Abstract
Cystic Fibrosis (CF) is an autosomal recessive disease which is most commonly found in Caucasian peoples. In the UK it affects about 1 infant in 2000 (Kuzemko, 1986). The recently isolated gene for CF (Rommens, et al., 1989) is located on the long arm of chromosome number 7 (Wainwright et al., 1985), and genetic probes which lie close to it are already in use for the purposes of prenatal diagnosis (Farrall et al, 1986). The basic biochemical abnormality probably lies at the cellular level but has not yet been identified (Williamson et al, 1983). It is thought to involve a defect in the transport of chloride ions across membranes (Quinton, 1983).
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References
Batten, J. (1988) Cystic Fibrosis in adolescents and adults. Excerpta Medica, Asia Pacific Congress Series 74, 187–94.
Batten, J. C. and Matthew, D.J. (1983) The respiratory system. In: Hodson, M. E., Norman, A. P. and Batten, J. C. (eds) Cystic Fibrosis, Baillière Tindall, London, pp. 105–31.
Brock, D. J. H. (1988) Prenatal diagnosis of Cystic Fibrosis. Arch. Dis. Child. 63, 701–4.
Bywater, M. (1981) Adolescents with Cystic Fibrosis: psychosocial adjustment. Arch. Dis. Child. 56, 538–43.
Cerney, F. J., Pullano, T. P. and Cropp, G. J. A. (1982) Cardiorespiratory adaptations to exercise in C. F. Am. Rev. Resp. Dis. 126, 217–22.
Chrispin, A. R. and Norman, A. P. (1974) The systematic evaluation of the chest radiograph in Cystic Fibrosis. Paediatr. Radiol. 2, 101–6.
Cropp, G. J., Pullano, T. P., Cerney, F.J. and Nathanson, T.J. (1982) Exercise tolerance and cardiorespiratory adjustments at peak work capacity in C.F. Am. Rev. Resp. Dis. 126, 211–26.
Dinwiddie, R. (1986) Management of the chest in Cystic Fibrosis. J. Roy. Soc. Med. 79, 6–9.
Dinwiddie, R. and Madge, S. (1988) Intensive calorie counting in C.F.: an effective way of achieving weight gain? 10th International Cystic Fibrosis Congress, Excerpta Medica, Asia Pacific Congress Series 74, 165.
Drake-Lee, A. B. and Pitcher-Wilmott, R. W. (1982) The clinical and laboratory correlation of nasal polyps in Cystic Fibrosis. Int. J. Pediatr. Otorhinolaryngol. 4, 209–14.
Farrall, M., Law, H.-Y., Rodeck, C. H., Warren, R., Stanier, P., Super, M., Lissens, W., Scambler, P., Watson, E., Wainwright, B. and Williamson, R. (1986) First-trimester prenatal diagnosis of Cystic Fibrosis with linked DNA probes. Lancet i, 1402–5.
Geddes, D. M. (1984) Physical exercise and Cystic Fibrosis. In: Lawson, D. (ed.) Cystic Fibrosis: horizons, Wiley, Chichester, pp. 117–33.
Gibson, L. E. and Cooke, R. E. (1959) A test for the concentration of electrolytes in sweat in Cystic Fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics 23, 545–9.
Heaf, D. P., Webb, G.J. and Matthew, D.J. (1983) In vitro assessment of combined antibiotic and mucolytic therapy for Pseudomonas aeruginosa in Cystic Fibrosis. Arch. Dis. Child. 58, 824–6.
Hodson, M. E. and Gaskill, D. V. (1983) Physiotherapy. In: Hodson, M. E., Norman, A. P. and Batten, J. C. (eds) Cystic Fibrosis, Baillière Tindall, London, pp. 219–41.
Hodson, M. E., Penketh, A. R. L. and Batten, J. C. (1981) Aerosol carbenicillin and gentamicin treatment for Pseudomonas aeruginosa infection in Cystic Fibrosis. Lancet ii, 1137–9.
Hodson, M. E., Butland, R.J. A., Roberts, C. M., Smith, J. and Batten, J. C. (1987) Oral ciprofloxacin compared with conventional intravenous treatment for Pseudomonas aeruginosa infection in patients with Cystic Fibrosis. Lancet i, 235–7.
Høiby, N. (1982) Microbiology of lung infections in Cystic Fibrosis patients. Acta Paediatr. Scand. Suppl. 301, 33–54.
