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 47,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intestinal permeability was investigated in 14 children with cystic fibrosis making use of [51Cr]EDTA as probe molecule. Ten normal young adults and 11 children served as controls. After oral administration of [51Cr]EDTA, 24 h urine was collected. Urinary radioactivity was calculated and results expressed as percentage of oral dose excreted in 24 h urine. Mean and SEM were as follows: 2.51 +/- 0.21, 2.35 +/- 0.24, and 13.19 +/- 1.72 for control children, normal adults, and cystic fibrosis patients, respectively. The permeability differences between cystic fibrosis patients and either control children or control adults are significant (p less than 0.001).
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PMID:Intestinal permeability to [51Cr]EDTA in children with cystic fibrosis. 308 49

Antibiotic aerosol treatment is successful in treating Pseudomonas infection in some patients with cystic fibrosis, but the amount of drug reaching the lungs is unknown. The deposition patterns of carbenicillin aerosols delivered from two commercially available nebuliser systems (the Turret nebuliser plus Maxi compressor and the Inspiron nebuliser plus Traveller compressor) have been compared in six patients with cystic fibrosis during tidal breathing. The aerosol mass median diameters were 3.2 and 7.3 microns. In addition, the aerosol from the Turret-Maxi nebuliser system was inhaled by a combination of tidal and deep breathing. After two minutes' breathing via a mouthpiece the mean (SEM) deposition in the lungs was 15.60 (1.5) mg carbenicillin with the Turret nebuliser plus Maxi compressor, but only 6.54 (1.09) mg with the Inspiron nebuliser plus Traveller compressor; the distribution pattern within the lung was significantly more peripheral with the former nebuliser system. These differences may be ascribed partly to the smaller droplet size from the Turret system and partly to the higher nebulisation rate from the more powerful Maxi compressor. Tidal plus deep breathing produced a further small but non-significant increase in lung aerosol deposition. A seventh patient, who failed to complete the trial, had little aerosol deposited in his lungs because he inhaled through his nose. These results emphasise the importance of correct selection of nebuliser equipment for antibiotic aerosol treatment.
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PMID:Deposition of carbenicillin aerosols in cystic fibrosis: effects of nebuliser system and breathing pattern. 340 19

Boiled extracts of buccal epithelial cells from control subjects and cystic fibrosis patients were shown to possess calmodulin like activity, as assessed by their ability to activate calmodulin-deficient cyclic AMP phosphodiesterase. Estimation of calmodulin content, using pure calmodulin as standard revealed that control extracts contained 3.08 +/- 0.71 SEM (n = 7) micrograms calmodulin/mg protein and cystic fibrosis extracts 0.88 +/- 0.30 SEM (n = 12) micrograms calmodulin/mg protein (p less than 0.02 for difference from control). The results indicate that the biological activity of calmodulin is altered in buccal epithelial cells from cystic fibrosis individuals.
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PMID:Altered calmodulin activity in buccal epithelial cells from cystic fibrosis patients. 343 48

The enzymatic activity of plasma dopamine-beta-hydroxylase (DBH) was determined in 92 children/adolescents with cystic fibrosis, in 25 parents (heterozygotes) of these patients and in 68 healthy controls. Statistically significant (P less than 0.01) lower DBH levels were found in cystic fibrosis patients (17.9 +/- 1.4 mumol/min per 1, SEM) compared to the controls (25.5 +/- 2.1 mumol/min per 1). In 37 patients plasma norepinephrine and dopamine levels were compared to the enzymatic activity, no significant correlation between these variables being shown (r = -0.134 for norepinephrine; r = 0.159 for dopamine). However, due to the large variability of DBH within the groups and due to the lack of relationship of this enzymatic activity to plasma catecholamines, it is concluded that these differences may neither account for the observed elevation of plasma dopamine in cystic fibrosis patients (0.94-6.73 nmol/l vs. 0.01-0.986 nmol/l for controls) nor for their putative autonomic dysfunction.
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PMID:Dopamine-beta-hydroxylase in patients with cystic fibrosis. 373 34

