Diagnosis and treatment of cystic fibrosis

Respiration

Volumn 67, Issue 3, 2000, Pages 239-247

  Koch, Christian ^a   Høiby, Niels ^a  

^a Rigshospitalet   (Denmark)

Author keywords

Antibiotic treatment; Centralised care; Cystic fibrosis; Diabetes; Diagnosis; Infections; Pancreas

Indexed keywords

ACETYLCYSTEINE; ANTIINFLAMMATORY AGENT; BROMHEXINE; BRONCHODILATING AGENT; CIMETIDINE; COLISTIN; CORTICOSTEROID; DORNASE ALFA; IBUPROFEN; INSULIN; ITRACONAZOLE; MUCOLYTIC AGENT; OMEPRAZOLE; PANCREAS ENZYME; QUINOLINE DERIVED ANTIINFECTIVE AGENT; RANITIDINE; TOBRAMYCIN; URSODEOXYCHOLIC ACID; VITAMIN;

ANTIBIOTIC PROPHYLAXIS; ASPERGILLOSIS; BRONCHOPNEUMONIA; COLON DISEASE; CYSTIC FIBROSIS; DIABETES MELLITUS; GENE MUTATION; GRAM NEGATIVE INFECTION; HUMAN; LIFE EXPECTANCY; LIVER DISEASE; LUNG INFECTION; MYCOBACTERIOSIS; NEWBORN SCREENING; NOSE POLYP; PANCREAS INSUFFICIENCY; PRENATAL DIAGNOSIS; PRIORITY JOURNAL; PSEUDOMONAS AERUGINOSA; REVIEW; SIDE EFFECT; STEATORRHEA; SWEAT TEST;

EID: 0034127685     PISSN: 00257931     EISSN: None     Source Type: Journal    
DOI: 10.1159/000029503     Document Type: Review

References (87)

