16
Views
0
CrossRef citations to date
0
Altmetric
Miscellaneous

Ongoing planned clinical trials investigating the pulmonary management of cystic fibrosis

Pages 91-98 | Published online: 23 Feb 2005

Bibliography

  • ROMMENS JM, IANNUZZI MC, KEREM B et al.: Identifica-tion of the cystic fibrosis gene: chromosome walking and jumping. Science (1989) 245:1059–1065.
  • RIORDAN JR, ROMMENS JM, KEREM B et al.: Identifica-tion of the cystic fibrosis gene: cloning and characteri-sation of the complementary DNA. Science (1989) 245:1066–1073.
  • KEREM BS, ROMMENS JM, BUCHANAN JA et al.: Identifi-cation of the cystic fibrosis gene: genetic analysis. Sci-ence (1989) 245:1073–1080.
  • ANDERSON DH: Cystic fibrosis of the pancreas and its relation to coeliac disease. A clinical and pathologic study. Am. J. Dis, Child. (1938) 56:344–399.
  • UK cystic fibrosis survey. (1995).
  • ELBORN JS, SHALE DJ, BRITTON JR: Cystic fibrosis: cur-rent survival and population estimates to the year 2000. Thorax (1992) 46:881–885.
  • ALTON EW, MIDDLETON PG, CAPLEN NJ et al.: Non inva-sive liposome mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice. Na-ture Genetics (1993) 5:135–142.
  • ZABNER J, COUTURE LA, GREGORY RJ et al.: Adenovirus mediated gene transfer transiently corrects the chlo-ride transport defect in nasal epithelia of patients with cystic fibrosis. Cell (1993) 75:207–216.
  • CRYSTAL RG, MCELVANEY NG, ROSENFELD MA et al.: Ad-ministration of an adenovirus containing the human CFTR cDNA to the respiratory tract of individuals with cystic fibrosis. Nature Genetics (1994) 8:42–51.
  • WILSON JM, ENGELHARDT JF, GROSSMAN M, SIMON RH, YANG Y: Gene therapy of cystic fibrosis lung disease using an El deleted adenovirus: a Phase I trial. Human Gene Then (1994) 5:501–519.
  • BOUCHER RC, KNOWLES MR, JOHNSON LG et al.: Gene therapy for cystic fibrosis using El deleted adenovi-rus: a Phase I trial in the nasal cavity. Human Gene Then (1994) 5:615–639.
  • SORSCHER EJ, LOGAN JJ, FRIZZELL et al.: Gene therapy for cystic fibrosis using cationic liposome mediated gene transfer: a Phase I trial of safety and efficacy in the nasal airway. Human Gene Then (1994) 5:1259–1277.
  • CAPLEN NJ, ALTON EWFW, MIDDLETON PG et al.: Lipo-some mediated CFTR gene transfer to the nasal epithe-lium of patients with cystic fibrosis. Nature Medicine (1995) 1:39–46.
  • KNOWLES MR, HOHNEKER K, ZHOU Z et al.: A controlledstudy of adenoviral vector mediated gene transfer in the nasal epithelium of patients with cystic fibrosis. New Engl. J. Med. (1995) 333:821–831.
  • BELLON G, MICHEL CALEMARD L, THOUVENOT D et al.: Aerosol administration of a recombinant adenovirus vector expressing CFTR to cystic fibrosis patients: a Phase I clinical trial. Human Gene Then (1997) 8:15–25.
  • GILL DR, SOUTHERN KW, MOFFORD KA et al.: A placebo controlled study of liposome mediated gene transfer to the nasal epithelium of patients with cystic fibrosis. Gene Then (1997) 4:199–209.
  • JOHNSON LG, OLSEN JC, SAKARADI B et al.: Efficiency of gene transfer for restoration of normal airway epithe-lial function in cystic fibrosis. Nature Genetics (1992) 2:21–25.
  • KNOWLES M, STUTTS MJ, SPOCK A et al.: Abnormal ion permeation through cystic fibrosis respiratory epithe-lium. Science (1983) 221:1067–1070.
  • BOUCHER RC, COTTON CU, GATZY JT, KNOWLES MR, YANKASKAS JR: Evidence of reduced a- and increased Isla+ permeability in cystic fibrosis primary cell cul-tures. J. PhysioL (1988) 405:77–103.
  • KNOWLES MR, CHURCH NL, WALTNER et al.: A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. New Engl. J. Med. (1990) 322:1189–1194.
  • APP EM, KING M, HILFESMEDER R, KOHLER D, MATTHYSH: Acute and long term amiloride inhalation in cystic fibrosis lung disease. Am. Rev. Resp, Dis, (1990) 141:605–612.
  • GRAHAM A, HASANI A, ALTON EWFW et al.: No addedbenefit from nebulised amiloride in patients with cys-tic fibrosis. Eur, Resp, J. (1993) 6:1243–1248.
  • KNOWLES MR, OLIVIER KN, HOHNEKER K et at.: Acute safety and effect of aerosolised uridine 5'-triphosphate +/- amiloride. Pediatr, Pulmonol, (1993) 9(Suppl.):148–149.
  • BENNETT WD, OLIVIER KN, ZEMAN KL et al.: Effects ofuridine 5'-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis. Am. J. Respir, Crit, Care Med. (1996) 153:1796–1801.
