Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0432222 (SEM)
 47,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The aim of this study was to examine baseline mucociliary clearance (MCC) in patients with cystic fibrosis (n = 30; mean +/- SEM age, 23 +/- 1 yr; FEV1, 68 +/- 5% pred; range, 14 to 126%) and a group of normal subjects (n = 12; mean age, 27 +/- 1 yr) after an aerosol deposition of 99mTc-sulphur colloid (mass median diameter, 4.8 microns; geometric standard deviation, 1.6). Dynamic geometric mean images were formed from gamma camera data, and the percent clearance of activity after 60 min (%C60) was calculated for the whole right lung. Initial deposition of the aerosol was determined in terms of the penetration index, the ratio of peripheral to central activity. For normal subjects, an increase in mean inspiratory flow rate (MIFR) (49 +/- 5 versus 21 +/- 3 L/min, p < 0.05) resulted in an increase in whole right lung MCC (%C60, 31 +/- 4 versus 18 +/- 2%; p < 0.05). When aerosol delivery was controlled (MIFR, 34 +/- 5 versus 36 +/- 5 L/min), there was excellent reproducibility between studies (whole lung %C60, 34 +/- 8 versus 31 +/- 7; NS). The measurement of MCC was highly reproducible in six patients studied on four occasions with a mean coefficient of variation of 3.3 +/- 1%. A breathing pattern to accentuate central deposition was utilized in the patient studies (MIFR, 49 +/- 4 L/min).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Mucociliary clearance in patients with cystic fibrosis and in normal subjects. 802 74

To determine the extent of airway infection and inflammation in adolescents and adults with cystic fibrosis (CF) who have mild lung disease and are without symptoms of active infection, we performed bronchoalveolar lavage (BAL) on 18 CF patients > or = 12 yr of age who were stable, appeared clinically well, and had mean (+/- SEM) FEV1 of 79 +/- 4% of predicted. We quantitated the bacteria, inflammatory cells, immunoglobulins, and mediators of inflammatory tissue damage in the epithelial lining fluid (ELF) of these patients and in 23 healthy control subjects. All CF patients were found to be infected with Pseudomonas aeruginosa, Staphylococcus aureus, and/or Haemophilus influenzae; no organisms were isolated from the control subjects. The mean number of cells in the ELF was 14 times greater in the CF patients than in the control subjects. Neutrophils constituted 57% of the recovered cells in the CF patients versus 3% in the control subjects, and their concentration was 380 times greater in the CF patients versus the control subjects. IgG, IgA, and IgM were 2.5 to 6 times greater in CF ELF versus that of control subjects. Abundant active elastase was present in the ELF of the CF patients (2.3 +/- 0.9 microM) despite threefold elevated levels of alpha 1-protease inhibitor (alpha 1-PI). No active elastase was detectable in the control subjects. alpha 1-PI was functional in CF as demonstrated by elevated elastase:alpha 1-PI complex (0.045 microM in CF versus 0.002 microM in control subjects). This active elastase caused proteolytic destruction of surface complement receptors on airway neutrophils in situ.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. 804 28

Previous Ussing chamber measurements of secretagogue-provoked changes in short circuit current in rectal suction biopsies of cystic fibrosis (CF) patients showed that in a minority of patients chloride secretion in response to cholinergic agonists is reduced but not completely absent. To assess a possible relationship between this phenomenon and both the genotype and the phenotype, we performed Ussing chamber experiments on rectal suction biopsies of 51 CF patients. The CF mutation was identified in 89 out of 102 CF alleles. No apparent chloride secretion was found in 30 CF patients (group I). Low residual chloride secretion was found in 11 CF patients (group II), while a relatively high residual secretion appeared in 10 CF patients (group III). Pancreatic function was preserved more frequently in CF patients displaying residual secretion: 0% in group I, 27% in group II, and 60% in group III (P < 0.001). The age at diagnosis (mean +/- SEM) in group III (18.4 +/- 6.6) was significantly different from group I (1.2 +/- 0.4, P < 0.01) and group II (3.5 +/- 1.4, P = 0.05). Residual chloride secretion was found in some of the 28 dF508 homozygous patients (three in group II, and one in group III), disclosing that other factors than the CF gene defect itself affect the transepithelial chloride transport. The age at diagnosis correlates significantly with the magnitude of the secretory response, even within the dF508 homozygous patients (r = 0.4, P < 0.05). We conclude that residual chloride secretion in CF is the pathophysiological basis of preserved pancreatic function and delayed presentation of the disease, which is not exclusively determined by the CF genotype.
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PMID:Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype. 811 84

