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1/55. 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.
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2/55. 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.
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ranking = 68.046744609365
keywords = family
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3/55. The clinical course of multifocal atrial tachycardia in infants and children.

    OBJECTIVES: This study outlines the clinical course, treatment and the late outcome of infants and children with multifocal atrial tachycardia (MAT). BACKGROUND: Multifocal atrial tachycardia is defined by three distinct P-waveforms, irregular P-P intervals, isoelectric baseline between P-waves and rapid rate on an electrocardiogram. Several smaller prior reports have described pediatric patients with MAT, but their long-term outcome has not been fully assessed. methods: The clinical records, echocardiograms and long-term follow-up of patients with MAT were reviewed and compared to previous reports of MAT. RESULTS: Fourteen boys and seven girls (median age 1.8 months) presented with MAT. At diagnosis, six patients had respiratory illness, of whom two were critical. Ten were asymptomatic. Seven patients had structural heart disease (SHD), one of whom died. Four of 15 patients (27%) with echocardiograms had diminished ventricular function. Ventricular rates were 111 to 253 beats/min (mean 181 beats/min). Median duration of the arrhythmia was 4.9 months (mean 6.7 months). Electrical cardioversion was attempted in 4 patients without success and 15 patients received antiarrhythmic medication. Seventeen patients were followed for a mean of 60 months. Four patients were lost to follow-up. There were no late arrhythmias. CONCLUSIONS: The majority of children with MAT are healthy infants under one year of age; a few may exhibit mild to life-threatening cardiorespiratory disease. Less often, MAT accompanies SHD. Mild ventricular dysfunction may be observed in the presence of MAT, but symptoms are few and resolution is generally complete. Response to antiarrhythmic agents is mixed, and cardioversion is of no avail. Finally, long-term cardiovascular and developmental outcome depends principally on underlying condition; for otherwise healthy children, it is excellent.
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ranking = 0.42862978930026
keywords = life
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4/55. Successful treatment of pseudomonas aeruginosa respiratory tract infection with a sugar solution--a case report on a lectin based therapeutic principle.

    BACKGROUND: Airway infections with pseudomonas aeruginosa often represent a life-threatening event in immuno-compromised patients or patients with cystic fibrosis. The adhesion of this bacterium to surfaces such as the airway epithelium is mediated by two lectins, sugar binding proteins. In addition to their adhesive properties, these lectins have been shown to stop human ciliary beating thus compromising the mucociliary clearance as an important non-specific defence mechanism of the airways. Inhibition of these lectins by their specific sugars galactose and fucose, respectively, could therefore be of benefit in the elimination therapy of P. aeruginosa. CASE REPORT: An infant suffering from P. aeruginosa airway infection after chemotherapy for neuroblastoma, which could not successfully be treated by antibiotics, was subjected to a series of additional galactose/fucose inhalations, which eliminated the germ as evidenced by microbiological testing. This is the first report suggesting the effectiveness of a lectin-based therapeutic principle in P. aeruginosa airway infection. CONCLUSION: The competitive inhibition of P. aeruginosa lectins by the lectin specific sugars galactose and fucose may overcome particular mechanisms of bacterial resistance in patients with P. aeruginosa airway infection. This underlying biochemical mechanism and the outcome of our patient suggest a clinical benefit of this novel therapeutic approach for immunocompromised patients or patients with cystic fibrosis suffering from infection with P. aeruginosa.
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keywords = life
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5/55. death in Antarctica.

    Antarctic tourism is flourishing, but Antarctic cruises are often more physically demanding than typical "tropical" cruises. An 82-year-old Antarctic tourist died of probable septic shock secondary to lower respiratory tract infection six days after sustaining a suspected vertebral fracture in a minor fall from an inflatable boat. This case highlights the need for Antarctic cruise ships to be equipped to provide life support and for better screening and education of prospective Antarctic tourists.
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keywords = life
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6/55. Recurrent respiratory tract infections and dysphagia in a child with an aortic vascular ring.

    Recurrent respiratory tract infections and dysphagia after the first years of life are rarely caused by vascular rings, and only a high index of clinical suspicion helps to avoid diagnostic delay and inappropriate treatment. Diagnostic workup in a 2-year-old girl with acute foreign body impaction into the esophagus and frequent respiratory tract infections revealed right descending aortic arch with ligamentum arteriosum as the cause of extrinsic esophageal-tracheal compression. Dividing the ligament gave release to the encircled esophagus and trachea. Relief of symptoms was achieved immediately after surgery.
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keywords = life
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7/55. 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 = 2.1431489465013
keywords = life
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8/55. Congenital lobar emphysema.

    Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cause acute respiratory distress in early life. This paper reviews 6 cases of congenital lobar emphysema seen over a period of 10 years. The medical records of children with the diagnosis of congenital lobar emphysema were retrospectively reviewed for age at diagnosis, sex, presenting symptoms, investigations, treatment and outcome. There were 4 males and 2 females, and all of them presented before 6 months of age. Three presented with recurrent chest infection, while the other 3 had acute respiratory distress soon after birth. In all, the diagnosis was confirmed by chest x-ray, and the left upper lobe was affected in all of them. Although congenital lobar emphysema is rare, clinical awareness of this condition is important for early diagnosis and effective surgical treatment.
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keywords = life
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9/55. hemoptysis resulting from unilateral pulmonary artery agenesis.

    Unilateral pulmonary artery agenesis is a rare congenital anomaly often associated with other cardiovascular abnormalities. It is usually diagnosed and surgically treated in childhood. Subjects without associated cardiac anomalies (isolated unilateral pulmonary artery agenesis) may be asymptomatic or have recurrent respiratory infections. We report a case of left pulmonary artery agenesis in a 35-year-old man complicated by hemoptysis and treated by pneumonectomy. physicians should be aware of unilateral pulmonary artery agenesis presenting later in life as a source of chronic respiratory symptomatology or hemoptysis.
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keywords = life
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10/55. Respiratory tract reinfections by the new human metapneumovirus in an immunocompromised child.

    The human metapneumovirus (HMPV), a new member of the paramyxoviridae family, has been recently associated with respiratory tract infections in young children. We report the case of a young, immunocompromised child who had severe lower respiratory tract infections during two consecutive winter seasons caused by genetically distinct HMPV strains.
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ranking = 69.046744609365
keywords = family, member
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