman.

    inhalation anthrax with complicating subarachnoid hemorrhage due to simultaneous infection with two capsular biotypes of bacillus anthracis of different virulence for the mouse is reported. The patient, a home craftsman, acquired his infection from imported animal-origin yarn. ( info)

3/552. An unusual case of subluxation of C.3-C.4.

    A case is reported of spontaneous subluxation of C.3 on C.4 in a 6-year-old boy due to an upper respiratory infection after surgical treatment for muscular torticollis. ( info)

4/552. torsades de pointes ventricular tachycardia induced by clarithromycin and disopyramide in the presence of hypokalemia.

    We report a 76-year-old woman who developed TdP ventricular tachycardia induced by combined use of clarithromycin and disopyramide. She had a history of myocardial infarction 5 years earlier and has taken disopyramide for supraventricular arrhythmias. In addition, she had taken clarithromycin for upper respiratory tract infection. On admission, an ECG showed prolongation of QTc interval to 0.71 seconds and self-terminating TdP occurred several times. disopyramide was metabolized by the cytochrome enzyme CYP3A4 and clarithromycin competitively inhibits this enzyme, probably resulting in an increase in plasma concentration of disopyramide. We should consider this possibility when prescribing clarithromycin in combination with antiarrhythmic agent disopyramide. ( info)

5/552. pseudomonas putrefaciens from clinical material.

    Three strains of pseudomonas putrefaciens were isolated from routine clinical specimens at the University Hospital, Kuala Lumpur, malaysia. Their cultural and biochemical characteristic, and antibiotic susceptibilities are presented. Characteristics of diagnostic value were stressed. Two isolates appeared to have played a pathogenic role in chronic otitis media. ( info)

6/552. Upper respiratory problems in the yellow nail syndrome.

    A case of the yellow nail syndrome is described. The characteristic nail changes were associated with pleural effusions, lymphoedema of the face and legs and upper respiratory symptoms. The finding of extremely hard ear wax has not been reported previously. A satisfactory response to combination diuretic therapy was seen. The case of recognition of the Syndrome is emphasized together with the danger of excessive investigation if the nails are not inspected. Possible mechanisms for the formation of extremely hard cerumen are discussed. ( info)

7/552. Postanginal septicaemia with external jugular venous thrombosis: case report.

    Postanginal septicaemia is a syndrome of anaerobic septicaemia, septic thrombophlebitis of the internal jugular vein, and metastatic infections, that follows a localized infection in the area drained by the large cervical veins. The syndrome was well-known and often fatal in the preantibiotic era. It is now rather rare, presumably as a result of the almost routine use of prophylactic antibiotics. The symptoms are classic, and it should be suspected in any case where septicaemia and metastatic lesions are preceded by a head and neck infection. We report a case that is typical, except that branches of the external jugular vein were thrombosed. To our knowledge this has not been reported previously. ( info)

8/552. XLMR syndrome characterized by multiple respiratory infections, hypertelorism, severe CNS deterioration and early death localizes to distal Xq28.

    We report on a family with severe X-linked mental retardation (XLMR) and progressive, severe central nervous system deterioration. Three of the five affected males died of secondary complications before the age of 10 years and none have survived past the age of 10. These complications included swallowing dysfunction and gastroesophageal reflux with secondary recurrent respiratory infections. In addition, hypotonia and a mild myopathy were also present. All had a characteristic facies, including downslanting palpebral fissures, hypertelorism, and a short nose with a low nasal bridge. The two older boys showed cerebral atrophy by CT. No metabolic abnormalities were identified. Three obligate carriers had an IQ less than 80. The causal gene has been localized distal to DXS8103 in Xq28, a region spanning 5cM. No other XLMR disorder with these manifestations have been localized to this region and this appears to be a new disorder. ( info)

9/552. De novo complete trisomy 5p: clinical report and FISH studies.

    We describe a de novo trisomy 5p in a 1-year-old severely retarded boy. The complete short arm of chromosome 5 segregated as an additional marker chromosome in all metaphases. The marker was identified as 5p by conventional cytogenetic techniques (GTG, GBG, CBG) and molecular cytogenetic techniques (whole chromosome-painting probe, probes for the cri-du-chat region and the centromere, and additionally high-resolution multicolor banding using a chromosome 5-specific dna probe cocktail). The clinical findings were similar to the established trisomy 5p phenotype including macrocephaly, facial abnormalities, tracheobronchial defects with subsequent respiratory infections, hypotonia, and psychomotor retardation. To the best of our knowledge this is the first description of an isolated complete 5p trisomy without involvement of the aberrant chromosome in any structural chromosomal rearrangements. ( info)

10/552. 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. ( info)
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