Høiby, N. and Schiøtz, P. O. (1982) Immune complex mediated tissue damage in the lungs of Cystic Fibrosis patients with chronic Pseudomonas aeruginosa infection. Acta Paediatr. Scand. Suppl. 301, 63–73.
Holtz, F. J., Olinsky, A. and Phelan, P. D. (1981) Variability of airways hyperreactivity and allergy in Cystic Fibrosis. Arch. Dis. Child. 56, 495–9.
Kram, E. R., Crane, M. M., Sirkin, M. G. and Brown, M. L. (1962) A Cystic Fibrosis pilot survey in three New England States. Am. J. Dis. Child 52, 2041–57.
Kulzycki, L. L. and Shauf, V. (1974) Cystic Fibrosis in blacks in Washington DC. Am. J. Dis. Child. 127, 64–7.
Kuzemko,J. A. (1986) Screening, early neonatal diagnosis and prenatal diagnosis. J. Roy. Soc. Med. 79 (Suppl. 12), 2–5.
Kuzemko, J. A. and Heeley, A. F. (1983) Diagnostic methods and screening. In: Hodson, M. E., Norman, A. D. and Batten, J. C. (eds) Cystic Fibrosis, Baillière Tindall, London, pp. 21–30.
Lancet (1986) Editorial. Supplementary nutrition in Cystic Fibrosis. Lancet i, 249.
Levin, S. (1963) Fibrocystic disease of the pancreas. In: Goldschmidt, E. (ed.) Genetics of Migrant and Isolated Populations, Williams and Wilkins, Baltimore, p. 293.
Mearns, M. B. (1980) Natural history of pulmonary infection in Cystic Fibrosis. In: Sturgess, J. N. (ed.) Perspectives in Cystic Fibrosis, Canadian Cystic Fibrosis Foundation, Toronto, p. 325.
Meindl, R. S. (1987) Hypothesis: a selective advantage for Cystic Fibrosis heterozygotes. Am. J. Clin. Nutr. 74, 39–54.
Morton, R. E., Hutchings, J., Halliday, D., Rennie, M.J. and Wolman, S. L. (1988) Protein metabolism during treatment of chest inf ection in patients with Cystic Fibrosis. Am. J. Clin. Nutr Am. J. Clin. Nutr 47, 214–19.
Pedersen, S. S., Jensen, T., HØiby, N., Koch, C. and Flensborg, E. W. (1987) Management of Pseudomonas aeruginosa lung infection in danish Cystic Fibrosis patients. Acta Paediatr. Scand. 76, 955–61.
Penketh, A. R. L., Wise, A., Mearns, M. B., Hodson, M. E. and Batten, J. C. (1987) Cystic Fibrosis in adolescents and adults. Thorax 42, 526–32.
Peterson, N. T., H0iby, N., Mordhorst, C. H., Lind, K., Flensburg, E. W. and Rune, B. (1981) Respiratory infection in Cystic Fibrosis patients caused by virus, chlamydia and mycoplasma. Possible synergism with Pseudomonas aeruginosa. Acta Paediatr. Scand. 70, 623–8.
Phillips, B. M. and David, T.J. (1987) Management of the chest in Cystic Fibrosis. J. Roy. Soc. Med. 80 (Suppl. 18), 30–7.
Pitcher-Wilmott, R. W., Levinsky, J., Gordon, I., Turner, M. W. and Matthew, D. J. (1982) Pseudomonas infection allergy and Cystic Fibrosis. Arch. Dis. Child. 57, 582–6.
Prior, J. A., Webber, B. A., Hodson, M. E. and Batten, J. C. (1979) Evaluation of the forced expiratory technique as an adjunct to postural drainage in treatment of Cystic Fibrosis. Brit. Med. J. 2, 417–18.
Quinton, P. M. (1983) Chloride impermeability in Cystic Fibrosis. Nature 301, 421–2.
Reid, L. and De Haller, R. (1964) Lung changes in Cystic Fibrosis. In: Hubble, D. (ed.) Cystic Fibrosis, London Chest and Heart Association, London, p. 21.
Righetti, A. B. B., Mighavacca, M., Prampolini, L. and Guinta, A. (1976) Extensive neonatal screening of CF. Proceedings of VII International Cystic Fibrosis Conference, p. 153.
