Cystic fibrosis in adults from researcher to practitioner

Western Journal of Medicine

Volumn 164, Issue 4, 1996, Pages 321-334

  Marelich, Gregory P ^a,b   Cross, Carroll E ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; AMILORIDE; AMINOGLYCOSIDE ANTIBIOTIC AGENT; ANTIBIOTIC AGENT; AZTREONAM; CEFTAZIDIME; CILASTATIN PLUS IMIPENEM; CIPROFLOXACIN; CONJUGATED ESTROGEN; CORTICOSTEROID; DEOXYRIBONUCLEASE I; ESTROGEN; GELSOLIN; IBUPROFEN; KETOCONAZOLE; LEUKOCYTE ELASTASE; LIPOSOME; PENTOXIFYLLINE; PIPERACILLIN; PREDNISONE; PSEUDOMONAS VACCINE; RECOMBINANT DEOXYRIBONUCLEASE; TICARCILLIN; TOBRAMYCIN; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG; URIDINE TRIPHOSPHATE; VASOPRESSIN; VIRUS VECTOR;

ADENOVIRUS; AEROSOL; ARTIFICIAL EMBOLISM; ASPERGILLOSIS; ASPERGILLUS FUMIGATUS; BURKHOLDERIA CEPACIA; CLINICAL TRIAL; CONFERENCE PAPER; CYSTIC FIBROSIS; EMPLOYMENT; FORCED EXPIRATORY VOLUME; GENE TRANSFER; HEART LUNG TRANSPLANTATION; HEMOPTYSIS; HEPATOBILIARY DISEASE; HUMAN; IMMUNE RESPONSE; INFLAMMATION; INHALATIONAL DRUG ADMINISTRATION; INTRAVENOUS DRUG ADMINISTRATION; KINESIOTHERAPY; LUNG CLEARANCE; LUNG TRANSPLANTATION; MALABSORPTION; NUTRITION; PARANASAL SINUS DISEASE; PHASE 3 CLINICAL TRIAL; PNEUMOTHORAX; POLYARTHRITIS; PSEUDOMONAS AERUGINOSA; RESPIRATORY TRACT INFECTION; SEPSIS; SEXUALITY; SPUTUM ANALYSIS; STAPHYLOCOCCUS AUREUS;

ADULT; ANTI-BACTERIAL AGENTS; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EXERCISE THERAPY; FEMALE; GENE THERAPY; GENE TRANSFER TECHNIQUES; HUMANS; LUNG TRANSPLANTATION; MALE; SPUTUM;

EID: 0029965836     PISSN: 00930415     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Conference Paper

References (147)

1. Fanconi G, Uehlinger E, Knauer C: Das coeliakiesyndrom bei angeborener zysticher pankreasfibromatose und bronkiektasen. Wien Med Wochenschr 1936; 86. 753-756
2. Anderson DH: Cystic fibrosis of the pancreas and its relation to celiac disease: Clinical and pathological study Am J Dis Child 1938; 56-344-399
3. Matthews LW, Doershuk CF, Wise M, Eddy G, Nudelman H. Spector S: A therapeutic regimen for patients with cystic fibrosis. J Pediatr 1964; 65: 558-575
4. Patient Registry 1994 Annual Data Report, Bethesda. Md, Cystic Fibrosis Foundation, August 1995
5. Wilson JM: Prospects for Human Gene Therapy for Cystic Fibrosis. Plenary session. Eighth Annual North American Cystic Fibrosis Conference, Orlando, Fla, October 1994
6. Eiberg H, Mohr J. Schmiegelow K, Nielsen LS, Williamson R. Linkage relationships of paraoxinase (PON) with other markers: Evidence of PON-cystic fibrosis synteny. Clin Genet 1985, 28 265-271
7. Tsui LC, Buchwald M, Barker D, et al: Cystic fibrosis locus defined by a genetically linked polymorphic marker. Science 1985; 230:1054-1057
8. Rommens JM, Ianuzzi MC, Kerem BS, et al: Identification of the cystic fibrosis gene. Chromosome walking and jumping. Science 1989; 245.1059-1065
9. Riordan JR, Rommens JM, Kerem BS. et al: Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 1989; 245.1066-1072
10. Kerem BS, Rommens JM, Buchanan JA. et al: Identification of the cystic fibrosis gene: Genetic analysis. Science 1989; 245:1073-1080
11. Engelhardt JF, Yankaskas JR. Ernst SA: Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nature Genet 1992; 2:240-248
12. Crawford I, Maloney P, Zeitlin PL. et al: Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci USA 1991; 88:9262-9266