The time course of resting free intracellular calcium concentrations in isolated mononuclear blood cells following a one hour incubation period with the fluorescent dye quin2 was evaluated. Under equal experimental conditions, a slow time-dependent increase of intracellular free calcium in patients with cystic fibrosis and normal healthy controls was noted. Using regression analysis, cystic fibrosis patients were seen to exhibit significantly higher free intracellular calcium concentrations than the controls over the time span covered. At an arbitrarily selected time (60 minutes) the free calcium level was 143.7 +/- 4.3 nM (SEM) in the patients, and 125.5 +/- 2.6 nM in controls. From these data it is concluded that neglecting the time-dependent (Ca2+)i changes following quin2 incubation leads to over- and/or underestimation of the unstimulated resting, basic free calcium levels and prevents the detection of differences between normals and cystic fibrosis patients.
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PMID:Intracellular free calcium levels in mononuclear cells of patients with cystic fibrosis and normal controls. 382 22

Fat absorption was studied in 11 Cystic Fibrosis patients and 6 controls by measuring a) fecal fat excretion and b) serum turbidity using a nephelometer between 0 and 2 hours after a butter-fat load. Cystic Fibrosis patients showed a mean +/- SEM fecal fat excretion of 21.6 +/- 5.5%, and controls a mean of 2.7 +/- 0.45%, of the dietary fat. The turbidity change expressed as delta turbidity showed a mean value of 1.37 +/- 0.57 in Cystic Fibrosis patients and 31.8 +/- 5.7 in controls. Fecal fat excretion method showed a good negative correlation with the butter fat method. The butter fat absorption method was also compared with fecal fat excretion in rats with induced malabsorption and control rats. The results obtained were comparable to those in the patients. The method described here is rapid and can be performed with small blood volumes and therefore may be suitable to study fat malabsorption in pediatric patients.
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PMID:A study of fat absorption in cystic fibrosis patients and controls by a rapid micro procedure: a comparison with fecal fat excretion method. 665 87

To investigate nutritional growth retardation and the adaptive response to malnutrition in cystic fibrosis (CF), body composition and muscle protein catabolism were studied in nine malnourished CF children and eight healthy controls by anthropometry, measurement of whole body potassium, urinary creatinine excretion, creatinine height index, and urinary 3-methylhistidine excretion, an index of myofibrillar protein catabolism. CF children had a significant deficit of body mass (p less than 0.001), derived from both the body fat and the fat-free compartments, including a deficit in muscle mass (p less than 0.005). A deficit of muscle mass in CF was also reflected by a lower creatinine height index (mean +/- 1 SEM = 0.66 +/- 0.04 in CF, versus 0.85 +/- 0.5 in controls, p less than 0.02). Urinary 3-methylhistidine excretion was elevated in CF children and the mean (+/- 1 SEM) rate of muscle protein catabolism was 0.82 +/- 0.06 versus 0.53 +/- 0.04 kg-1 24 h-1 in CF and controls, respectively (p less than 0.01). 3-Methylhistidine excretion rates did not correlate with severity of disease as assessed by clinical score. We conclude that nutritional growth retardation in CF is characterized by a protein energy deficit resembling that of protein-energy malnutrition, but that in contrast to the normal adaptive response to protein-energy malnutrition, muscle protein catabolism is markedly increased. These data may have important implications regarding the clinical course and prognosis of CF and the design of optimal therapy.
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PMID:Altered body composition and muscle protein degradation in nutritionally growth-retarded children with cystic fibrosis. 711 55

We assessed ileal functional integrity, as reflected in the capacity to reabsorb the bile acid, cholylglycine, by sequential measurement of postprandial serum levels of this compound. A consistent increase in cholylglycine concentration (mean +/- SEM, peak above fasting baseline = 1.86 +/- 0.14 microM) occurred in eight normal children. Five patients with ileal resection and two with primary bile acid malabsorption had a minimal postprandial increase (0.27 +/- 0.08 microM; P less than 0.005 vs controls) and excess bile acid loss in feces. Similarly, in seven patients with Crohn disease involving the ileum the postprandial increase (0.38 +/- 0.06) was less than controls (P less than 0.001). In eight patients with cystic fibrosis, the administration of pancreatic enzymes normalized the blunted response demonstrated when enzymatic therapy was interrupted suggesting an intact ileal active transport mechanism. Meal-stimulated response of serum cholylglycine concentration is a sensitive indicator of altered ileal integrity and bile acid malabsorption due to ileal resection, inflammation, or dysfunction.
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PMID:Serum bile acid response to a test meal stimulus: a sensitive test of ileal function. 735 62