Rommens,J. M., Iannuzzi, M. C., Kerem, B.-S. (1989) Identification of the Cystic Fibrosis: chromosome walking and jumping. Science, 245, 1059–65.
Scully, B. E., Neu, H. C., Parry, M. F. and Mandell, W. (1986) Oral ciprofloxacin therapy of infections due to Pseudomonas aeruginosa. Lancet i, 819–22.
Selander, P. (1962) The frequency of Cystic Fibrosis of the pancreas in Sweden. Acta Paediatr. Sccind. 51, 65–7.
Stern, R. C., Boat, T. F., Wood, R. E., Matthews, L. W. and Doershuk, C. F. (1982) Treatment and prognosis of nasal polyps in Cystic Fibrosis. Am. J. Dis. Child. 136, 1067–70.
Stroobant, J. (1986) Viral infection in Cystic Fibrosis. J Roy. Soc. Med, 79, 19–22.
Szaff, M., Høiby, N. and Flensborg, E. W. (1983) Frequent antibiotic therapy improves survival of Cystic Fibrosis patients with chronic Pseudomonas infec-tion. Acta Paediatr.Scand. 72, 651 — 7.
Tomashefski, G. F., Vawter, G. F. and Reid, L. (1983) Pulmonary pathology. In: Hodson, M. E., Norman, A. P. and Batten, J. C. (eds) Cystic Fibrosis, Baillière Tindall, London, pp. 31–51.
Tyrell, J. C., Weller, E. J. and Martin, J. (1986) Face mask physiotherapy in Cystic Fibrosis. Arch. Dis. Child. 61, 598–601.
Wainwright, B. J., Scambler, P. J., Schmidtke, J., Watson, E. A., Law, H.-Y., Farrall, M., Cooke, H. J., Eiberg, H. and Williamson, R. (1985) Localization of Cystic Fibrosis locus to human chromosome 7cen—q22. Nature 318, 384–5.
Warner, J. O., Taylor, B. W., Norman, A. P. and Soothill, J. L. (1976) Association of Cystic Fibrosis with allergy. Arch. Dis. Child. 51, 507—11.
Williams, J., Alfaham, M., Ryley, H. C., Goodchild, M. C., Weller, P. H. and Dodge, J. A. (1988a) Screening for Cystic Fibrosis in Wales and the West Midlands. 10th International Cystic Fibrosis Congress, Excerpta Medica, Asia Pacific Congress Series 74, 20–1.
Williams, J., Smith, H. L., Woods, C. G. and Weller, P. H. (1988b) Silastic catheters for antibiotics in Cystic Fibrosis. Arch. Dis. Child. 63, 658–9.
Williamson, R., Crampton, J. M. and Clarke, B. E. (1983) Research perspectives: the basic defect in Cystic Fibrosis. In: Hodson, M. E., Norman, A. P. and Batten, J. C. (eds) Cystic Fibrosis, Baillière Tindall, London, pp. 260–72.
Wilmott, R. W. (1985) Allergy and infection in Cystic Fibrosis. In: Milner, A. D. and Martin, R. J. (eds) Neonatal and Pediatric Respiratory Medicine, Butterworths, London,pp. 190–210.
Wilmott, R. W., Tyson, S. L., Dinwiddie, R. and Matthew, D.J. (1983) Survival rates in Cystic Fibrosis. Arch. Dis. Child. 58, 835–6.
Wilmott, R. W., Tyson, L. S. and Matthew, D. J. (1985) Cystic Fibrosis survival rates. The influence of allergy and Pseudomonas aeruginosa. Am. J. Dis. Child. 139, 669–73.
Wright, S. W. and Norton, N. E. (1966) Genetic studies in Cystic Fibrosis in Hawaii. Am.J. Human Gen. 20, 157–69.
Zach, M. S. (1988) Lung disease in Systic Fibrosis: current concepts. Excerpta Medica, Asia Pacific Congress Series 74, 72–9.
Zach, M. S., Oberwaldner, B. and Hausler, F. (1982) Cystic Fibrosis: physical exercise versus chest physiotherapy. Arch. Dis. Child. 57, 587–9.
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Dinwiddie, R. (1990). Clinical aspects of mucoid Pseudomonas aeruginosa infections. In: Gacesa, P., Russell, N.J. (eds) Pseudomonas Infection and Alginates. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1836-8_2
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