-
1
-
-
0000026508
-
Cystic fibrosis
-
C. R. Scriver, A. L. Beaudet, W. S. Sly, D. Valle, J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, editors. McGraw-Hill, New York
-
Welsh, M. J., L. Tsui, T. F. Boat, and A. L. Beaudet. 1995. Cystic fibrosis. In C. R. Scriver, A. L. Beaudet, W. S. Sly, D. Valle, J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, editors. The Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill, New York. 3799-3876.
-
(1995)
The Metabolic and Molecular Bases of Inherited Disease
, pp. 3799-3876
-
-
Welsh, M.J.1
Tsui, L.2
Boat, T.F.3
Beaudet, A.L.4
-
2
-
-
0020596438
-
Relative ion permeability of normal and cystic fibrosis nasal epithelium
-
Knowles, M., J. Gatzy, and R. Boucher. 1983. Relative ion permeability of normal and cystic fibrosis nasal epithelium. J. Clin. Invest. 71:1410-1417.
-
(1983)
J. Clin. Invest.
, vol.71
, pp. 1410-1417
-
-
Knowles, M.1
Gatzy, J.2
Boucher, R.3
-
3
-
-
0025863209
-
Generation of cAMP-activated chloride currents by expression of CFTR
-
Anderson, M. P., D. P. Rich, R. J. Gregory, A. E. Smith, and M. J. Welsh. 1991. Generation of cAMP-activated chloride currents by expression of CFTR. Science 251:679-682.
-
(1991)
Science
, vol.251
, pp. 679-682
-
-
Anderson, M.P.1
Rich, D.P.2
Gregory, R.J.3
Smith, A.E.4
Welsh, M.J.5
-
6
-
-
0027520191
-
Amiloride inhalation therapy in cystic fibrosis: Influence on ion content, hydration, and rheology of sputum
-
Tomkiewicz, R. P., E. M. App, J. G. Zayas, O. Ramirez, N. Church, R. C. Boucher, M. R. Knowles, and M. King. 1993. Amiloride inhalation therapy in cystic fibrosis: influence on ion content, hydration, and rheology of sputum. Am. Rev. Respir. Dis. 148:1002-1007.
-
(1993)
Am. Rev. Respir. Dis.
, vol.148
, pp. 1002-1007
-
-
Tomkiewicz, R.P.1
App, E.M.2
Zayas, J.G.3
Ramirez, O.4
Church, N.5
Boucher, R.C.6
Knowles, M.R.7
King, M.8
-
7
-
-
0019490443
-
Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo
-
Knowles, M. R., J. L. Carson, A. M. Collier, J. T. Gatzy, and R. C. Boucher. 1981. Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo. Am. Rev. Respir. Dis. 124:484-490.
-
(1981)
Am. Rev. Respir. Dis.
, vol.124
, pp. 484-490
-
-
Knowles, M.R.1
Carson, J.L.2
Collier, A.M.3
Gatzy, J.T.4
Boucher, R.C.5
-
8
-
-
0022588248
-
Abnormal respiratory epithelial ion transport in cystic fibrosis
-
Knowles, M. R., M. J. Stutts, J. R. Yankaskas, J. T. Gatzy, and R. C. Boucher, Jr. 1986. Abnormal respiratory epithelial ion transport in cystic fibrosis. Clin. Chest Med. 7:285-297.
-
(1986)
Clin. Chest Med.
, vol.7
, pp. 285-297
-
-
Knowles, M.R.1
Stutts, M.J.2
Yankaskas, J.R.3
Gatzy, J.T.4
Boucher Jr., R.C.5
-
9
-
-
0019809960
-
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis
-
Knowles, M. R., J. Gatzy, and R. Boucher. 1981. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N. Engl. J. Med. 305:1489-1495.
-
(1981)
N. Engl. J. Med.
, vol.305
, pp. 1489-1495
-
-
Knowles, M.R.1
Gatzy, J.2
Boucher, R.3
-
10
-
-
0030982349
-
Aerosolized amiloride: Dose-effect on nasal bioelectric properties, pharmacokinetics, and effect on sputum expectoration in patients with cystic fibrosis
-
Hofmann, T., I. Senier, P. Bittner, G. Huels, H. J. Schwandt, and H. Lindemann. 1997. Aerosolized amiloride: dose-effect on nasal bioelectric properties, pharmacokinetics, and effect on sputum expectoration in patients with cystic fibrosis. J. Aerosol Med. 10:147-158.
-
(1997)
J. Aerosol Med.
, vol.10
, pp. 147-158
-
-
Hofmann, T.1
Senier, I.2
Bittner, P.3
Huels, G.4
Schwandt, H.J.5
Lindemann, H.6
-
11
-
-
0022988338
-
Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses
-
Kohler, D., E. App, M. Schmitz-Schumann, G. Wuertemberger, and H. Matthys. 1986. Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses. Eur. J. Respir. Dis. 69(Suppl. 146):319-326.
