Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149514 (bronchitis)
 6,902 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe a case of association cystic fibrosis coeliac disease, which they have observed. The frequency of this association in the general population is of 1:2,000,000. M.A. presents little growth in terms of height and weight and intestinal alterations (an abundance of fatty and mucous stool). The initial diagnosis was that of coeliac disease. After two months of diet without gluten one observed an increase in weight, but o result for the intestinal disturbances. The positive result revealed from the sweat-test and the study of genomic mutation (delta F508) showed the diagnosis of cystic fibrosis associated with coeliac disease. The specific treatment of both syndromes resolved intestinal alterations and caused the diminution of the acute bronchitis frequent during the first and the second infancy.
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PMID:[Celiac disease and cystic fibrosis: casual association?]. 779 4

We report a case of pulmonary infection by Mycobacterium avium complex revealing in a 21 years old patient a cystic fibrosis heterozygous for the delta F 508 deletion. The role of this bacteria in the lung infection is suggested by repeated isolation. This infection caused bronchitis, hypoxemia and pulmonary nodules at CT scan. The clinical and radiological signs improved after treatment with four antibiotics whereas only clarithromycin showed in vitro activity. An obstructive disease due to allergic bronchopulmonary aspergillosis developed and was controlled by steroid therapy.
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PMID:[Delayed detection of cystic fibrosis in a patient with Mycobacterium avium complex infection]. 781 97

Air compressors for inhalation devices were equipped with counters which were activated whenever the inhalation was started. They recorded the time of the activated inhaler. The counters were invisible for the parent and the doctor. 40 outpatients, all children, were randomly given such a device for treatment at home under surveillance of their parents. The duration, dose and frequency of inhalation was explained, and the parents were instructed that if the child should not be well, the inhalation frequency could be doubled. 21 children (age: 3.8 +/- 3.7 years from two months to 14 years), five girls, 16 boys, were on the inhalation devices equipped with the counter; ten had asthma, four acute bronchitis, four mild bronchiolitis, one had pertussis, one cystic fibrosis and one pneumonia. The prescribed inhalation treatment extended over 21 +/- 14 days (3 to 50), the inhalation frequency per day was one to three times. The measured inhalation unit (recorded inhalation time times frequency) amounted to 4.87 +/- 5.01 vs. 4.31 +/- 3.58 (n.s.) for the expected unit (expected inhalation units according to the prescription). The maximum and the minimum differences for the measured vs. the expected unit were significantly different (p < 0.001). Ten children inhaled 80 to 120% of the prescribed inhalation units, six children inhaled more than 140%, and five children less than 70% of the prescribed units. This resulted in an overall inhalation compliance of 47.6%. This is the first double-blind study carried out so far studying inhalation compliance in children with respiratory diseases.
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PMID:[Compliance with inhalation therapy in children with respiratory diseases]. 790 18

Cystic fibrosis (CF) is characterized in the lung by chronic purulent bronchitis culminating in pulmonary insufficiency. There is evidence to suggest that neutrophil elastase (NE) released by neutrophils on the respiratory epithelial surface plays a major role in the pathogenesis of this lung disease. This study sought to determine the age of onset of the chronic neutrophil-dominated inflammation in CF and the consequences to the NE-anti-NE screen on the respiratory epithelial surface of the CF lung. NE and anti-NE defensive molecules were evaluated in respiratory epithelial lining fluid (ELF) in 27 children with stable CF (1 to 18 yr of age). Despite normal antigenic concentrations of alpha 1-antitrypsin (alpha 1AT) and secretory leukoprotease inhibitor (SLPI), 25 of 27 children with CF had neutrophil-dominated inflammation (> 500 neutrophils/microliters ELF). Active NE was found in ELF in 20 of 27 children, including two of four aged 1 yr. Western blot analysis showed the majority of alpha 1AT and SLPI molecules to be complexed and/or degraded. These observations demonstrate that a chronic imbalance of the NE-anti-NE protective screen develops early on the respiratory epithelial surface in persons with CF and is likely well established by 1 yr of age, with resultant potential for lung damage.
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PMID:Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. 791 87

Airway mucus is a complex airway secretion whose primary function as part of the mucociliary transport mechanism is to to serve as renewable and transportable barrier against inhaled particulates and toxic agents. The rheologic properties necessary for this function are imparted by glycoproteins, or mucins. Some respiratory disease states, e.g., asthma, cystic fibrosis, and bronchitis, are characterized by quantitative and qualitative changes in mucus biosynthesis that contribute to pulmonary pathology. Similar alterations in various aspects of mucin biochemistry and biophysics, leading to mucus hypersecretion and altered mucus rheology, result from inhalation of certain air pollutants, such as ozone, sulfur dioxide, nitrogen dioxide, and cigarette smoke. The consequences of these pollutant-induced alterations in mucus biology are discussed in the context of pulmonary pathophysiology and toxicology.
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PMID:The role of airway mucus in pulmonary toxicology. 792 90

