Skip to main content
PMC home page
Thorax logoLink to Thorax
. 1996 Oct;51(10):1023–1027. doi: 10.1136/thx.51.10.1023

Long-term survival and nutritional data in patients with cystic fibrosis treated in a Danish centre.

M Nir 1, S Lanng 1, H K Johansen 1, C Koch 1
PMCID: PMC472653  PMID: 8977604

Abstract

BACKGROUND: Adequate nutrition and optimal treatment of bronchopulmonary infections are both of critical importance in maintaining the health of patients with cystic fibrosis. The cystic fibrosis centre in Copenhagen has followed a regimen of very early and aggressive antimicrobial treatment, especially against Pseudomonas aeruginosa infection. An unrestricted diet of low fat and high protein without hyperalimentation was recommended before 1985 which was then changed to a high fat, high calorie intake. METHODS: The overall impact of the treatment regimen was evaluated by a cross sectional analysis of all 223 patients who attended the centre in 1989. Growth and nutritional parameters were combined with lung function parameters and with a retrospective analysis of chronic P aeruginosa infection and its duration. Survival curves for all 313 patients treated at the centre since 1949 were calculated. RESULTS: All the patients with cystic fibrosis had normal height, although the final height was achieved a little later than in healthy controls. Body weight was lower than normal in males above 15 and in females above 10 years of age. The body mass index (BMI), which was approximately 98% of normal in the younger patients, declined to 90% in adult men and to 83% in adult women with cystic fibrosis, and was strongly correlated with lung function parameters. In 1989 the median age of survival of all patients treated in the centre since 1949 was 30 years (32 years in males and 29 years in females). CONCLUSIONS: The overall treatment regimen in the cystic fibrosis centre in Copenhagen is associated with growth and survival rates that are at least equal to those in other cystic fibrosis centres in other countries.

