Doubling time α-aminoisobutyrate transport and calcium exchange in cultured fibroblasts from cystic fibrosis and control subjects

1978 ◽  
Vol 82 (1-2) ◽  
pp. 125-131 ◽  
Author(s):  
Burton L. Shapiro ◽  
Robert J. Feigal ◽  
Nancy J. Laible ◽  
Michelle H. Biros ◽  
Warren J. Warwick
1984 ◽  
Vol 18 (12) ◽  
pp. 1336-1339 ◽  
Author(s):  
Richard C Boucher ◽  
Dennis W Ross ◽  
Michael R Knowles ◽  
John T Gatzy ◽  
John C Parker

PEDIATRICS ◽  
1968 ◽  
Vol 42 (3) ◽  
pp. 458-464
Author(s):  
Lewis E. Gibson

An abnormality of the autonomic nervous system has frequently been postulated as having a role in the pathogenesis of cystic fibrosis. The author investigated the effects of adrenergic stimulation upon sweating in cystic fibrosis patients and in control subjects. It was found that the iontophoresis of isoproterenol, prior to the iontophoresis of pilocarpine, produces, in all subjects, more sweat than that produced by the iontophoresis of pilocarpine alone. In the cystic fibrosis patients, but not in the control subjects, the iontophoresis of isoproterenol led to a small (8.9%) but significant drop in the chloride concentration of the sweat. Sweating produced by the intracutaneous injection of isoproterenol, but not that produced by intracutaneous norepinephrine, could be blocked by the iontophoresis of atropine. This occurred in both cystic fibrosis patients and control subjects. These experimental results can be explained by hypothesizing that excessive water reabsorption occurs in the sweat duct of cystic fibrosis patients and that isoproterenol inhibits ductal water reabsorption in both the cystic fibrosis patients and the unaffected controls.


1987 ◽  
Vol 66 (2_suppl) ◽  
pp. 654-659 ◽  
Author(s):  
K.T. Izutsu ◽  
M.M. Schubert ◽  
E.L. Truelove ◽  
D.E. Johnson

Previous findings from studies utilizing human labial and palatine minor salivary glands are reviewed. These studies took histopathological, biochemical, and ultrastructural approaches, and focused on control and diseased glands. Disease-oriented summarizations are used, and control results are discussed in the context of disease-related findings. Findings are reviewed separately for electrolytes, macromolecules, and ultrastructure. In control subjects, minor gland salivary electrolyte concentrations are dependent on flow rate, and this dependence may be altered by diseases such as cystic fibrosis as-well as by inflammatory situations such as graft-versus-host disease. There is also evidence that salivary electrolyte secretion processes are not similar in labial and palatine minor glands. Studies of salivary macromolecular composition are reviewed for control subjects and for patients with graft-versus-host disease and Sjögren's syndrome. The findings indicate that the macromolecular contents of labial and palatine gland saliva are similar, but that both are significantly different from that for major gland saliva. Finally, studies attempting to measure disease-related changes in intracellular composition are reviewed. It is concluded that the minor salivary glands are important models for the study of exocrine gland physiology and pathophysiology in man.


PEDIATRICS ◽  
1974 ◽  
Vol 53 (4) ◽  
pp. 584-585
Author(s):  
Lewis E. Gibson

The authors of the recent article which failed to show a significant difference between sweat calcium concentrations of CF patients and control subjects, suggest that the findings refute our hyperpermeability hypothesis. They found sweat Ca of 0.32 to 1.67 mEq/liter in 26 control subjects and 0.42 to 2.03 mEq/liter in 16 CF patients. With these tremendous ranges many more subjects would be required to prove or disprove a difference. A difference undetectable in a moderate number of subjects is probably not of pathophysiologic significance if the Ca really varies unpredictably.


1984 ◽  
Vol 73 (2) ◽  
pp. 263-266 ◽  
Author(s):  
A. BARDON ◽  
O. CEDER ◽  
G. EKBOHM ◽  
H. KOLLBERG

PLoS ONE ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. e0245696
Author(s):  
Daniel Aridgides ◽  
John Dessaint ◽  
Graham Atkins ◽  
James Carroll ◽  
Alix Ashare

Data on adverse events from research bronchoscopy with bronchoalveolar lavage (BAL) in patients with cystic fibrosis (CF) is lacking. As research bronchoscopy with BAL is useful for isolation of immune cells and investigation of CF lung microbiome, we sought to investigate the safety of bronchoscopy in adult patients with CF. Between November 2016 and September 2019, we performed research bronchoscopies on CF subjects (32) and control subjects (82). Control subjects were nonsmokers without respiratory disease. CF subjects had mild or moderate obstructive lung disease (FEV1 > 50% predicted) and no evidence of recent CF pulmonary exacerbation. There was no significant difference in the age or sex of each cohort. Neither group experienced life threatening adverse events. The number of adverse events was similar between CF and control subjects. The most common adverse events were sore throat and cough, which occurred at similar frequencies in control and CF subjects. Fever and headache occurred more frequently in CF subjects. However, the majority of fevers were seen in CF subjects with FEV1 values below 65% predicted. We found that CF subjects had similar adverse event profiles following research bronchoscopy compared to healthy subjects. While CF subjects had a higher rate of fevers, this adverse event occurred with greater frequency in CF subjects with lower FEV1. Our data demonstrate that research bronchoscopy with BAL is safe in CF subjects and that safety profile is improved if bronchoscopies are limited to subjects with an FEV1 > 65% predicted.


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