1. Rosenstein BJ, Cutting GR: The diagnosis of cystic fibrosis: A consensus statement. J Pediatr 1998;132:589-595.
2. Fiel SB: Clinical management of pulmonary disease in cystic fibrosis. Lancet 1993;341: 1070-1074.
3. Di Sant'Agnese PA, Darling RC, Perera GA, Shea E: Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas: clinical significance and relationship to disease. Pediatrics 1953;12:549-563.
4. Quinton PM: Chloride impermeability in cystic fibrosis. Nature 1983;301:421-422.
5. Rommens JM, Iannuzzi MC, Kerem B-S, et al: Identification of the cystic fibrosis gene: Chromosome walking and jumping. Science 1989; 245:1059-1065.
6. Riordan JR, Rommens JM, Kerem B-S, et al: Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 1989;245:1066-1073.
7. Kerem B-S, Rommens JM, Buchanan JA: Identification of the cystic fibrosis gene: Genetic analysis. Science 1989;245:1073-1080.
8. Zielenski J: Genotype and phenotypc in cystic fibrosis. Respiration 2000;67:117-133.
9. Koch C, Høiby N: Pathogenesis of cystic fibrosis. Lancet 1993;341:1065-1069.
10. Gibson LE, Cooke RE: A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics 1959;23:545-549.
11. Hammond KB, Turcios NL, Gibson LE: Clinical evaluation of the macroduct sweat collection system and conductivity analyzer in the diagnosis of cystic fibrosis. J Pediatr 1994;124: 255-260.
12. Nielsen OH, Flensborg EW: Examination of sweat electrolytes with the pilocarpin-iontophoresis method (in Danish). Ugeskr Læg 1981;143:2302-2306.
13. Kerem E, Kerem B: Genotype-phenotype correlations in cystic fibrosis. Pediatr Pulmonol 1996;22:387-395.
14. Dankert-Roelse JE, Meerman GJ: Long term prognosis of patients with cystic fibrosis in relation to early detection by neonatal screening and treatment in a cystic fibrosis centre. Thorax 1995;50:712-718.
15. Schwartz M, Brandt NJ, Skovby F: Screening for carriers of cystic fibrosis among pregnant women: A pilot study. Eur J Hum Genet 1993; 1:239-244.
16. Andersen DH: Therapy and prognosis of fibrocystic disease of the pancreas. Pediatrics 1949; 3:406-417.
17. Matthews LW, Doershuk CF, Wise M, et al: A therapeutic regimen tor patients with cystic fibrosis. J Pediatr 1964;65:558-575.
18. Smyth RL, Van Velzen D, Smyth AR, et al: Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. Lancet 1994;343:85-86.
19. Knabe N, Zak M, Hansen A, et al: Extensive pathological changes of the colon in cystic fibrosis and high strength enzymes. Lancet 1994;343:1230.
20. 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.
21. Poole S: Dietary treatment of cystic fibrosis; in Hodson ME, Geddes DM (eds): Cystic Fibrosis. London, Chapman & Hall, 1995, pp 383-395.
22. Nir M, Lanng S, Johansen HK, Koch C: Long term survival and nutritional data in patients with cystic fibrosis treated in a Danish centre. Thorax 1996;51:1023-1027.
23. Koch C, Høiby N: Cystic fibrosis: Management of CF in different countries. Cystic fibrosis in Copenhagen. Thorax 1991;46:383-386.
24. Hartling SG, Garne S, Binder C, et al: Proinsulin, insulin, and C-peptide in cystic fibrosis after an oral glucose tolerance test. Diabetes Res 1988;7:165-169.
25. Lanng S, Thorsteinsson B, Erichsen G, et al: Glucose tolerance in cystic fibrosis. Arch Dis Child 1991;66:612-616.
26. Finkelstein SM, Wiclinski CL, Elliott GR, et al: Diabetes mellitus associated with cystic fibrosis. J Pediatr 1988;112:373-377.
27. Lanng S, Thorsteinsson B, Nerup J, Koch C: Influence of the development of diabetes on clinical status in patients with cystic fibrosis. Eur J Pediatr 1992;151:684-687.
28. Koch C, Lanng S: Other organ systems; in Hodson ME, Geddes DM (eds): Cystic Fibrosis. London, Chapman & Hall, 1995, pp 295-314.
29. Nagel RA, Javaid A, Meire HB, et al: Liver disease and bile duct abnormalities in adults with cystic fibrosis. Lancet 1989;ii:1422-1425.
30. Colombo C, Setchell KDR, Podda M, et al: Effects of ursodeoxycholic acid therapy in liver disease associated with cystic fibrosis. J Pediatr 1990;117:482-489.
31. Cotting J, Lentze M, Reichen J: Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis. Gut 1990;31: 918-921.
32. Koch C, McKenzie SG, Kaplowitz H, et al: International practice patterns by age and severity of lung disease in cystic fibrosis: Data from the Epidemiologic Registry of Cystic Fibrosis (ERCF). Pediatr Pulmonol 1997;24: 147-154.