Full text

PDF
1023

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Andersen E., Hutchings B., Jansen J., Nyholm M. Højde og vaegt hos danske børn. Ugeskr Laeger. 1982 Jun 14;144(24):1760–1765. [PubMed] [Google Scholar]
  2. Bertrand J. M., Morin C. L., Lasalle R., Patrick J., Coates A. L. Short-term clinical, nutritional, and functional effects of continuous elemental enteral alimentation in children with cystic fibrosis. J Pediatr. 1984 Jan;104(1):41–46. doi: 10.1016/s0022-3476(84)80586-7. [DOI] [PubMed] [Google Scholar]
  3. Boland M. P., Stoski D. S., MacDonald N. E., Soucy P., Patrick J. Chronic jejunostomy feeding with a non-elemental formula in undernourished patients with cystic fibrosis. Lancet. 1986 Feb 1;1(8475):232–234. doi: 10.1016/s0140-6736(86)90772-5. [DOI] [PubMed] [Google Scholar]
  4. Corey M., McLaughlin F. 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(6):583–591. doi: 10.1016/0895-4356(88)90063-7. [DOI] [PubMed] [Google Scholar]
  5. FitzSimmons S. C. The changing epidemiology of cystic fibrosis. J Pediatr. 1993 Jan;122(1):1–9. doi: 10.1016/s0022-3476(05)83478-x. [DOI] [PubMed] [Google Scholar]
  6. Gaskin K. J., Waters D. L., Baur L. A., Soutter V. L., Gruca M. A. Nutritional status, growth and development in children undergoing intensive treatment for cystic fibrosis. Acta Paediatr Scand Suppl. 1990;366:106–110. doi: 10.1111/j.1651-2227.1990.tb11610.x. [DOI] [PubMed] [Google Scholar]
  7. Høiby N., Pedersen S. S. Estimated risk of cross-infection with Pseudomonas aeruginosa in Danish cystic fibrosis patients. Acta Paediatr Scand. 1989 May;78(3):395–404. doi: 10.1111/j.1651-2227.1989.tb11099.x. [DOI] [PubMed] [Google Scholar]
  8. Kaas Ibsen K. Factors influencing blood pressure in children and adolescents. Acta Paediatr Scand. 1985 May;74(3):416–422. doi: 10.1111/j.1651-2227.1985.tb10995.x. [DOI] [PubMed] [Google Scholar]
  9. Koch C., Høiby N. Cystic fibrosis. 7. Management of cystic fibrosis in different countries. Cystic fibrosis in Copenhagen. Thorax. 1991 May;46(5):385–390. doi: 10.1136/thx.46.5.385. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Levy L. D., Durie P. R., Pencharz P. B., Corey M. L. Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J Pediatr. 1985 Aug;107(2):225–230. doi: 10.1016/s0022-3476(85)80130-x. [DOI] [PubMed] [Google Scholar]
  11. Mansell A. L., Andersen J. C., Muttart C. R., Ores C. N., Loeff D. S., Levy J. S., Heird W. C. Short-term pulmonary effects of total parenteral nutrition in children with cystic fibrosis. J Pediatr. 1984 May;104(5):700–705. doi: 10.1016/s0022-3476(84)80947-6. [DOI] [PubMed] [Google Scholar]
  12. O'Loughlin E., Forbes D., Parsons H., Scott B., Cooper D., Gall G. Nutritional rehabilitation of malnourished patients with cystic fibrosis. Am J Clin Nutr. 1986 May;43(5):732–737. doi: 10.1093/ajcn/43.5.732. [DOI] [PubMed] [Google Scholar]
  13. Pedersen S. S., Jensen T., Høiby N., Koch C., Flensborg E. W. Management of Pseudomonas aeruginosa lung infection in Danish cystic fibrosis patients. Acta Paediatr Scand. 1987 Nov;76(6):955–961. doi: 10.1111/j.1651-2227.1987.tb17271.x. [DOI] [PubMed] [Google Scholar]
  14. Pedersen S. S., Jensen T., Høiby N., Koch C., Flensborg E. W. Management of Pseudomonas aeruginosa lung infection in Danish cystic fibrosis patients. Acta Paediatr Scand. 1987 Nov;76(6):955–961. doi: 10.1111/j.1651-2227.1987.tb17271.x. [DOI] [PubMed] [Google Scholar]
  15. Schwartz M., Johansen H. K., Koch C., Brandt N. J. Frequency of the delta F508 mutation on cystic fibrosis chromosomes in Denmark. Hum Genet. 1990 Sep;85(4):427–428. doi: 10.1007/BF02428297. [DOI] [PubMed] [Google Scholar]
  16. Shepherd R. W., Holt T. L., Cleghorn G., Ward L. C., Isles A., Francis P. Short-term nutritional supplementation during management of pulmonary exacerbations in cystic fibrosis: a controlled study, including effects of protein turnover. Am J Clin Nutr. 1988 Aug;48(2):235–239. doi: 10.1093/ajcn/48.2.235. [DOI] [PubMed] [Google Scholar]
  17. Shepherd R. W., Holt T. L., Thomas B. J., Kay L., Isles A., Francis P. J., Ward L. C. Nutritional rehabilitation in cystic fibrosis: controlled studies of effects on nutritional growth retardation, body protein turnover, and course of pulmonary disease. J Pediatr. 1986 Nov;109(5):788–794. doi: 10.1016/s0022-3476(86)80695-3. [DOI] [PubMed] [Google Scholar]
  18. Shepherd R., Cooksley W. G., Cooke W. D. Improved growth and clinical, nutritional, and respiratory changes in response to nutritional therapy in cystic fibrosis. J Pediatr. 1980 Sep;97(3):351–357. doi: 10.1016/s0022-3476(80)80180-6. [DOI] [PubMed] [Google Scholar]
  19. Soutter V. L., Kristidis P., Gruca M. A., Gaskin K. J. Chronic undernutrition/growth retardation in cystic fibrosis. Clin Gastroenterol. 1986 Jan;15(1):137–155. [PubMed] [Google Scholar]
  20. Suter S., Schaad U. B., Roux-Lombard P., Girardin E., Grau G., Dayer J. M. Relation between tumor necrosis factor-alpha and granulocyte elastase-alpha 1-proteinase inhibitor complexes in the plasma of patients with cystic fibrosis. Am Rev Respir Dis. 1989 Dec;140(6):1640–1644. doi: 10.1164/ajrccm/140.6.1640. [DOI] [PubMed] [Google Scholar]
  21. Szaff M., Høiby N., Flensborg E. W. Frequent antibiotic therapy improves survival of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection. Acta Paediatr Scand. 1983 Sep;72(5):651–657. doi: 10.1111/j.1651-2227.1983.tb09789.x. [DOI] [PubMed] [Google Scholar]
  22. Vaisman N., Clarke R., Pencharz P. B. Nutritional rehabilitation increases resting energy expenditure without affecting protein turnover in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 1991 Nov;13(4):383–390. doi: 10.1097/00005176-199111000-00008. [DOI] [PubMed] [Google Scholar]
  23. Vaisman N., Pencharz P. B., Corey M., Canny G. J., Hahn E. Energy expenditure of patients with cystic fibrosis. J Pediatr. 1987 Oct;111(4):496–500. doi: 10.1016/s0022-3476(87)80107-5. [DOI] [PubMed] [Google Scholar]
  24. Zeitlin S. R., Bond S., Wootton S., Gregson R. K., Radford M. Increased resting energy expenditure in childhood asthma: does this contribute towards growth failure? Arch Dis Child. 1992 Nov;67(11):1366–1369. doi: 10.1136/adc.67.11.1366. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Thorax are provided here courtesy of BMJ Publishing Group

RESOURCES