13. Cohn JA, McGill J, Basavappa S, Kole J, Fitz JG: Bile duct epithelial cells are the predominant site of CFTR in liver. Pediatr Pulmonol Suppl 1992; 8.249
14. Miller PW: CFTR mutations and adult pulmonary diseases. Pediatr Pulmonol Suppl 1994; 10:137
15. Zielenski J, Tsui LC: Cystic fibrosis genotype and phenotype variations, Annu Rev Genet 1995: 29-777-807
16. Cystic Fibrosis Genetic Analysis Consortium: Population variation of common cystic fibrosis mutations. Hum Mutat 1994; 4:167-177
17. Gadsby DC, Nairn AC. Regulation of CFTR channel gating Trends Biochem Sci 1994; 19:513-518
18. Gabnel SE, Bngman KN, Koller BH, Boucher RC, Stutts MJ. Cystic fibrosis heterozysote resistance to cholera toxin in the cystic fibrosis mouse model. Science 1994; 266.107-109
19. Goldstein JL, Sahi J, Bhuva M, Layden TJ. Rao MC Eschericia coli heat-stable enterotoxin-mediated colonie Cl- secretion is absent in cystic fibrosis. Gastroenterology 1994; 107 950-956
20. Tsui LC: The cystic fibrosis transmembrane conductance regulator gene. Am J Respir Crit Care Med 1995; 151.S47-S53
21. Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 1993; 73.1251-1254
22. Knowles MR, Gatzy J, Boucher R: Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med 1981: 305:1489-1495
23. Boucher RC. Human airway ion transport (pt 1) Am J Respir Crit Care Med 1994; 150 271-281
24. Boucher RC: Human airway ion transport (pt 2). Am J Respir Crit Care Med 1994. 150:581-593
25. + channel subunits in normal and cystic fibrosis airways Am J Physiol 1995; 269:C511-C518
26. Stutts MJ, Canessa CM, Olsen JC, et al CFTR as a cAMP-dependent regulator of sodium channels. Science 1995; 269:847-850
27. Schwiebert EM, Egan ME, Hwang TH. et al: CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 1995; 81:1063-1073
28. Hamosh A, Corey M. and the Cystic Fibrosis Genotype-Phenotype Consortium: Correlation between genotype and phenotype in cystic fibrosis. N Engl J Med 1993. 329:1308-1313
29. Denning GM, Ostedgaard LS, Welsh MJ: Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia J Cell Biol 1992; 118:551-559
30. - channels with altered pore properties. Nature 1993; 362:160-164
31. Imundo L, Barasch J, Prince A, Al-Awqati Q: Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA 1995; 92:3019-3023
32. Warner JO: Immunology of cystic fibrosis Br Med Bull 1992; 48: 893-911
33. Roussey M, Lamarre A, Marcotte JE. et al: Pulmonary function and clinical course in 252 CF patients after colonization with Pseudomonas aeruginosa (pa), cepacia and Xanthomonas maltophilia. Pediatr Pulmonol Suppl 1994; 10:253
34. Schuster A, Fahy JV, Ueki I, Nadel JA: Cystic fibrosis sputum induces a secretory response from airway gland serous cells that can be prevented by neutrophil protease inhibitors. Eur Respir J 1995: 8:1-14
35. Widdicombe JH: Accumulation of airway mucus in cystic fibrosis. Pulmon Pharmacol 1994; 7:225-233
36. Regnis JA, Robinson M, Bailey DL. et al: Mucociliary clearance in patients with cystic fibrosis and in normal subjects. Am J Respir Crit Care Med 1994; 150:66-71
37. Dupuit F, Kalin N, Brézillon S, Hinnrasky J, Tummler B, Puchelle E: CFTR and differentiation markers expression in non-CF and ΔF508 homozygous CF nasal epithelium. J Clin Invest 1995; 96:1601-1611
38. Hingley ST, Hastie AT, Kueppers F, Higgins ML, Weinbaum G, Shryock T: Effect of ciliostaitc factors from Pseudomonas aeruginosa on rabbit respiratory cilia. Infect Immun 1986; 51:254-262