-
(1986)
Eur. J. Respir. Dis.
, vol.69
, Issue.146 SUPPL.
, pp. 319-326
-
-
Kohler, D.1
App, E.2
Schmitz-Schumann, M.3
Wuertemberger, G.4
Matthys, H.5
-
12
-
-
0025274624
-
Acute and long-term amiloride inhalation in cystic fibrosis lung disease: A rational approach to cystic fibrosis therapy
-
App, E. M., M. King, R. Helfesrieder, D. Kohler, and H. Matthys. 1990. Acute and long-term amiloride inhalation in cystic fibrosis lung disease: a rational approach to cystic fibrosis therapy. Am. Rev. Respir. Dis. 141:605-612.
-
(1990)
Am. Rev. Respir. Dis.
, vol.141
, pp. 605-612
-
-
App, E.M.1
King, M.2
Helfesrieder, R.3
Kohler, D.4
Matthys, H.5
-
13
-
-
0028787073
-
Nebulized amiloride in respiratory exacerbations of cystic fibrosis: A randomised controlled trial
-
Bowler, I. M., B. Kelman, D. Worthington, J. M. Littlewood, A. Watson, S. P. Conway, S. W. Smye, S. L. James, and T. A. Sheldon. 1995. Nebulized amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial. Arch. Dis. Child. 73:427-430.
-
(1995)
Arch. Dis. Child.
, vol.73
, pp. 427-430
-
-
Bowler, I.M.1
Kelman, B.2
Worthington, D.3
Littlewood, J.M.4
Watson, A.5
Conway, S.P.6
Smye, S.W.7
James, S.L.8
Sheldon, T.A.9
-
14
-
-
0029988412
-
Concentration of inhaled amiloride in cystic fibrosis
-
Visca, A., and E. Bignamini. 1996. Concentration of inhaled amiloride in cystic fibrosis. Lancet 347:1126.
-
(1996)
Lancet
, vol.347
, pp. 1126
-
-
Visca, A.1
Bignamini, E.2
-
15
-
-
0029916071
-
Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis
-
Robinson, M., J. A. Regnis, D. L. Bailey, M. King, G. J. Bautovich, and P. T. Bye. 1996. Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis. Am. J. Respir. Cril. Care Med. 153:1503-1509.
-
(1996)
Am. J. Respir. Cril. Care Med.
, vol.153
, pp. 1503-1509
-
-
Robinson, M.1
Regnis, J.A.2
Bailey, D.L.3
King, M.4
Bautovich, G.J.5
Bye, P.T.6
-
16
-
-
0029887172
-
Effect of uridine 5′-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis
-
Bennett, W. D., K. N. Olivier, K. L. Zeman, K. W. Hohneker, R. C. Boucher, and M. R. Knowles. 1996. Effect of uridine 5′-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis. Am. J. Respir. Crit. Care Med. 153:1796-1801.
-
(1996)
Am. J. Respir. Crit. Care Med.
, vol.153
, pp. 1796-1801
-
-
Bennett, W.D.1
Olivier, K.N.2
Zeman, K.L.3
Hohneker, K.W.4
Boucher, R.C.5
Knowles, M.R.6
-
17
-
-
0025236867
-
A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
-
Knowles, M. R., N. L. Church, W. E. Waltner, J. R. Yankaskas, P. H. Gilligan, M. King, L. J. Edwards, R. W. Helms, and R. C. Boucher. 1990. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N. Engl. J. Med. 322:1189-1194.
-
(1990)
N. Engl. J. Med.
, vol.322
, pp. 1189-1194
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
Yankaskas, J.R.4
Gilligan, P.H.5
King, M.6
Edwards, L.J.7
Helms, R.W.8
Boucher, R.C.9
-
18
-
-
0027486593
-
No added benefit from nebulized amiloride in patients with cystic fibrosis
-
Graham, A., A. Hasani, E. W. F. W. Alton, G. P. Martin, C. Marriott, M. E. Hodson, S. W. Clarke, and D. M. Geddes. 1993. No added benefit from nebulized amiloride in patients with cystic fibrosis. Eur. Respir. J. 6:1243-1248.
-
(1993)
Eur. Respir. J.