Dietary supplementation with fish oils high in the omega-3 fatty acids, eicosapentaenoic acid and docosahexaenoic acid, may have an antiinflammatory effect. We determined whether patients with cystic fibrosis (CF) could incorporate omega-3 fatty acids into their plasma and cell membrane phospholipids without adverse effects. In this double-blind study, 12 patients with pancreatic insufficiency who have CF (mean age, 12.2 +/- 5.4 (SD) years) and 13 subjects without CF (mean age, 13.4 +/- 6.3 (SD) years) were randomly assigned to ingest 8 gm daily of either encapsulated fish oil (3.2 gm of eicosapentaenoic acid and 2.2 gm of docosahexaenoic acid daily) or olive oil ethyl esters for 6 weeks. Two of seven and two of five patients with CF who received fish and olive oils, respectively, and one of eight and none of five subjects without CF discontinued taking the capsules before 6 weeks because of eructation or diarrhea. Significant incorporation of omega-3 fatty acids into plasma and erythrocyte membrane phospholipids was observed in subjects with and those without CF randomly assigned to the fish oil treatment. For example, in subjects randomly assigned to receive fish oil, the eicosapentaenoic acid/arachidonic acid ratio in plasma increased 9.8-fold, from 0.04 +/- 0.02 (mean +/- SEM) to 0.39 +/- 0.11 (p = 0.02), in the patients with CF (n = 7) and 23.0-fold, from 0.04 +/- 0.01 to 0.92 +/- 0.17 (p = 0.001), in the subjects without CF (n = 8) who received fish oil (p = 0.02, patients with CF vs subjects without CF at 6 weeks). No clinically or statistically significant changes from baseline were observed in platelet aggregation or levels of vitamin E or A in subjects who received fish oil. Future studies are indicated to determine whether omega-3 fatty acid enrichment provides a clinically beneficial antiinflammatory effect in patients with CF.
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PMID:Oral absorption of omega-3 fatty acids in patients with cystic fibrosis who have pancreatic insufficiency and in healthy control subjects. 807 70

Patients with cystic fibrosis (CF) have a high prevalence of Pseudomonas aeruginosa infection which causes chronic infection of the mucosal surfaces of the lung. This results in recurrent immune stimulation and hypergammaglobulinemia. The present study examines the levels of circulating Ig classes, IgG and IgA subclasses in 13 adult patients with CF during acute pulmonary infection and post-exacerbation. Total serum IgG levels were raised in the patients during infection and post-treatment when compared to the normal range (mean +/- SEM: 17.21 +/- 1.4 g/l vs 16.45 +/- 1.5 g/l respectively; normal range 8-16 g/l). In contrast, total IgM (2.6 +/- 0.26 vs 2.69 +/- 2.74 g/l; normal range 0.6-2.8 g/l) and IgA levels (2.5 +/- 0.52 vs 2.41 +/- 0.48 g/l; normal range 0.5-4 g/l) remained unchanged when examined during all stages of the disease. Of the 13 patients studied, 69%, 39% and 31% had IgG, IgM and IgA levels respectively raised above the normal range values. The mean levels of individual IgG subclasses examined in this group of patients revealed values within the normal ranges, however IgG2 and IgG3 were increased in 31% and 46% of patients. Individual IgG3 levels fell in 77% (10/13) and IgG4 in 62% (8/13) of the patients post-exacerbation. With regard to IgA subclasses, significant reduction in the IgA1 levels were observed post treatment (3687 +/- 539 mg/l vs 2713 +/- 498 mg/l, p < 0.01). In contrast, IgA2 levels were increased from 279 +/- 49 mg/l to 421 +/- 69 mg/l, although statistical significance was not reached. Upon antibiotic treatment for infection, the findings in this study show that IgA1 which is susceptible to bacterial proteases is reduced with a concommitant increase in the protease resistant IgA2 subclass. Moreover, patients colonised with non-mucoid strains of P. aeroginosa had higher total IgA levels due to the raised IgA1 subclass whereas they had lower IgG levels due to low IgG2 and IgG4 subclasses.
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PMID:Serum IgA and IgG subclasses during treatment for acute respiratory exacerbation in cystic fibrosis: analysis of patients colonised with mucoid or non-mucoid strains of pseudomonas aeruginosa. 814 95