39. Fick RB, Baltimore RS, Squier SV. Reynolds HY: IgG proteolytic activity of Pseudomonas aeraginosa in cystic fibrosis. J Infect Dis 1985; 151:589-598
40. Wood DE, To M. Sohol PA: Pseudomonas aeruginosa exoenzymes as a pathogenic determinant in respiratory infections. Antibiot Chemother 1989, 42:27-35
41. Nelson JW. Tredgett MW, Sheehan JK, Thornton DJ, Notman D, Govan JRW: Mucinophilic and chemotactic properties of Pseudomonas aeruginosa in relation to pulmonary colonization in cystic fibrosis. Infect Immun 1990; 58:1489-1495
42. Govan JRW, Nelson JW, Microbiology of lune infection in cystic fibrosis. Br Med Bull 1992, 48.912-930
43. Speert DP, Farmer SW, Campbell ME, Musser JM, Selander RK, Kuo S: Conversion of Pseudomonas aeruginosa to the phenotype characteristic of strains from patients with cystic fibrosis J Clin Microbiol 1990; 28:188-194
44. Zielinski NA, Maharaj R, Roychoudhury S, Danganan C, Hendrickson W, Chakrabarty AM. Alginate synthesis in Pseudomonas aerugmosa: Environmental regulation of the algC promoter. J Bacteriol 1992: 174.7680-7688
45. Lam J, Chan R, Lam K, Costerton JW: Production of mucoid microcolonies by Pseudomonas aeruginosa within infected lungs in cystic fibrosis Infect Immun 1980; 28:546-556
46. Bruce MC, Poncz L, Klinger JD, Stem RC, Tomashefski JF Jr, Dearborn DG: Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibroais Am Rev Respir Dis 1985; 132:529-535
47. 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; 150:448-454
48. Birrer P, McElvaney NG, Rudeberg A. et al Protease-antiprotease imbalance in the lungs of children with cystic fibrosis Am J Respir Cm Care Med 1994; 150:207-213
49. Kilby JM, Gilligan PH, Yankaskas JR, Highsmith WE Jr, Edwards LJ, Knowles MR. Nontuberculous mycobacteria in adult patients with cystic fibrosis. Chest 1992; 102:70-75
50. Pegues DA, Carson LA, Tablan OC. et al, and the Summer Camp Study Group: Acquisition of Pseudomonas cepacia at summer camps for patients, with cystic fibrosis. J Pediatr 1994; 124:694-702
51. Steinbach S, Sun L, Jiang RZ, et al: Transmissibility of Pseudomonas cepacia infection in clinic patients and Iung-transplant recipients with cystic fibrosis. N Engl J Med 1994; 331:981-987
52. Lindsey CA, Bosso JA: Optimisation of antibiotic therapy in cystic fibrosis patients. Clin Pharmacokinet 1993; 24.496-506
53. Mannes GPM, van Aalderen WMC, Manson WL: Tobramycin in a single daily dose in adult CF patients. Pediatr Pulmonol Suppl 1994; 10:259
54. Vic P, Ategbo S, Turck D. et al: Efficacy and tolerance of once-daily amikacin (AM) in cystic fibrosis (CF) patients. Pediatr Pulmonol Suppl 1994; 10:251
55. Mendelman PM, Smith AL, Levy J, Weber A, Ramsey B, Davis RL: Aminoglycoside penetration, inactivation, and efficacy in cystic fibrosis sputum Am Rev Respir Dis 1985; 132:761-765
56. van Aalderen WMC, Mannes GPM, Bosma ES, Roorda RJ, Heymans HSA: Home care in cystic fibrosis patients. Eur Respir J 1995; 8:172-175
57. Pleasants RA, Walker TR, Samuelson WM: Allergic reactions to parenteral beta-lactam antibiotics in patients with cystic fibrosis. Chest 1994; 106:1124-1128
58. Ramsey BW, Dorkin HL, Eisenberg JD, et al: Efficacy of aerosolized tobramycin in patients with cystic fibrosis. N Engl J Med 1993; 328:1740-1746
59. Steinkamp G, Tümmler B, Gappa M, et al: Longterm tobramycin aerosol therapy in cystic fibrosis. Pediatr Pulmonol 1989; 6:91-98
60. MacLusky IB, Gold R, Corey M, Levison H: Long-term effects of inhaled tobramycin in patients with cystic fibrosis colonized with Pseudomonas aeruginosa. Pediatr Pulmonol 1989; 7:42-48
