Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0031350 (pharyngitis)
 2,405 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Respiratory symptoms, recurrent infectious exacerbations, and progressive lung destruction in cystic fibrosis can be attributed to bacterial persistence and the accumulation of viscous purulent secretions in the airways. Purulent secretions contain high concentrations of extracellular DNA, a viscous material released by leukocytes. To evaluate the potential clinical utility of recombinant human DNase I (rhDNase or Pulmozyme), the human enzyme was cloned, sequenced, and expressed. In in vitro studies, rhDNase has been shown to reduce the viscoelasticity, reduce the adhesiveness, and improve the mucociliary transportability of cystic fibrosis sputum. In short-term phase 1 and phase 2 clinical trials, rhDNase has been shown to be safely tolerated and to improve the FEV1, FVC, and symptoms of dyspnea. A long-term placebo-controlled phase 3 study was performed in 968 adults and children (> or = 5 years) with cystic fibrosis to determine the effect of rhDNase on the risk of respiratory exacerbations requiring parenteral antibiotics and on the FEV1. Compared with placebo-treated patients, patients treated with rhDNase once daily or twice daily experienced a reduced risk of respiratory exacerbations by 28% (p = 0.04) and 37% (p = 0.01), respectively, and had a mean improvement in FEV1 of 5.8% (p < 0.01) and 5.6% (p < 0.01), respectively. Compared with placebo-treated patients, patients treated with rhDNase spent 2.7 fewer days receiving parenteral antibiotics (p = 0.04) and spent 1.3 fewer days in the hospital (p = 0.06) over the 6-month treatment period. Inhalation of rhDNase did not cause anaphylaxis but was associated with upper airway symptoms (ie, voice alteration, hoarseness, pharyngitis) that were generally mild and transient. In conclusion, aerosol administration of rhDNase was safely tolerated, reduced the risk of infectious exacerbations requiring parenteral antibiotics, and improved pulmonary function and patient well-being.
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PMID:Aerosolized recombinant human DNase I for the treatment of cystic fibrosis. 784 16

The major causes of morbidity and mortality in cystic fibrosis (CF) are the obstruction and damaged airways that result from the accumulation of viscid and infected secretions. Dornase alfa, also called recombinant human DNase I (rhDNase), cleaves extracellular DNA, which is present in inordinately high concentrations in purulent CF airway secretions. Dornase alfa has been found to increase the pourability and reduce the viscoelasticity of CF sputum in vitro and, in an animal model, to increase its mucociliary transportability. Short-term (10-day) Phase I and II clinical trials showed dornase alfa to be safe and effective in improving pulmonary function in clinically stable CF patients with mild to moderate pulmonary disease (FVC > or = 40% of predicted value). A long-term (24-week) Phase IIB clinical trial demonstrated the importance of administering dornase alfa daily to maintain its efficacy. A large-scale, long-term, Phase III clinical trial, consisting of a 24-week double-blind period and a 24-week open-label extension, confirmed these findings and further demonstrated that dornase alfa reduces the incidence of respiratory tract infectious exacerbations requiring parenteral antibiotic therapy. Dornase alfa also decreased the rate of hospitalizations, the number of days missed from work or school, and the frequency of CF-related symptoms. Adverse events were limited to upper airway irritation (i.e., voice alteration, laryngitis, pharyngitis), rash, chest pain, and conjunctivitis. These manifestations generally were mild and transient, and they did not limit the use of dornase alfa. A small proportion (2 to 4%) of patients developed serum antibodies to dornase alfa, but no patient developed anaphylaxis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Aerosolized dornase alfa (rhDNase) for therapy of cystic fibrosis. 788 98

Cystic fibrosis is characterized by the accumulation of thick viscous purulent secretions. Recombinant human deoxyribonuclease I (rhDNase) breaks down extracellular DNA, which contributes to the increased viscosity of sputum. A multinational, open-label study was conducted in 974 cystic fibrosis patients with moderate lung disease [forced vital capacity (FVC) 40-70% of predicted values] to examine the safety and efficacy of aerosolized rhDNase, 2.5 mg, once daily over a period of at least 12 weeks. Patients were assessed under conditions reflecting routine clinical practice. During rhDNase therapy, at least one respiratory tract infection (RTI) requiring intravenous antibiotics was experienced by 29.5% of patients. Forced expiratory volume in 1 second (FEV1) and FVC were significantly improved from baseline by a mean of 10.5% and 7.2%, respectively. Voice alteration and pharyngitis were the most frequent rhDNase-related adverse events, but only 2% of all patients discontinued treatment due to adverse events. The results obtained were similar to a subanalysis of data from the first 3 months of a placebo-controlled U.S. study. The patients in the present study had a similar frequency of RTIs and improvement in pulmonary function, and reported fewer rhDNase-related and cystic fibrosis-related adverse events than patients in the U.S. study. We conclude that administration of rhDNase is safe, well tolerated, and effective under conditions reflecting routine clinical practice in patients with cystic fibrosis and moderate lung disease.
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PMID:Multicenter, open-label study of recombinant human DNase in cystic fibrosis patients with moderate lung disease. DNase International Study Group. 977 9

Burkholderia cepacia is a Gram-negative bacillus that is widely distributed in nature; it is isolated from the ground, water, plants and vegetables. Generally, it produces nosocomial infection due to contamination of disinfectants, medical equipment, prosthetic material and drugs, such as anesthetics or liquids used in urological irrigation. The most probable mechanism of transmission is through hospital material or through fomites among people after contact for several weeks or months. Recently, it has been considered as an important pathogen in immunocompromised patients, or in those with significant underlying diseases, such as chronic granulomastosis or cystic fibrosis. We present a case of pharyngitis due to B. cepacia and its transmission within a few days in two immunocompetent twin siblings without previous underlying diseases. The infection disappeared after specific treatment for this microorganism was started. We believe that samples should be taken from the pharynx and nasal pits in patients with acute upper respiratory tract processes that do not respond to empiric antibiotic treatment, before classifying them as viral infection without etiologic diagnosis.
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PMID:[Pharyngitis due to Burkholderia cepacia. Person-to-person transmission]. 1520 71

An 8-year-old female with pancreatic-insufficiency cystic fibrosis presented with recurrent pharyngitis, and reduction in body mass index and height velocity during the previous 2 years. Her symptoms (eg, snoring and restless sleep) suggested obstructive sleep apnea, and physical examination revealed tonsillar hypertrophy. While her respiratory disturbance index on nocturnal polysomnography was normal, there was evidence of prolonged periods of snoring, associated with hypercapnia. Adenotonsillectomy decreased the snoring, improved her sleep, and in the 18-month follow-up period she had substantial weight-gain and growth improvement. This case demonstrates that adenotonsillar hypertrophy associated with recurrent pharyngitis and primary snoring might hinder growth in a patient with cystic fibrosis.
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PMID:Primary snoring and growth failure in a patient with cystic fibrosis. 1996 40

We report a case of Pseudomonas pharyngitis in a cystic fibrosis (CF) patient not known to have prior colonization of Pseudomonas. We believe that this is one of the first cases of pseudomonas pharyngitis in a CF patient.
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PMID:Pseudomonas pharyngitis in a cystic fibrosis patient. 2973 41