This study aimed at examining the mechanisms participating in excessive faecal bile acid loss in cystic fibrosis. The study was designed to define the relation between faecal fat and faecal bile acid loss in patients with and without cystic fibrosis related liver disease; to assess terminal ileal bile acid absorption by a seven day whole body retention of selenium labelled homotaurocholic acid (SeHCAT); and to determine if small intestinal bacterial overgrowth contributes to faecal bile acid loss. The study population comprised 40 patients (27 men; median age 18 years) with cystic fibrosis (n = 8) and without (n = 32) liver disease and eight control subjects. Faecal bile acid excretion was significantly higher in cystic fibrosis patients without liver disease compared with control subjects (mean (SEM) 21.5 (2.4) and 7.3 (1.2) micromoles/kg/24 hours respectively; p < 0.01) and patients with liver disease (7.9 (1.3) micromoles/kg/24 hours; p < 0.01). No correlation was found between faecal fat (g fat/24 hours) and faecal bile acid (micromoles 24 hours) excretion. Eight (33%) of cystic fibrosis patients had seven day SeHCAT retention < 10% (normal retention > 20%). SeHCAT retention in cystic fibrosis patients with liver disease was comparable with control subjects (30.0 (SEM) 8.3% v 36.8 (5.9)%; p = NS) while SeHCAT retention in cystic fibrosis patients who did not have liver disease was significantly reduced (19.9 (3.8); p < 0.05). Although evidence of small bowel bacterial overgrowth was present in 40% of patients no relation was found between breath hydrogen excretion, faecal fat, and faecal bile acid loss. The results are consistent with the presence of an abnormality in terminal ideal function in patients with cystic fibrosis who do not have liver disease and that a defect in the ileal absorption of bile acids may be a contributory factor to excessive faecal bile acid loss. Faecal bile acid loss in cystic fibrosis is unrelated to the presence of intraluminal fat or intestinal bacterial overgrowth.
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PMID:Intestinal bile acid malabsorption in cystic fibrosis. 817 69

The longitudinal growth in 139 patients with cystic fibrosis (CF) was investigated from birth until the age of 19 years. Already at birth weight and length were reduced (weight: -0.83 +/- 0.13 SDS in girls, -0.44 +/- 0.13 SDS in boys; length: 0.55 +/- 0.13 SDS in girls, -0.39 +/- 0.14 SDS in boys; mean +/- SEM). Both variables showed a further decline until diagnosis was established (weight: -1.57 +/- 0.21 SDS in girls, -1.46 +/- 0.25 SDS in boys; length: -1.15 +/- 0.32 SDS in girls, -1.03 +/- 0.52 SDS in boys; mean +/- SEM). Six to 12 months after diagnosis length improved and reached the 25th percentile in both sexes. Height and weight followed the 25th percentile throughout childhood. Growth velocity was fairly normal during this period. There was a loss in percentiles of both height and weight after the age of 8 years and the pubertal growth spurt was delayed and reduced. However, the 25th percentile was reached again in the adolescent period. At the age of 19 years median height was 161.5 cm in girls and 173 cm in boys, both representing the 25th percentile. Using a sensitive statistical method for analysis of growth data we present CF specific growth curves for height, weight and growth velocity. There was no significant effect of pulmonary colonization with Pseudomonas aeruginosa on growth velocity.
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PMID:Perspectives of longitudinal growth in cystic fibrosis from birth to adult age. 818 96