61. Smith JA' Neutrophils, host defense, and inflammation. A double-edged sword J Leukoc Biol 1994; 56:672-686
62. Auerbach HS, Kirkpatrick JA, Williams M, Colten HR: Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 1985; 2:686-688
63. Eigen H, Rosenstein BJ, FitzSimmons S, Schidlow DV, and the Cystic Fibrosis Foundation Prednisone Trial Group. A multicenter study of alternateday. prednisone therapy in patients with cystic fibrosis. J Pediatr 1995, 126: 515-523
64. Konstan MW, Byard PJ, Hoppel CL, Davis PB: Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995; 332:848-854
65. 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 Pediatr 1994; 125:992-997
66. Rees DD, Brain JD, Effects of cystic0 fibrosis airway secretions on rat lung: Role of neutrophil elastase. Am J Physiol 1995; 269:L195-L202
67. Cryz SJ Jr, Wedgwood J, Lang AB. et al. Immunization of noncolonized cystic fibrosis patients against Pseudomonas aeruginosa. J Infect Dis 1994; 169:1159-1162
68. Dune PR, Pencharz PB: Nutritional deficiencies in cystic fibrosis. Br Med Bull 1992; 48:823-847
69. Kraisinger M, Hochhaus G, Stecenko A, Bowser E, Hendeles L. Clinical pharmacology of pancreatic enzymes in patients with cystic fibrosis and in vitro performance of microencapsulated formulations. J Clin Pharmacol 1994: 34:158-166
70. Smyth RL, Van Aelzen D, Smyth AR, Lloyd DA, Heaf DP: Strictures of the ascending colon in cystic fibrosis and high-strength pancreatic enzymes Lancet 1994; 343:85-86
71. MacSweeney EJ, Oades PJ, Buchdahl R, Rosenthal M, Bush A: Relation of thickening of colon wall to pancreatic-enzyme treatment in cystic fibrosis. Lancet 1995; 345:752-756
72. Corey M, McLaughlin FJ, 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; 41:583-591
73. Ramsey BW, Farrel PM, Pencharz P, and the Consensus Committee. Nutritional assessment and management in cystic fibrosis: A consensus report. Am J Clin Nutr 1992; 55:108-116
74. Homnick DN, Cox JH, DeLoof MJ, Ringer TV: Carotenoid levels in normal children and in children with cystic fibrosis. J Pediatr 1993; 122(pt 1): 703-707
75. Winklhofer-Roob BM, Puhl H, Khoschsorur G, van't Hof MA, Esterbauer H. Shmerling DH. Enhanced resistance to oxidation of low density lipoproteins and decreased lipid peroxide formation during ß-carotene supple-mentation in cystic fibrosis. Free Radic Biol Med 1995, 18:849-859
76. Gorlin R: The biological actions and potential clinical significance of dietary omega-3 fatty acids. Arch Intern Med 1988; 148.2043-2048
77. Konstan MW, Walenga RW, Hilliard KA, Berger M: Eicosanoid content of bronchoalveolar lavage fluid is markedly elevated in cystic fibrosis. Pediatr Pulmonol Suppl 1991, 6.302
78. Henderson WR Jr, Astley SJ, McCready MM, et al: Oral absorption of omega-3 fatty acids in patients with cystic fibrosis who have pancreatic insufficiency and in healthy control subjects. J Pediatr 1994; 124.400-408
79. O'Bnen S, Keosan M, Casey M, et al: Biliary complications of cystic fibrosis. Gut 1992; 33:387-391
80. Colombo C, Apostolo MG, Ferrari M, et al: Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr 1994; 124:393-399
81. Waters DL, Dorney SFA, Gruca MA, et al: Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency. Hepatology 1995; 21:963-969
82. Feigelson J, Anagnostopoulos C, Poquet M, Pecau Y, Munck A, Navarro J: Liver cirrhosis in cystic fibrosis - Therapeutic implications and lonc-term follow up. Arch Dis Child 1993; 68:653-657
