Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with cystic fibrosis (CF) were investigated for mucociliary clearance (with and without stimulation by terbutaline), clinical picture, ventilatory function and ultrastructure of cilia. The results were compared with those of patients with congenitally immotile cilia (immotile-cilia syndrome). Mucociliary clearance could be demonstrated in all the seven CF patients who succeeded in inhaling the test aerosol. Ciliary ultrastructure from a deceased CF patient was normal. Patients with the immotile cilia syndrome had no substantial clearance and defective cilia. The CF patients coughed more during the clearance measurements than any other group studied earlier, and their coughing was effective. One patient succeeded in avoiding coughing in both measurements and had faster clearance when he got terbutaline than when he got the vehicle. Although younger, the CF patients tended to be more obstructed in their lungs and more handicapped than the patients suffering from the immotile-cilia syndrome. The latter patients had more discomfort from rhinitis, sinusitis and otitis than had the CF patients. An impairment of the mucociliary transport rate is hence unlikely to be a primary pathogenic factor for the respiratory tract disease in CF patients.
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PMID:Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function. 37 89

The output of secretions from the airway submucosal glands is regulated by vagal efferent nerves. Stimulation of cough receptors increases mucus output reflexly via the vagus nerves. Adrenergic agonists increase submucosal gland secretions in some species, which indicates that adrenergic receptors are present in these cells. However, evidence for adrenergic nervous pathways to the glands is limited. Irritants and drugs stimulate secretion from epithelial cells by direct effects. There is also evidence that the secretion of epithelial cells can be stimulated by parasympathetic nervous pathways in birds but not in mammals. Active ion transport of Cl- toward the lumen and of Na+ toward the submucosa results in net ion movement toward the airway lumen in unstimulated tracheal epithelia. Drugs and mediators increase the net movement of ions toward the lumen. No agents have yet been found that increase net ion movement toward the submucosa. The link between ion transport and water secretion in airway epithelia, although speculative, seems likely in view of the evidence from other epithelia. Since airway epithelium is a "tight junction" epithelium, modification of the tight junction may alter the transepithelial movement of water and ions. We suggest that the depth and consistency of the periciliary layer of airway secretions determine the ability of the cilia to propel the mucoprotein gel and thereby modify mucociliary transport. To achieve this, secretion of mucus must be controlled separately from the secretion of water. Studies are needed to determine which of the specialized functions of the epithelial cells interact to regulate the clearance of secretions from the airway. Is the sol maintained by secretion and reabsorption of fluid across the epithelium? Does the sol move with the gel by ciliary action or does it remain stationary? Do changes in the epithelial tight junctions influence net water movement and thus indirectly alter the depth of the sol layer? To answer these questions, techniques are needed to study subunits of the airway, including isolated surface cells and submucosal glands; and sensitive methods are required to analyze the very small samples of secretions for glycoprotein and electrolyte content. Intracellular measurements of electrolyte concentrations and electrical potentials may help to elucidate the mechanisms of transepithelial ion and water movement. The control system for the production and removal of respiratory tract secretions may be altered in disease. For instance, chronic stimulation of cough receptors causes reflex secretion and may be the cause of the hyperplasia of submucosal glands and of the abnormal secretions that occur in chronic bronchitis and asthma (50, 58). The abnormally viscid mucus in cystic fibrosis may be due to a defect in Cl- transport, which provides too little water for both the gel and sol layers. These speculations are intended to identify areas for further research, which hopefully will reduce the morbidity and mortality in these common lung diseases.
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PMID:Control of mucus secretion and ion transport in airways. 37 97

Flows measured from maximal expiratory flow-volume (MEFV) curves were used to evaluate the efficacy of postural drainage in improving ventilatory function acutely. Maximal expiratory flow-volume curves were obtained for 9 cystic fibrosis subjects and 10 subjects with chronic bronchitis before and 5, 15, and 45 min after a 30-min session of postural drainage with percussion, vibration, and coughing. Forced vital capacity (FVC) was significantly increased 45 min after drainage for the combining group. Flows at high lung volumes were different for the 2 subgroups. Subjects with cystic fibrosis demonstrated a significant increase in peak expiratory flow rates 45 min after drainage and an increase in forced expiratory volume in one sec at all time intervals. The subjects with chronic bronchitis had a decreased peak expiratory flow rate 5 min after drainage, but by 45 min, it had returned to baseline. There was no significant change in one-sec forced expiratory volume at any time interval for the chronic bronchitis subgroup. Changes in flows at low lung volumes were similar for the 2 subgroups. Forty-five min after drainage there was an increase in flow rates near 50 per cent of FVC. Flows near 25 per cent of FVC were increased 15 and 45 min after drainage. This study demonstrated that postural drainage with coughing resulted in significant improvement in flows at low lung volumes. Changes in flows at high lung volumes were less consistent.
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PMID:Maximal expiratory flows after postural drainage. 43 96