, vol.6
, pp. 1243-1248
-
-
Graham, A.1
Hasani, A.2
Alton, E.W.F.W.3
Martin, G.P.4
Marriott, C.5
Hodson, M.E.6
Clarke, S.W.7
Geddes, D.M.8
-
19
-
-
0000930452
-
Amiloride in cystic fibrosis: Safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease
-
E. J. Cragoe, Jr., T. R. Kleyman, and L. Simchowitz, editors. VCH Publishers, New York
-
Knowles, M. R., N. L. Church, W. E. Waltner, J. T. Gatzy, and R. C. Boucher. 1992. Amiloride in cystic fibrosis: safety, pharmacokinetics, and efficacy in the treatment of pulmonary disease. In E. J. Cragoe, Jr., T. R. Kleyman, and L. Simchowitz, editors. Amiloride and Its Analogs: Unique Cation Transport Inhibitors. VCH Publishers, New York. 301-316
-
(1992)
Amiloride and its Analogs: Unique Cation Transport Inhibitors
, pp. 301-316
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
Gatzy, J.T.4
Boucher, R.C.5
-
20
-
-
0029584796
-
Effects of benzamil in human cystic fibrosis airway epithelium
-
Blank, U., W. Clauss, and W. M. Weber. 1995. Effects of benzamil in human cystic fibrosis airway epithelium. Cell Physiol. Biochem. 5:385-390.
-
(1995)
Cell Physiol. Biochem.
, vol.5
, pp. 385-390
-
-
Blank, U.1
Clauss, W.2
Weber, W.M.3
-
21
-
-
0028982894
-
CFTR as a cAMP-dependent regulator of sodium channels
-
Stutts, M. J., C. M. Canessa, J. C. Olsen, M. Hamrick, J. A. Cohn, B. C. Rossier, and R. C. Boucher. 1995. CFTR as a cAMP-dependent regulator of sodium channels. Science 269:847-850.
-
(1995)
Science
, vol.269
, pp. 847-850
-
-
Stutts, M.J.1
Canessa, C.M.2
Olsen, J.C.3
Hamrick, M.4
Cohn, J.A.5
Rossier, B.C.6
Boucher, R.C.7
-
22
-
-
0344040199
-
An overview of the structure-activity relations in the amiloride series
-
E. J. Cragoe, T. R. Kleyman, and L. Simchowitz, editors. VCH Publishers, New York
-
Simchowitz, L., T. R. Kleyman, and E. J. Cragoe. 1992. An overview of the structure-activity relations in the amiloride series. In E. J. Cragoe, T. R. Kleyman, and L. Simchowitz, editors. Amiloride and Its Analogs. VCH Publishers, New York. 9-24.
-
(1992)
Amiloride and its Analogs
, pp. 9-24
-
-
Simchowitz, L.1
Kleyman, T.R.2
Cragoe, E.J.3
-
23
-
-
0025997759
-
Sodium transport and intracellular sodium activity in cultured human nasal epithelium
-
Willumsen, N. J., and R. C. Boucher. 1991. Sodium transport and intracellular sodium activity in cultured human nasal epithelium. Am. J. Physiol. 261:C319-C331.
-
(1991)
Am. J. Physiol.
, vol.261
-
-
Willumsen, N.J.1
Boucher, R.C.2
-
25
-
-
0002781622
-
Blood and nerve supply of the nasal lining
-
D. F. Proctor and I. B. Andersen, editors. Elsevier Biomedical Press, Amsterdam
-
Cauna, N. 1982. Blood and nerve supply of the nasal lining. In D. F. Proctor and I. B. Andersen, editors. The Nose. Upper Airway Physiology and the Atmospheric Environment. Elsevier Biomedical Press, Amsterdam. 45-69.
-
(1982)
The Nose. Upper Airway Physiology and the Atmospheric Environment
, pp. 45-69
-
-
Cauna, N.1
-
26
-
-
0022396481
-
Culture of human nasal epithelial cells on collagen matrix supports
-
Yankaskas, J. R., C. U. Cotton, M. R. Knowles, J. T. Gatzy, and R. C. Boucher. 1985. Culture of human nasal epithelial cells on collagen matrix supports. Am. Rev. Respir. Dis. 132:1281-1287.
-
(1985)
Am. Rev. Respir. Dis.
, vol.132
, pp. 1281-1287
-
-
Yankaskas, J.R.1
Cotton, C.U.2
Knowles, M.R.3
Gatzy, J.T.4
Boucher, R.C.5
-
27
-
-
0004282518
-
-
SAS Institute, Inc. SAS Institute, Inc., Cary, NC
-
Anonymous. 1990. SAS Institute, Inc. SAS/STAT Users Guide, Version 6, SAS Institute, Inc., Cary, NC.
-
(1990)
SAS/STAT Users Guide, Version 6
-
-
-
28
-
-
0023275376
-
Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis
-
Alton, E. W., J. G. Hay, C. Munro, and D. M. Geddes. 1987. Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis. Thorax 42:815-817.
-
(1987)
Thorax
, vol.42
, pp. 815-817
-
-
Alton, E.W.1
Hay, J.G.2
Munro, C.3
Geddes, D.M.4
-
29
-
-
0025609981
-
Nasal potential difference: A clinical diagnostic test for cystic fibrosis
-
Alton, E. W. F. W., D. Currie, R. Logan-Sinclair, J. O. Warner, M. E. Hodson, and D. M. Geddes. 1990. Nasal potential difference: a clinical diagnostic test for cystic fibrosis. Eur. Respir. J. 3:922-926.