83. Galabert C, Montet JC, Lengrand D, et al: Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chrome cholestasis. J Pediatr 1992; 121:138-141
84. Borowitz D: Pathophysiology of gastrointestinal complications of cystic fibrosis. Semin Respir Crit Care Med 1994; 15:391-401
85. Neglia JP, FitzSimmons SC, Maisonneuve P. et al. and the Cystic Fibrosis and Cancer Study Group: The risk of cancer among patients with cystic fibrosis. N Engl J Med 1995, 332:494-499
86. Patterson JM, Budd J, Goetz D, Warwick WJ: Family correlates of a 10-year pulmonary health trend in cystic fibrosis. Pediatrics 1993; 91:383-389
87. Williams MT, Chest physiotherapy and cystic fibrosis - Why is the most effective form of treatment stil unclear? Chest 1994; 106:1872-1882
88. Miller S, Hall D, Clayton CB, Nelson R, Chest physiotherapy in cystic fibrosis (CF) a comparative study of autogenic drainage (AD) and active cycle of breathing technique (ACBT) (formerly FET). Pediatr Pulmonol Suppl 1993; 9:267
89. Hofmeyr JL, Webber BA, Hudson ME. Evaluation of positive pressure as an adjunct to chest physiotherapy in the treatment of cystic fibrosis. Thorax 1986; 41.951-954
90. Konstan MW, Stern RC. Doershuk CF Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis. J Pediatr 1994; 124: 689-693
91. Arens R, Gozal D, Omlin KJ, et al: Comparison of high frequency chest compression and conventional chest physiotherapy in hospitalized patients with cystic fibrosis. Am J Respir Cm Care Med 1994; 150:1154-1157
92. George RJD, Pavia D, Woodman G, et al: Oral high frequency oscillation (OHFO) as an adjunct to physiotherapy (PHYSIO) in cystic fibrosis (CF). Thorax 1986, 41:235P
93. Thomas J, Cook DJ. Brooks D: Chest physical therapy management of patients with cystic fibrosis Am J Respir Crit Care Med 1995; 151:846-850
94. Smith AL, Redding G, Doershuk C, et al. Sputum changes associated with therapy for endobronchial exacerbation in cystic fibrosis. J Pediatr 1988; 112.547-554
95. Shak S, Capon DJ, Hellniss R, Marsters SA, Baker CL: Recombinant human DNase I reduces the viscosity of cystic fibrosis sputum. Proc Natl Acad Sci USA 1990; 87.9188-9192
96. Fuchs HJ, Borowitz DS, Christiansen DH, et al: Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N End J Med 1994; 331:637-642
97. Rosenstein BJ, Johnson CAC, for the rhDNase Phase III Study Group-Long-term follow-up of Phase III rhDNase trial. Pediatr Pulmonol Suppl 1994; 10:113-114
98. Vasconcellos CA, Allen PG, Wohl ME, Drazen JM, Janmey PA, Stossel TP: Reduction m viscosity of cystic fibrosis sputum in vitro by geisolin. Science 1994; 263:969-971
99. Know les MR, Olivier KN, Bennett W. et al: Aerosolized uridine triphosphate (UTP) + amiloride: Safety and effect on mucociliary clearance in normal subjects and CF patients Pediatr Pulmonol Suppl 1994; 10:99-100
100. Stafanger G, Koch C, N-Acetylcysteme in cystic fibrosis and Pseudomonas aeruginosa infection. Clinical score, spirometry and ciliary motility. Eur Respir J 1989; 2:234-237
101. Stafanger G, Garne S, Howitz P, Morkassel E, Koch C: The clinical effect and the effect on the ciliary moulity of oral N-acetylcysteine in patients with cystic fibrosis and primary ciliary dyskinesia. Eur Respir J 1988; 1:161-167
102. Schidlow DV, Taussig LM, Knowles MR: Cystic Fibrosis Foundation consensus report on pulmonary complications of cystic fibrosis. Pediatr Pulmonol 1993; 15:187-198