Nebulized antibiotic therapy in cystic fibrosis is an established procedure. The present study was designed to quantitate deposition, and assess its relation to the disease state. Twenty seven children and young adults with cystic fibrosis (mean 11.6 years, range 4-23 years, 12 females) were studied to establish the quantity and pattern of deposition of nebulized tobramycin in the respiratory tract. A single (120 mg) dose of nebulized 99 m technetium-labelled tobramycin was administered, and imaged with a gamma-camera. The mean penetration index (which compares the distribution of 81 m-Krypton gas with Tc-radioaerosol) was also used to measure peripheral deposition efficiency. The aerosol mass median diameter (MMAD) for the compressor-nebulizer system used was 5.3 u, measured with the Malvern Mastersizer. Serial sputum samples were fluroimmunoassayed for tobramycin in nine patients. A mean of 8.0 (SEM 1.0) mg tobramycin reached the lungs. There was no relationship between the total pulmonary deposition and indices of pulmonary damage in cystic fibrosis. Sixteen percent of the lung tobramycin reached the periphery. The greater the lung damage as indicated by FEV1 and Chrispin-Norman scores, the smaller the proportion of pulmonary tobramycin that reached the periphery. The mean penetration index increased with increase in the FRC, but bore no relation to other respiratory function tests or to chest X-ray scores. Sputum tobramycin concentrations reached levels bactericidal for Pseudomonas aeruginosa. Airway obstruction and damage affected the proportion of pulmonary tobramycin reaching the periphery. The proportion of tobramycin reaching the lungs was small and variable.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The quantitative distribution of nebulized antibiotic in the lung in cystic fibrosis. 820 69

We compared the efficacy of manual and mechanical chest percussion during hospitalization for acute exacerbations of cystic fibrosis by evaluating changes in spirometry values. Fifty-one participants were randomly assigned to receive manual or mechanical chest percussion three times a day. Twenty-two participated during one subsequent admission and were assigned to the opposite form of chest percussion. The two groups were equal in severity of illness (mean National Institutes of Health score (+/- SEM): manual = 66.7 +/- 2.2; mechanical = 35.8 +/- 2.2; p = not significant). Mean improvement in forced expiratory volume at 1 second, forced vital capacity, and forced expiratory flow between 25% and 75% of forced vital capacity (+/- SEM) for manual percussion was 32.6% +/- 7%, 27.2% +/- 5%, and 38.1% +/- 10%, and for mechanical percussion was 28.5% +/- 4%, 28.7% +/- 4%, and 25.1% +/- 8%, respectively; p = not significant. Our participants did not prefer mechanical chest percussion. Although equal efficacy of outpatient therapy remains to be proved, this study suggests that patients can be encouraged to use the form of chest percussion that they prefer.
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PMID:Comparison of manual and mechanical chest percussion in hospitalized patients with cystic fibrosis. 830 32

Physical training in patients with pulmonary diseases, including cystic fibrosis (CF), may improve exercise tolerance in these patients. Most training programs are performed in a clinical setting. Little information is available concerning the effect of home exercise training programs in CF patients. The purpose of this study was to investigate the effect of a home exercise training program in CF patients. Ten adolescent patients (seven male and three female) with CF, mean (SD) age 20.6 (6.5) years, participated in a home exercise training program for 3 months. Pretraining condition was assessed during a control period of 2 months in which patients were asked to continue their normal daily activities. Home exercise training consisted of cycle training of 15 min at a submaximal workload once a day and was supervised by a physiotherapist two times a week. A follow-up period of 1 month, in which the patients were advised to continue cycling at home without supervision, was included after the training program. Pulmonary function, bicycle exercise test performance, and the degree of limitation in activities of daily living (ADL) were measured. No significant differences were found between the two pretraining assessments. After the training period we found significant improvement in maximal exercise capacity (Wmax) (mean [SEM]: 126 [10] W before and 146 [11] W after; p = 0.004), maximal oxygen uptake (VO2max) (mean [SEM]: 31.4 [2.1] ml.kg-1.min-1 before and 36.5 [2.5] ml.kg-1.min-1 after; p = 0.008), oxygen pulse (VO2/HR) (mean [SEM]: 10.9 [0.66] ml before and 11.9 [0.74] ml after; p = 0.047), and degree of limitation in ADL (mean [SEM]: 2.4 [0.43] before and 1.5 [0.48] after; p = 0.019) as compared with the initial values. Since no significant differences were found between the posttraining values and the results at the end of the follow-up period, the patients were apparently able to maintain the training effects during the follow-up period. We conclude that in CF patients, a home exercise training program, after clinical assessment of exercise tolerance, is an effective and relatively simple treatment to improve physical performance and decrease limitation in ADL and should be included in the maintenance treatment in these patients.
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PMID:Effect of a home exercise training program in patients with cystic fibrosis. 830 22


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