33. Kingdom TT, Lee KC, FitzSimmons SC, Cropp GJ: Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis requiring surgery. Arch Otolaryngol Head Neck Surg 1996;122:1209-1213.
34. Smith JJ, Travis SM, Greenberg EP, Welsh MJ: Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 1996;85:229-236.
35. Matsui H, Grubb BR, Tarran R, et al: Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998;95: 1005-1015.
36. Saiman L, Prince A: Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells. J Clin Invest 1993;92:1875-1880.
37. Barasch J, Kiss B, Prince A, et al: Defective acidification of intracellular organelles in cystic fibrosis. Nature 1991;352:70-73.
38. FitzSimmons SC: The changing epidemiology of cystic fibrosis. J Pediatr 1993;122:1-9.
39. Cystic Fibrosis Foundation: Patient Registry 1992. Annual Data Report. Bethesda, Cystic Fibrosis Foundation, 1993.
40. Corey M, Farewell V: Determinants of mortality from cystic fibrosis in Canada 1970-1989. Am J Epidemiol 1996;143:1007-1017.
41. Elborn JS, Shale DJ, Britton JR: Cystic fibrosis: Current survival and population estimates to the year 2000. Thorax 1991;46:881-885.
42. Cystic Fibrosis Foundation: Patient Registry 1999. Annual Data Report, Bethesda, Cystic Fibrosis Foundation, 1999.
43. Ramsey BW, Pepe MS, Quan JM, et al: Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. N Engl J Med 1999;340:23-30.
44. Frederiksen B, Thorsteinsson SL, Koch C, Høiby N: Improved prognosis for patients with cystic fibrosis. A result of aggressive center-based treatment. Pediatr Pulmonol 1996;21:153-158.
45. Mahadeva R, Webb K, Westerbeck RC, et al: Clinical outcome in relation to care in centers specialising in cystic fibrosis: Cross-sectional study. BMJ 1998;316:1771-1775.
46. Warwick WJ: Prognosis for survival with cystic fibrosis: The effects of early diagnosis and cystic fibrosis center care. Acta Paediatr Scand 1982(suppl 301):27-31.
47. Høiby N, Friis B, Jensen K, et al: Antimicrobial chemotherapy in cystic fibrosis patients. Acta Paediatr Scand 1982(suppl 301):75-100.
48. Høiby N, Koch C, Spencer D: Cystic fibrosis: With special reference to respiratory infections: in Ryan DW (ed.): Current Practice in Critical Illness. London, Arnold. 1999, vol 3, pp 15-43.
49. Szaff M, Høiby N, Flensborg EW: Frequent antibiotic therapy improves survival of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection. Acta Paediatr Scand 1983; 72:651-657.
50. Johansen HK, Høiby N: Seasonal onset of initial colonisation and chronic infection with Pseudomonas aeruginosa in patients with cystic fibrosis. Thorax 1992;47:109-111.
51. Pedersen SS, Koch C, Høiby N, Rosendal K: An epidemic spread of multiresistant Pseudomonas aeruginosa in a cystic fibrosis center. J Antimicrob Chemother 1986;17:505-516.
52. Høiby N, Pedersen SS: Estimated risk of cross-infection with Pseudomonas aeruginosa in Danish cystic fibrosis patients. Acta Paediatr Scand 1989;78:395-404.
53. Wolz C, Kiosz G, Ogle JW, et al: Pseudomonas aeruginosa cross-colonization and persistence in patients with cystic fibrosis: Use of a DNA probe. Epidemiol Infect 1989;102:205-214.
54. Tümmler B, Koopmann U, Grothues D, et al: Nosocomial acquisition of Pseudomonas aeruginosa by cystic fibrosis patients. J Clin Microbiol 1991;29:1265-1267.
55. LiPuma JJ, Dasen SE, Nielson DW, et al: Person-to-person transmission of Pseudomonas cepacia between persons with cystic fibrosis. Lancet 1990;336:1094-1096.
56. Pegues DA, Carson LA, Tablan OC, et al: Acquisition of Pseudomonas cepacia at summer camps for patients with cystic fibrosis. J Pediatr 1994;124:694-702.
57. Govan JRW, Brown PH, Madison J, et al: Evidence for transmission of Pseudomonas cepacia by social contact in cystic fibrosis. Lancet 1993;342:15-19.
58. Johansen HK, Kovesi TA, Koch C, et al: Pseudomonas aeruginosa and Burkholderia cepacia infection in cystic fibrosis patients treated in Toronto and Copenhagen. Pediatr Pulmonol 1998;26:89-96.
59. Frederiksen B, Koch C, Høiby N: Changing epidemiology of Pseudomonas aeruginosa infection in Danish cystic fibrosis patients (1974-1995). Pediatr Pulmonol 1999;28:159-166.
60. Valerius NH, Koch C, Høiby N: Prevention of chronic Pseudomonas aeruginosa colonization in cystic fibrosis by early treatment. Lancet 1991;338:725-726.