Among the roles of mediators damaging the respiratory epithelium in patients with cystic fibrosis (CF) during the course of chronic, purulent bronchitis, that of neutrophil proteases is well established. The role of bacterial proteases is less well known. Among all pathogens colonizing the airways in CF, Pseudomonas aeruginosa is quantitatively the dominant pathogen; Staphylococcus aureus and Haemophilus influenzae are present in lower numbers. Anaerobic bacteria may be detected in numbers exceeding those of Staphylococcus aureus and Haemophilus influenzae. Among all enzymes secreted by these bacterial strains, Pseudomonas elastase and alkaline protease were shown to be secreted in vivo over prolonged periods in the airways. These enzymes, mainly elastase, have proteolytic activity on many proteins involved in host defense mechanisms, often the same as those hydrolyzed by neutrophil proteases. Pseudomonas elastase has damaging effects on the respiratory epithelium; it has recently also been shown to augment the permeability of the respiratory epithelium cultured in vitro by proteolytic attack of tight junctions. The potential role of proteases and other enzymes secreted by anaerobic bacteria has not been studied in this disease. In conclusion, bacterial proteases secreted in vivo may play a role in the pathogenesis of the airway disease in CF; their relative importance to the role of host proteases is, however, often difficult to determine.
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PMID:The role of bacterial proteases in the pathogenesis of cystic fibrosis. 795 46

A 7-year-old boy with recurrent otitis media, bronchitis, pneumonia, asthma, and sinusitis was found to have primary ciliary dyskinesia. It was important to rule out other systemic diseases such as immune deficiency and cystic fibrosis. Electron microscopy of a properly obtained and prepared biopsy of the mucosal surface of the nose, trachea, or bronchus is essential.
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PMID:A seven-year-old boy with sinusitis, otitis media, and asthma. 803 Jul 98

Patients with cystic fibrosis (CF) suffer from severe chronic pulmonary infections but rarely develop bacteremia/septicemia suggestive of an intact splenic mononuclear phagocyte function. The splenic function of 25 patients diagnosed with CF, aged 2 to 37 years, was evaluated using erythrocyte pit count by direct interference contrast microscopy. Results were compared with patients with sickle cell disease and normal individuals. All CF patients displayed normal splenic function by pit count. The mean percentage of pitted erythrocytes was 0.20 +/- 0.28 (range: 0.0% to 1.0%) versus 0.19 +/- 0.33 (range: 0.0% to 1.4%) in normal eusplenic controls. There were no episodes of bacteremia or septicemia despite recurrent acute exacerbations of chronic bacterial bronchitis and the use of central lines. We conclude that splenic function in CF is unabridged and may account for the rarity of bacteremia/septicemia in patients with CF despite the high prevalence of chronic bronchial infection in this population.
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PMID:Intact splenic function in cystic fibrosis. 804 Sep 2

The term sinobronchial syndrome is often used, but there is no generally accepted definition. Due to the functional and clinical unity of the whole respiratory tract infectious and allergic irritations lead to homogeneous reactions of the bronchi and the paranasal sinuses. On the other hand there are hints that a disease in one region can cause problems in the other or reinforce it. Probably bronchi and sinuses can also become infected simultaneously from the pharynx. In chronic cases sinusitis and bronchitis are often only symptoms masking an underlying disease that requires differentiated diagnostics and treatment, e.g. cystic fibrosis, primary ciliary dyskinesia, and immunological defects.
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PMID:[Sinobronchial syndrome--a meaningful diagnosis?]. 836 Nov 41

Cystic fibrosis, an autosomal recessive disorder, is the most common genetic disease of Caucasians. One in 25 Caucasians are carriers of the gene. The gene is found far less commonly in other races. There are over 230 different alleles of the gene, located on the 7th chromosome. The gene encodes for a membrane protein that functions as an ion channel. The survival of cystic fibrosis patients has been gradually increasing, with a mean survival in 1990 of 28 years. If the current trend of improved survival continues, it is estimated that half of cystic fibrosis patients will be over 18 years old by 1996. Disease is found in many organs including the lungs, sinuses, pancreas, gastrointestinal tract, hepatobiliary system, sweat glands and reproductive tract. The majority of patients die of pulmonary disease. The airways become chronically colonized with bacteria that cannot be eradicated, leading to bronchitis, bronchiectasis, and finally, pulmonary fibrosis with respiratory failure. The pulmonary disease may be complicated by massive hemoptysis and pneumothorax. Patient survival rates have increased because of antibiotic therapy and improved nutrition with pancreatic enzyme replacements. New treatments for the pulmonary disease are under clinical trial and include antiproteases, amiloride, a sodium channel blocker, and DNase. The insertion of the normal cystic fibrosis allele into an animal model using a modified adenovirus with effective transcription suggests that gene therapy may be possible in the future, but safety and technical problems have to be addressed.
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PMID:Cystic fibrosis. 842 40


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