To assess the mechanism of the decrease in hemoglobin O2 saturation during rapid-eye-movement sleep, we studied 5 normal subjects 22 to 30 yr of age and 20 patients with cystic fibrosis 9 to 29 yr of age. The largest decrease in arterial O2 saturation, as monitored with an ear oximeter during sleep, occurred during rapid-eye-movement sleep, with a mean +/- SEM decrease of 2 +/- 0.31% in the normal subjects and 7.4 +/- 1.3% in the patients in both groups. Rapid-eye-movement sleep was associated with a significant loss of intercostal and diaphragmatic tonic muscle activity (p < 0.01), as monitored with surface electrodes, and a decrease in the baseline position of the rib cage and abdomen, as recorded by magnetometers (p < 0.01). This suggests a decrease in functional residual capacity, which was accompanied by a consistently lower arterial O2 saturation during rapid-eye-movement sleep. Short periods (< 20 s) of inhibition of phasic respiratory muscle activity during rapid-eye-movement sleep were followed by further decreases in arterial O2 saturation. We conclude that the desaturation during rapid-eye-movement sleep in all subjects was mainly due to a decrease in functional residual capacity, leading to airway closure in the dependent lung regions. The hemoglobin desaturation was further aggravated by transient periods of hypoventilation.
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PMID:Mechanism of hemoglobin desaturation during rapid-eye-movement sleep in normal subjects and in patients with cystic fibrosis. 741 80

The cystic fibrosis transmembrane conductance regulator (CFTR) is an epithelial Cl- channel regulated by protein kinase A. The most common mutation in cystic fibrosis (CF), deletion of Phe-508 (delta F508-CFTR), reduces Cl- secretion, but the fatal consequences of CF have been difficult to rationalize solely in terms of this defect. The aim of this study was to determine the role of CFTR in HCO3- transport across cell membranes. HCO3- permeability was assessed from measurements of intracellular pH [pHi; from spectrofluorimetry of the pH-sensitive dye 2',7'-bis(2-carboxyethyl)-5-(and -6)carboxyfluorescein] and of channel activity (patch clamp; cell attached and isolated, inside-out patches) on NIH 3T3 fibroblasts and C127 mammary epithelial cells transfected with wild-type CFTR (WT-CFTR) or delta F508-CFTR, and also on mock-transfected cells. When WT-CFTR-transfected cells were acidified (pulsed with NH4Cl) and incubated in Na(+)-free (N-methyl-D-glucamine substitution) solutions (to block Na(+)-dependent pHi regulatory mechanisms), pHi remained acidic (pH approximately 6.5) until the cells were treated with 20 microM forskolin (increases cellular [cAMP]); pHi then increased toward (but not completely to) control level (pHi 7.2) at a rate of 0.055 pH unit/min. Forskolin had no effect on rate of pHi recovery in delta F508 and mock-transfected cells. This Na(+)-independent, forskolin-dependent pHi recovery was not observed in HCO3-/CO2-free medium. Forskolin-treated WT-CFTR-transfected (but not delta F508-CFTR or mock-transfected) cells in Cl(-)-containing, HCO3(-)-free solutions showed Cl- channels with a linear I/V relationship and a conductance of 10.4 +/- 0.5 pS in symmetrical 150 mM Cl-. When channels were incubated with different [Cl-] and [HCO3-] on the inside and outside, the Cl-/HCO3- permeability ratio (determined from reversal potentials of I/V curves) was 3.8 +/- 1.0 (mean +/- SEM; n = 9); the ratio of conductances was 3.9 +/- 0.5 (at 150 mM Cl- and 127 mM HCO3-. We conclude that in acidified cells the WT-CFTR functions as a base loader by allowing a cAMP-dependent influx of HCO3- through channels that conduct HCO3- about one-quarter as efficiently as it conducts Cl-. Under physiological conditions, the electrochemical gradients for both Cl- and HCO3- are directed outward, so CFTR likely contributes to the epithelial secretion of both ions. HCO3- secretion may be important for controlling pH of the luminal, but probably not the cytoplasmic, fluid in CFTR-containing epithelia. In CF, a decreased secretion of HCO3- may lead to decreased pH of the luminal fluid.
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PMID:Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator. 751 98


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