Forty-five patients (25 male and 20 female) over 12 years of age with cystic fibrosis have been studied clinically, radiologically and physiologically. Their mean age at the first visit was 17 years; they were followed for a mean period of 4 years and attended at least every six months. The first symptom which developed before the age of five in 42 of the 45 patients was respiratory. Thirty-two of the 45 patients had severe lung disease (Group III) at the start of the study of the seven patients died during the study. Cough and sputum were almost universal, 23 had haemoptyses and eight pneumothoraces. Staphylococcus pyogenes, Haemophilus influenzae and Pseudomonas aeruginosa were the common pathogens isolated from sputum and the increasing prevalence of the latter was again confirmed. Acquisition of the mucoid strain of pseudomonas signified poor prognosis. Established infection was never eradicated. Forty-three patients had evidence of pancreatic insufficiency; in all but one patient the symptoms were mild and five patients abandoned dietary restriction and pancreatin without ill effect. Seven patients had symptoms of partial bowel obstruction (meconium ileus equivalent) but only one required surgical relief. The liver was enlarged in seven patients and the spleen was felt in three. Three patients had diabetes mellitus. The influence of cystic fibrosis on growth and development is reported--the growth spurt is late in the majority but growth failure is not confined to those with severe lung infection or malabsorption and in these circumstances remains unexplained. Mean weight was low in relation to height and puberty was delayed in both sexes.
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PMID:Cystic fibrosis in adolescents and adults. 82 Oct 91

Fourteen patients with cystic fibrosis were trained in 2 self-administered chest physiotherapy (PT) techniques: high-pressure PEP-mask physiotherapy (PEP), and autogenic drainage (AD). They then visited the clinic on 5 consecutive days, and, in a random order, performed 1 of the following: PEP, AD, PEP followed by AD (PEP-AD), AD followed by PEP (AD-PEP), and, no PT except for spontaneous coughing. Lung function was measured repeatedly before, during, and after PT; time needed for and sputum produced by each form of PT was recorded. PEP produced the highest amount of sputum, followed by PEP-AD, AD-PEP, and AD; all 4 forms of PT produced significantly more sputum than coughing. Lung function improved significantly after PEP, AD, and PEP-AD, but PEP-induced changes did not exceed those after AD. Within the investigated group, the PEP-induced lung function improvement per milliliter of sputum produced was significantly lower for those patients with airway hyperreactivity. The fact that the highest sputum yield with PEP was not reflected in higher PEP-effected lung function changes might thus be explained by PEP-induced bronchospasm in patients with airway hyperreactivity. PEP clears more sputum than AD or combined techniques; patients with airway hyperreactivity, however, should either prefer AD or should take a bronchodilator premedication before PEP.
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PMID:Self-administered chest physiotherapy in cystic fibrosis: a comparative study of high-pressure PEP and autogenic drainage. 143 82

Sinusitis can occur as an acute, subacute, recurrent acute, or chronic clinical disease process in children. Sinusitis most often manifests as a prolongation or complication of a viral upper respiratory tract infection. Because children average six to eight upper respiratory tract infections per year, sinusitis is probably a more frequent diagnosis in the pediatric age group compared with adults who average two to three upper respiratory infections per year. Upward of 5 to 13% of children may experience sinusitis, but precise incidence data are not available because many imaging techniques currently available are inappropriate procedures for a prospective pediatric survey. Symptoms of acute sinusitis in children can vary from the more common persistent, purulent rhinorrhea and cough to the less common symptoms of fever, headache, facial pain, and swelling. Recurrent acute and chronic sinusitis may be associated with another condition such as a host-defense defect, cystic fibrosis, asthma, or a local condition that predisposes to obstruction of the sinus ostia such as nasal polyps, deviated septum, foreign body, or allergic inflammation. Diagnosis of sinusitis can be made on the basis of a careful history and physical examination with radiography reserved for confirmation of clinical impression or documentation of disease. Although fiberoptic rhinoscopy is used more frequently as an adjunct in adults for the evaluation and management of sinusitis, more studies need to be performed to document its clinical usefulness in children.
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PMID:Diagnosis of sinusitis in children: emphasis on the history and physical examination. 152 32

The respiratory mucus is a very complex biological material, which possesses both flow and deformation rheological properties, characterized by non-linear and time-dependent viscoelasticity and physical properties of adhesiveness and wettability. Viscosity and elasticity are directly involved in the transport capacity of mucus, whereas wettability and adhesiveness contribute to the optimal interface properties between the mucus and the epithelial surface. Optimal conditions for the protective and lubricant properties of respiratory mucus are represented by high wettability, and adhesiveness high enough not to induce flow of mucus in the respiratory bronchioles under gravity but low enough to mobilize mucus by airflow during coughing. An intermediate viscoelasticity is also required for an optimal mucociliary transport. Different biochemical constituents such as glycoproteins, proteins, proteoglycans and lipids are involved in the gel properties of respiratory mucus. During bronchial infection and particularly in cystic fibrosis, the loss of water and the increase in macromolecules result in a marked increase in viscosity and adhesiveness responsible for the mucus transport impairment. The various lipids present in mucus contribute differently to the physicochemical properties. Surface-active phospholipids, such as phosphatidylcholine and phosphatidylglycerol improve the wettability of mucus, whereas neutral lipids and glycosphyngolipids contribute to the hyperviscosity of mucus during infection. Phospholipids and associated mucins are also implicated in the interaction between bacteria and epithelial cells. Therefore, the respiratory mucus needs appropriate physicochemical properties for the protection, hydration and lubrication of the underlying airway epithelium.
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PMID:Role of the physiochemical properties of mucus in the protection of the respiratory epithelium. 156 6