  • RUBENSTEIN RC, BRUSILOW SW, ZEITLIN PL: A clinicaltrial of oral sodium 4-phenylbutyrate (Bupheny0 for correction of epithelial CFTR function in AF508 homo-zygous cystic fibrosis patients. Am. J. Respir, Grit. Care Med. (1997) 155:A199.
  • EIDELMAN O, GUAY BRODER C, VAN GALEN PJ et al.: Aladenosine receptor antagonists activate chloride ef-flux from cystic fibrosis cells. Proc. Natl. Acad. Sci, USA (1992) 89:5562–5566.
  • POTTER J, MATTHEWS LW, LEMM J, SPECTOR JS: Compo-sition of pulmonary secretions from patients with and without cystic fibrosis. Am. J. Dis, Child. (1960) 100:493–495.
  • MATTHEWS LW, SPECTOR S, LEMM J, POTTER JL: Studieson pulmonary secretions. I. The overall composition of secretions from patients with cystic fibrosis, bron-chiectasis, and laryngectomy. Am. Rev. Respir, Dis, (1963) 88:199–204.
  • PICOT R, DAS I, REID L: Pus deoxyribonucleic acid andsputum viscosity. Thorax (1978) 33:235–242.
  • LETHEM MI, JAMES SL, MARRIOTT C, BURKE JF: The ori-gin of DNA associated with mucus glycoproteins in cystic fibrosis sputum. Eur, Respir, J. (1990) 3:19–23.
  • SHAK S, CAPON DJ, HELLMISS R, MARSTERS SA, BAKERCL: Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc. Natl. Acad. Sci, USA (1990) 87:9188–9192
  • FUCHS HJ, BOROWITZ DS, CHRISTIANSEN DH et at.: Ef-fect of aerosolised recombinant human DNase on ex-acerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. New Engl. J. Med. (1994) 331:637–642.
  • VASCONCELLOS CA, ALLEN PG, WOHL ME etal.: Reduc-tion in viscosity of cystic fibrosis sputum in vitro by gelsolin. Science (1994) 263:969–971.
  • RAMIREZ OE, TOMKIEWICZ RP, DIAN T, KISHIOKA C, RU-BIN BK: The effect of gelsolin on the physical and transport properties of cystic fibrosis sputum in vitro. Pediatr, PulmonoL (1996) 282 (Suppl 13). Abstract.
  • APP EM, BARAN D, TOMKIEWICZ RP et at.: A pilot studzof an aerosolised new mucolytic agent Nacystelyn (NAL) for the treatment of lung disease and cystic fi-brosis. Am. J. Respir, Crit, Care. Med, (1994) 149:A676.
  • BARAN D, APP EM, KING M: Acute, single dose study forefficacy and safety of a new mucolytic agent Nacyste-hyn® (NAL) in a metered dose inhaler for the treatment of lung disease in cystic fibrosis. Proceedings of the 19th European Cystic Fibrosis Conference. Paris, France (1994). Abstract.
  • APP EM, BARAN D, DAB I et al.: Efficacy, safety and toler-ability of chronic inhalation therapy with Nacystelyn® versus placebo in cystic fibrosis patients. Am. J. Respir. Crit. Care Med. (1997) 155:A199.
  • GIROD S, GALABERT C, LECURE A, ZAHM JM, PUCHELLE E: Phospholipid composition and surface active prop-erties of tracheobronchial secretions from patients with cystic fibrosis and chronic obstructive pulmo-nary disease. Pediatr. PulmonoL (1992) 13:22–27.
  • GILLJAM H, ANDERSON O, ELLIN A, ROBERTSON B, STANDVIK B: Composition and surface properties of the bronchial lipids in adult patients with cystic fibro-sis. Clin. Chim. Acta (1988) 176:29–38.
  • RUBIN BK, RAMIREZ OE, SMITH E, BARRETT J: Early re-sults of clinical trials of Exosure for the therapy of cystic fibrosis lung disease. Pediatr. PulmonoL (1995) 234 (Suppl 12). Abstract.
  • GRIESE M, BUFLER P, TELLER D, REINHARDT D: Nebuliza-tion of a bovine surfactant in cystic fibrosis: a pilot study. Eur. Respir. J. (1997) 10:1989–1994.
  • ROBINSON M, HEMMING AL, REGNIS et al.: Effect of in-creasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis. Thorax (1997) 52:900–903.
  • ENG PA, MORTON J, DOUGLASS JA et al.: Short-term effi-cacy of ultrasonically nebulised hypertonic saline in cystic fibrosis. Pediatr. Pulmonol. (1996) 21:77–83.
  • PIER GB, SAUNDERS JM, AMES P et al.: Opsonophago-cytic killing antibody to Pseudomonas aeruginosa mucoid exopolysaccharide in older non-colonised pa-tients with cystic fibrosis. New Engl. J. Med. (1987) 317:793–798.
  • ROSENSTEIN BJ, EIGER H: Risks of alternate day predni-solone in patients with cystic fibrosis. Pediatrics (1991) 87:245–246.
  • KONSTAN MW, BYARD PJ, HOPPEL CL, DAVIS PB: Effectof high dose ibuprofen in patients with cystic fibrosis. New Engl. J. Med. (1995) 332:848–854.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.