New Treatments in Adult Cystic Fibrosis

Corey M , McLaughlin J , Williams M , Levison H. A comparison of survival, growth and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988; 41: 583–91.

Fitzsimmons SC . The changing epidemiology of cystic fibrosis. J Paediatr 1993; 122: 1–9.

Cystic Fibrosis Foundation. Patient Registry 1992 Annual Data Report. Bethesda, Maryland, October 1993.

Elborn JS , Shale DJ , Britton JR . Cystic fibrosis: current survival and population estimates to the year 2000. Thorax 1991; 46: 881–5.

Frederiksen B , Lanng S , Kock C , Hoiby N. Improved survival in the Danish center-treated cystic fibrosis patients: results of aggressive treatment. Paediatr Pulmonol 1996; 21: 153–8.

Fuchs HJ , Borowitz DS , Christiansen DH Effect of aerosolised recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patient with cystic fibrosis. N Engl J Med 1994; 331: 637–42.

Knowles MR , Church NL , Waltner WE A pilot study of aerosolised Amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 1990; 322(17): 1189–94.

Graham A , Hasani A , Alton E.W.F.W. No added benefit from nebulised Amiloride in patients with cystic fibrosis. Eur Resp J 1993; 6: 1243–8.

Corey M , Levison H , Crozier D. Five to seven year course of pulmonary function in cystic fibrosis. Am Rev Respir Dis 1976; 114: 1202–18.

Konstan MW , Byard PJ , Hoppel CL , Davis PB . Effect of high dose ibuprofen in patients with cystic fibrosis. New Engl J Med 1995; 332: 348–54.

Shah PL , Scott SF , Geddes DM , Hodson ME . Two year experience with recombinant human DNase 1 in the treatment of pulmonary disease in cystic fibrosis. Resp Med 1995; 89: 499–502.

Pier GB , Grout M , Zaidi TS , Goldberg JB . How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cvstic fibrosis. Am J Resp Crit Care Med 1996; 154: 5175–82.

Auerbach HS , Williams M , Kirkpatrick JA , Colten HR . Alternate day Prednisolone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 1985; 2: 686–8.

Elgen H , Rosenstein BJ , Fitzsimmons S , Schidlow DV . A multicentre study of alternate-day Prednisolone therapy in patients with cystic fibrosis. From the Cystic Fibrosis Prednisolone Trial Group. J Paediatr 1995; 125(4): 515–23.

Armstrong DS , Grimwood K , Carzino R , Carlin JB , Olinsky A , Phelan PD . Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ 1995; 310: 1571–2.

Khan TZ , Wagener JS , Bost T , Martinez J , Accurso FJ , Riches DWH . Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995; 151: 1075–82.

Tosi MF , Zakem H , Berger M. Neutrophil elastase cleaves C3bi on opsonised Pseudomonas as well as CR1 on neutrophils to create a functionally important opsonin receptor mismatch. J Clin Invest 1990; 83: 300–8.

Fick RB , Nagel GP , Squier SU , Wood RE , Gee JBL , Reynolds HY . Proteins of the cystic fibrosis respiratory tract. Fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis. J Clin Invest 1984; 74: 236–8.

Konstan MW , Hilliard KA , Norvell TM , Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med 1994; 150: 448–54.

McElvaney NG , Hubbard RC , Birrier P Aerosol α₁-anti-trypsin treatment for cystic fibrosis. Lancet 1991; 337: 392–4.

Stolk J , Camps J , Feitsma HIJ , Hermans J , Dijkman JH , Pauwels EKJ . Pulmonary deposition and disappearance of aerosolised secretory leucocyte protease inhibitor. Thorax 1995; 50: 645–50.

Aronoff SC , Quinn FJ , Carpenter LS , Novick WJ . Effects of pentoxifylline on sputum neutrophil elastase and pulmonary function in patients with cystic fibrosis: preliminary observations. J Paediatr 1994; 125: 992–6.