The altered carbohydrate structure of sputum from patients with cystic fibrosis (CF) has been thought to be due to the inflammatory airway response. Carcinoembryonic antigen (CEA) and CA 19-9 detect sialosylated carbohydrates in mucus. The epitope of CA 19-9 is part of the Lewis A (Le(a)) blood group antigen. Serum concentrations of CEA and CA 19-9 were determined by radioimmunoassay in 41 CF patients, aged 6-34 years; 16 were asymptomatic Outpatients, and 25 had been admitted for pulmonary exacerbations. There was no difference in CEA between groups. The CA 19-9 serum concentration was elevated in 90% of patients who had at least one of the two Lewis antigens. The CA 19-9 concentration of Inpatients with exacerbations was 2.7 times that of stable Outpatients (263 +/- 44 versus 99 +/- 13 U/mL; P less than 0.02). CA 19-9 correlated significantly with age (r = 0.35, P less than 0.05), Brasfield score (r = 0.39, P less than 0.015), pulmonary function tests, cough severity (r = 0.50, P less than 0.001) and NIH clinical score (r = 0.57, P less than 0.001). CA 19-9 concentration of Inpatients decreased by 44% from admission to discharge (302 +/- 45 to 169 +/- 39, P less than 0.02). Fourteen of 25 (56%) of the Inpatients were Le(a) positive versus only 3/15 (20%) of Outpatients who had milder lung disease (P less than 0.002). Of the Inpatients, 25% with more advanced lung disease were Le(a+b+), a rare blood group in the normal population, and one not observed in the Outpatients with milder disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Changes in the CA 19-9 antigen and Lewis blood group with pulmonary disease severity in cystic fibrosis. 161 47

BACKGROUND Preliminary evidence suggests that regular inhalation of nebulised amiloride reduces sputum viscoelasticity, increases the clearance of sputum by mucociliary mechanisms and by coughing and reduces the rate of deterioration in lung function in patients with cystic fibrosis. These effects depend on adequate delivery of amiloride to the airways. This study was performed to quantify and compare pulmonary deposition of amiloride produced by two different nebuliser systems. METHODS The pulmonary deposition of nebulised amiloride (1 mg in 3 ml saline) was measured in eight patients with cystic fibrosis when given via a jet (System 22 with CR 60 compressor) and an ultrasonic (Fisoneb) nebuliser. Human serum albumin labelled with technectium-99m was used as an indirect marker for amiloride and its deposition in the lung was detected with a gamma camera. RESULTS Amiloride inhalation caused no side effects or changes in spirometric indices. The mean (SD) total pulmonary amiloride deposition was 57 (24) micrograms with the System 22 and 103 (53) micrograms with the Fisoneb nebuliser. Pulmonary deposition was completed more rapidly with the Fisoneb (4-5 minutes) than with the System 22 nebuliser (7-8 minutes) and the Fisoneb was preferred by the patients. CONCLUSIONS Both nebulisers appeared to deliver adequate amounts of amiloride to the lungs, but treatment with the Fisoneb nebuliser was quicker, more efficient, and more acceptable to the patients. Of the two nebulisers assessed, the Fisoneb would be preferred for clinical trials.
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PMID:Pulmonary deposition of nebulised amiloride in cystic fibrosis: comparison of two nebulisers. 175 18

A higher frequency (25%) of gastrooesophageal reflux (GOR) has been previously reported in patients over 5 years old with cystic fibrosis compared with controls without cystic fibrosis. It was believed that GOR was caused by the complications of cystic fibrosis. We looked for GOR in all 26 children younger than 60 months who had cystic fibrosis diagnosed. They had a classical genetic profile and the usual scattered clinical manifestations for age. GOR was confirmed in 21 (81%): 20 by abnormal pH tracings and in one on a clinical basis. After at least one month of adjusted cystic fibrosis treatment, antireflux treatment (cisapride) was given to 16 patients and variables of GOR improved dramatically. Weight gain was significant and recurrent cough and wheeze disappeared. One year later half of the patients still suffered from GOR. GOR is a major problem in the early life of those with cystic fibrosis and is not the consequence of either respiratory or gastrointestinal complications as it improves with age whereas cystic fibrosis becomes worse with age.
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PMID:New insights on gastro-oesophageal reflux in cystic fibrosis by longitudinal follow up. 175 49


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