61. Frederiksen B, Koch C, Høiby N: Antibiotic treatment of initial colonization with Pseudomona aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis. Pediatr Pulmonol 1997; 23:330-335.
62. Kutsen AP, Slavin RG: Allergic bronchopulmonary mycosis complicating cystic fibrosis. Semin Respir Infect 1992;7:179-192.
63. Nelson LA, Callerame ML, Schwartz RH: Aspergillosis and atopy in cystic fibrosis. Am Rev Respir Dis 1979;120:863-873.
64. Schønheyder H, Jensen T, Høiby N, Andersen P, Koch C: Frequency of Aspergillus fumigatus isolates and antibodies to aspergillus antigens in cystic fibrosis. Acta Pathol Microbiol Immunol Scand [B] 1985;93:105-112.
65. Schønheyder H, Jensen T, Høiby N, Koch C: Clinical and serological survey of pulmonary aspergillosis in patients with cystic fibrosis. Int Arch Allergy Appl Immunol 1988;85:472-477.
66. Nikolaizik WH, Brueton MJ, Warner JO: Aspergillus allergy and allergic bronchopulmonary aspergillosis in cystic fibrosis. Pediatr Allergy Immunol 1991;2:83-86.
67. Skov M, Poulsen I.K. Koch C: Increased antigen-specific Th-2 response in allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis. Pediatr Pulmonol 1999;27: 74-79.
68. Smith MJ, Efthimiou J, Hodson ME, Batten JC: Mycobacterial isolations in young adults with cystic fibrosis. Thorax 1984;39:369-375.
69. Hjelte L, Petrini B, Källenius G, Strandvik B: Prospective study of mycobacterial infections in patients with cystic fibrosis. Thorax 1990; 45:397-400.
70. Kilby JM, Gilligan PH, Yankaskas JR, et al: Nontuberculous mycobacteria in adult patients with cystic fibrosis. Chest 1992;102:70-75.
71. Hjelt K, Hojlyng N, Howitz P, et al: The role of mycobacteria other than tuberculosis (MOTT) in patients with cystic fibrosis. Scand J Infect Dis 1994;26:569-576.
72. Lewin LO, Byard PJ, Davis PB: Effect of Pseudomonas cepacia colonization on survival and pulmonary function in cystic fibrosis patients. J Clin Epidemiol 1990;43:125-131.
73. Editorial: Pseudomonas cepacia - More than a harmless commensal? Lancet 1992;339:1385-1386.
74. Eigen H, Rosenstein BJ, FitzSimmons S, Schidlow DV: A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. J Pediatr 1995;126:515-523.
75. Konstan MW, Butler SM, Schidlow DV, et al: Patterns of medical practice in cystic fibrosis. II. Use of therapies, Pediatr Pulmonol 1999;28: 248-254.
76. Nikolaizik WH, Schöni MH: Pilot study to assess the effect of inhaled corticosteroids on lung function in patients with cystic fibrosis. J Pediatr 1996;128:271-274.
77. Bisgaard H, Pedersen SS, Nielsen KG, et al: Controlled trial of inhaled budesonide in patients with cystic fibrosis and chronic bronchopulmonary Pseudomonas aeruginosa infection. Am J Respir Crit Care Med 1997;156:1190-1196.
78. Nieman RS, Williams C, Maden R, et al: A double blind, placebo-controlled study comparing the effects of the inhaled corticosteroid fluticasone dipropionate 500 μg bid with placebo in adults with cystic fibrosis (abstract). Am J Respir Crit Care Med 1996;153:A72.
79. Konstan MW, Byard PJ, Hoppel CL, Davies PB: Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995;332: 848-854.
80. Cropp GJ: Effectiveness of bronchodilators in cystic fibrosis. Am J Med 1996;100(suppl 1A): 19-29.
81. Eggleston PA, Rosenstein BJ, Stackhouse CM, Alexander MF: Airway hyperreactivity in cystic fibrosis: Clinical correlates and possible effects on the course of the disease. Chest 1988; 94:360-365.
82. Mortensen J, Hansen A, Falk M, et al: Reduced effect of inhaled beta-2-adrenergic agonists on lung mucociliary clearance in patients with cystic fibrosis. Chest 1993;103:805-811.
83. Ranasinha C, Assoufi B, Shak S, et al: Efficacy and safety of short-term administration of aerosolized recombinant human Dnase I (rhDNase) in adults with stable stage cystic fibrosis. Lancet 1993;342:199-202.
84. Fuchs HJ, Borowitz DS, Christiansen DM et al: Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med 1994;331: 637-642.
85. McCoy K, Hamilton S, Johnson C: Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Chest 1996;110:889-895.
86. Johnson CA, Butler SM, Konstan MW, et al: Estimating effectiveness in an observational study: A case study of dornase alfa in cystic fibrosis. J Pediatr 1999;134:734-739.
87. Zeitlin PL: Novel pharmacologic therapies for cystic fibrosis. J Clin Invest 1999;103:447-452.