103. Mroueh S, Spock A: Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. Chest 1994, 105:32-36
104. Hutcheson PS, Rejent AJ, Slavin RG: Variability in parameters of allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. J Allergy Clin Iramunol 1991; 88:390-394
105. Shale DJ, Faux JA, Lane DJ. Trial of ketoconazole in non-invasive pulmonary aspergillosis. Thorax 1987; 42.26-31
106. Slavin RG Medical manasement of nasal polyps and sinusitis. J Allergy Clin Immunol 1991; 88 141-146
107. Ramsey B, Richardson MA: Impact of sinusitis in cystic fibrosis. J Allergy Clin Immunol 1992, 90:547-552
108. Sharma GD, Doershuk CF, Stern RC: Erosion of the wall of the frontal sinus caused by mucopyocele in cystic fibrosis. J Pediatr 1994; 124.745-747
109. Stern RC, Boat TF, Wood RE, Matthews LW, Doershuk CF: Treatment and prognosis of nasal polyps in cystic fibrosis. Am J Dis Child 1982; 136:1067-1070
110. Cepero R, Smith RJH, Catlin FI, Bressler KL, Furuta GT, Shandera KC: Cystic fibrosis - An otolarynaologic perspective. Otolaryngol Head Neck Surg 1987;97:356-360
111. Johnson S, Knox AJ: Arthropathy in cystic fibrosis. Respir Med 1994; 88:567-570
112. Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF: The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med 1992; 327:1785-1788
113. Ramsey B, Pepe M, Warren JW Pulmonary exacerbation - How do we define it? Pediatr Pulmonol Suppl 1994; 10.77-78
114. Moorcroft AJ, Dodd ME, Webb AK. Exercise testing and prognosis in adult cystic fibrosis. Pediatr Pulmonol Suppl 1994; 10:265
115. Heijerman HGM, Bakker W, Sterk PJ, Dijkman JH: Oxygen-assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise. Int J Rehabil Res 1991; 14:101-115
116. de Jong W, Grevink RG, Roorda RJ, Kaptein AA, van der Schans CP: Effect of a home exercise training program in patients with cystic fibrosis. Chest 1994, 105:463-468
117. Gillen M, Lallas D, Brown C, Yelin E, Blanc P: Work disability in adults with cystic fibrosis. Am J Respir Crit Care Med 1995; 152:153-156
118. Shepherd SL, Hovell MF, Harwood IR. et al: A comparative study of the psychosocial assets of adults with cystic fibrosis and their healthy peers Chest 1990; 97 1310-1316
119. Burke W, McDonald GJ, Aitken ML: Reproductive decision-making in women affected with CF. Pediatr Pulmonol Suppl 1994, 10:292
120. Kotloff RM: Reproductive issues in patients with cystic fibrosis. Semin Respir Crit Care Med 1994; 15:402-413
121. Silber SJ, Ord T, Balmaceda J, Patrizio P, Asch RH: Congenital absence of the vas deferens - The fertilizing capacity of human epididymal sperm, N Engl J Med 1990; 323:1788-1792
122. Kredentser JV, Podrant C, McCoshen JA Intrauterine insemination for infertility due to cystic fibrosis. Fertil Steril 1986, 45.425-426
123. Edenborough FP, Stableforth DE, Webb AK, Mackenzie WE, Smith DL: Outcome of pregnancy in women with cystic fibrosis. Thorax 1995; 50: 170-174
124. Kerem E, Reisman J, Corey M, Canny GJ, Levison H: Prediction of mortality in patients with cystic fibrosis. N Engl J Med 1992; 326:1187-1191
125. Tamm M, Higenbottam T, Heart-lung and lung transplantation for cystic fibrosis. World experience. Semin Respir Crit Care Med 1994; 15:414-425
126. Egan TM, Detterbeck FC, Mill MR, et al: Improved results of lung transplantation for patients with cystic fibrosis. J Thorac Cardiovasc Surg 1995: 109:224-235