-
(1990)
Eur. Respir. J.
, vol.3
, pp. 922-926
-
-
Alton, E.W.F.W.1
Currie, D.2
Logan-Sinclair, R.3
Warner, J.O.4
Hodson, M.E.5
Geddes, D.M.6
-
30
-
-
0025134519
-
Sekretelimination bei CF-Patienten unter Amiloridinhalation
-
Lindemann, H., T. Becker, P. Bittner, A. Boldt, T. Hofmann, and H. J. Schwandt. 1990. Sekretelimination bei CF-Patienten unter Amiloridinhalation [Elimination of secretions in CF patients under amiloride inhalation]. Pneumologie 44:1148-1150.
-
(1990)
Pneumologie
, vol.44
, pp. 1148-1150
-
-
Lindemann, H.1
Becker, T.2
Bittner, P.3
Boldt, A.4
Hofmann, T.5
Schwandt, H.J.6
-
31
-
-
0031006817
-
Conventional and modified nasal potential difference measurement: Clinical use in cystic fibrosis
-
Hofmann, T., O. Böhmer, P. Bittner, G. Hüls, H. G. Terbrack, E. Heerd, and H. Lindemann. 1997. Conventional and modified nasal potential difference measurement: clinical use in cystic fibrosis. Am. J. Respir. Crit. Care Med. 155:1908-1913.
-
(1997)
Am. J. Respir. Crit. Care Med.
, vol.155
, pp. 1908-1913
-
-
Hofmann, T.1
Böhmer, O.2
Bittner, P.3
Hüls, G.4
Terbrack, H.G.5
Heerd, E.6
Lindemann, H.7
-
32
-
-
0344471348
-
Dexamethasone increases sodium transport in human nasal epithelium in vivo
-
Hofmann, T., C. E. Foy, J. M. Robinson, V. Homolya, R. C. Boucher, and M. R. Knowles. 1997. Dexamethasone increases sodium transport in human nasal epithelium in vivo. Ped. Pulm. (Suppl. 14):234.
-
(1997)
Ped. Pulm.
, Issue.14 SUPPL.
, pp. 234
-
-
Hofmann, T.1
Foy, C.E.2
Robinson, J.M.3
Homolya, V.4
Boucher, R.C.5
Knowles, M.R.6
-
33
-
-
22644445283
-
Inhibitory potency of amiloride, benzamil and phenamil on sodium conductances in primary cultured cystic fibrosis nasal epithelium
-
Blank, U., T. Hofmann, W. Clauss, H. Lindemann, G. Hüls, and W. Weber. 1996. Inhibitory potency of amiloride, benzamil and phenamil on sodium conductances in primary cultured cystic fibrosis nasal epithelium (abstract). Isr. J. Med. Sci. 32:S225.
-
(1996)
Isr. J. Med. Sci.
, vol.32
-
-
Blank, U.1
Hofmann, T.2
Clauss, W.3
Lindemann, H.4
Hüls, G.5
Weber, W.6
-
34
-
-
0026332905
-
Effect of benzamil on sheep tracheal epithelium
-
Acevedo, M., R. E. Olver, and M. R. Ward. 1991. Effect of benzamil on sheep tracheal epithelium. Exp. Physiol. 76:935-941.
-
(1991)
Exp. Physiol.
, vol.76
, pp. 935-941
-
-
Acevedo, M.1
Olver, R.E.2
Ward, M.R.3
-
35
-
-
0030898750
-
cGMP stimulates sodium and chloride currents in rat tracheal airway epithelia
-
Schwiebert, E. M., E. D. Potter, T. H. Hwang, J. S. Woo, C. Ding, W. Qiu, W. B. Guggino, M. A. Levine, and S. E. Guggino. 1997. cGMP stimulates sodium and chloride currents in rat tracheal airway epithelia. Am. J. Physiol. 212:C911-C022.
-
(1997)
Am. J. Physiol.
, vol.212
-
-
Schwiebert, E.M.1
Potter, E.D.2
Hwang, T.H.3
Woo, J.S.4
Ding, C.5
Qiu, W.6
Guggino, W.B.7
Levine, M.A.8
Guggino, S.E.9
-
36
-
-
0028859727
-
Effect of central infusion of benzamil on Dahl S rat hypertension
-
Gomez-Sanchez, E. P., and C. E. Gomez-Sanchez. 1995. Effect of central infusion of benzamil on Dahl S rat hypertension. Am. J. Physiol. 269: H1044-H1047.
-
(1995)
Am. J. Physiol.
, vol.269
-
-
Gomez-Sanchez, E.P.1
Gomez-Sanchez, C.E.2
|