127. Starnes VA, Barr ML, Cohen RG, Schenkel FA, and the USC Transplant Group: Liviing related lune transplantation in cystic fibrosis Pediatr Pulmonol Suppl 1994; 10.128-129
128. de Leval MR, Smith R, Whitehead B, et al: Heart and lung transplantation for terminal cystic fibrosis. J Thorac Cardiovasc Surg 1991; 101:633-642
129. Flume PA, Egan TM, Paradowski LJ, Detterbeck FC, Thompson JT, Yankaskas JR: Infectious complications of lung transplantation - Impact of cystic fibrosis Am J Respir Crit Care Med 1994;149:1601-1607
130. Kotloff RM, Zukerman JB. Lung transplantation for cystic fibrosis: Special considerations. Chest 1996, 109-787-798
131. Drumm ML, Pope HA, Cliff WH, et al: Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer Cell 1990; 62:1227-1233
132. Zabner J, Couture LA, Gregory RJ, Graham SM, Smith AE, Welsh MJ. Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis. Cell 1993; 75.207-216
133. Crystal RG, McElvaney NG, Rosenfeld MA, et al: Administration of an adenovirus containing the human CFTR cDNA to the respiratory tract of individuals with cystic fibrosis. Nature Genet 1994; 8:42-51
134. Rosenfeld MA. Collins FS Gene therapy for cystic fibrosis. Chest 1996, 109:241-252
135. Bebok A, Dong JY, Logan JJ, et al: Plasmid delivery is not a limiting factor usins lipid mediated gene transfer in vitro. Pediatr Pulmonol Suppl 1994; 10:223
136. Zabner J, Fasbender A, Moninger T, Welsh MJ: Evaluation of cationic lipids for gene transfer. Pediatr Pulmonol Suppl 1994, 10:223
137. Wilson JM: Cystic fibrosis: Strategies for gene therapy. Semin Respir Crit Care Med 1994, 15:439-445
138. Johnson LG, Boyles SE, Wilson J, Boucher RC: Normalization of raised sodium absorption and raised calcium-mediated chioride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells. J Clin Invest 1995: 95:1377-1382
139. Engelhardt JF, Zepeda M, Cohn JA, Yankaskas JR, Wilson JM: Expression of the cystic fibrosis gene in adult human lung. J Clin Invest 1994; 93:737-749
140. Pilewski JM, Engelhardt JF, Bavaria JE, Kaiser LR, Wilson JM, Albelda SM: Adenovirus-mediated gene transfer to human bronchial submucosal glands using xenografts. Am J Physiol 1995; 268:L657-L665
141. Yei S, Mittereder N, Whitsett JA, Wilmott R, Trapnell BC: The role of the host response in adenovirus vector-mediated gene therapy for cystic fibrosis lung disease: Toxicology and pharmacokinetics Pediatr Pulmonol Suppl 1994; 10:151-152
142. McCoy RD, Davidson BL, Roessler BJ, et al: Pulmonary inflammation induced by incomplete or inactivated adenoviral particles. Hum Gene Ther 1995; 6:1553-1560
143. Wilson JM. Gene therapy for cystic fibrosis lung disease with recombinant adenoviroses: Host-vector interactions. Pediatr Pulmonol Suppl 1994, 10:155
144. Teramoto S, Johnson LG, Huang W, Leigh MW, Boucher RC Adenoviral vector infection inhibits human airway epithelial cell proliferation reflecting apoptosis and alteration of the cell cycle. Pediatr Pulmonol Suppl 1994; 10.222-223
145. Kearns W, Afiore S, Cutting G, Carter B, Pearson P, Flotte T: AAV-CFTR vectors integrate at multiple chromosomal sites in a CF bronchial epithelial cell line. Pediatr Pulmonol Suppl 1993; 9:242
146. Yang Y, Raper SE, Cohn JA, Engelhardt JF, Wilson JM: An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer Proc Natl Acad Sci USA 1993; 90:4601-4605
147. Grubman SA, Fang SL, Mulberg AE, et al: Correction of the cystic fibrosis defect by gene complementation in human intrahepatic biliary epithelial cell lines Gastroenterology 1995; 108.584-592