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. 1995 Dec;73(6):519–523. doi: 10.1136/adc.73.6.519

Lung function from infancy to school age in cystic fibrosis.

C S Beardsmore 1
PMCID: PMC1511453  PMID: 8546509

Abstract

The aim was to investigate pulmonary mechanics in patients with cystic fibrosis during infancy and again in early childhood to see whether infant tests predicted status at school age. Plethysmographic measurements of thoracic gas volume and airways resistance were made in 29 patients at 6 months and again at 5 years 10 months. Maximum flow at functional residual capacity was measured during infancy for comparison with maximum expiratory flow rates during childhood. While many patients had normal measurements during infancy, pulmonary function had deteriorated by school age. Thoracic gas volume at school age was significantly related to the values in infancy, but other measurements made during childhood were independent of infant values. The relations between measurements in infancy and early childhood described here provide a background against which the role of external factors on pulmonary function in young children with cystic fibrosis can be investigated.

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Selected References

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  1. Beardsmore C. S., Bar-Yishay E., Maayan C., Yahav Y., Katznelson D., Godfrey S. Lung function in infants with cystic fibrosis. Thorax. 1988 Jul;43(7):545–551. doi: 10.1136/thx.43.7.545. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Beardsmore C. S., Thompson J. R., Williams A., McArdle E. K., Gregory G. A., Weaver L. T., Simpson H. Pulmonary function in infants with cystic fibrosis: the effect of antibiotic treatment. Arch Dis Child. 1994 Aug;71(2):133–137. doi: 10.1136/adc.71.2.133. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Chan K. N., Noble-Jamieson C. M., Elliman A., Bryan E. M., Silverman M. Lung function in children of low birth weight. Arch Dis Child. 1989 Sep;64(9):1284–1293. doi: 10.1136/adc.64.9.1284. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Chan K. N., Wong Y. C., Silverman M. Relationship between infant lung mechanics and childhood lung function in children of very low birthweight. Pediatr Pulmonol. 1990;8(2):74–81. doi: 10.1002/ppul.1950080204. [DOI] [PubMed] [Google Scholar]
  5. Coates A. L., Bergsteinsson H., Desmond K., Outerbridge E. W., Beaudry P. H. Long-term pulmonary sequelae of premature birth with and without idiopathic respiratory distress syndrome. J Pediatr. 1977 Apr;90(4):611–616. doi: 10.1016/s0022-3476(77)80382-x. [DOI] [PubMed] [Google Scholar]
  6. Cooper P. J., Robertson C. F., Hudson I. L., Phelan P. D. Variability of pulmonary function tests in cystic fibrosis. Pediatr Pulmonol. 1990;8(1):16–22. doi: 10.1002/ppul.1950080107. [DOI] [PubMed] [Google Scholar]
  7. Corey M., Levison H., Crozier D. Five- to seven-year course of pulmonary function in cystic fibrosis. Am Rev Respir Dis. 1976 Dec;114(6):1085–1092. doi: 10.1164/arrd.1976.114.6.1085. [DOI] [PubMed] [Google Scholar]
  8. DUBOIS A. B., BOTELHO S. Y., BEDELL G. N., MARSHALL R., COMROE J. H., Jr A rapid plethysmographic method for measuring thoracic gas volume: a comparison with a nitrogen washout method for measuring functional residual capacity in normal subjects. J Clin Invest. 1956 Mar;35(3):322–326. doi: 10.1172/JCI103281. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Gilljam H., Stenlund C., Ericsson-Hollsing A., Strandvik B. Passive smoking in cystic fibrosis. Respir Med. 1990 Jul;84(4):289–291. doi: 10.1016/s0954-6111(08)80055-8. [DOI] [PubMed] [Google Scholar]
  10. Godfrey S., Mearns M., Howlett G. Serial lung function studies in cystic fibrosis in the first 5 years of life. Arch Dis Child. 1978 Jan;53(1):83–85. doi: 10.1136/adc.53.1.83. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Gozal D., Bailey S. L., Keens T. G. Evolution of pulmonary function during an acute exacerbation in hospitalized patients with cystic fibrosis. Pediatr Pulmonol. 1993 Dec;16(6):347–353. doi: 10.1002/ppul.1950160605. [DOI] [PubMed] [Google Scholar]
  12. Green M. R., Weaver L. T., Heeley A. F., Nicholson K., Kuzemko J. A., Barton D. E., McMahon R., Payne S. J., Austin S., Yates J. R. Cystic fibrosis identified by neonatal screening: incidence, genotype, and early natural history. Arch Dis Child. 1993 Apr;68(4):464–467. doi: 10.1136/adc.68.4.464. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Hampton F., Beardsmore C. S., Morgan W., Williams A., Taussig L., Thompson J. R. A scoring system for lung function tests in infants. Pediatr Pulmonol. 1992 Nov;14(3):149–155. doi: 10.1002/ppul.1950140303. [DOI] [PubMed] [Google Scholar]
  14. Hanrahan J. P., Tager I. B., Castile R. G., Segal M. R., Weiss S. T., Speizer F. E. Pulmonary function measures in healthy infants. Variability and size correction. Am Rev Respir Dis. 1990 May;141(5 Pt 1):1127–1135. doi: 10.1164/ajrccm/141.5_Pt_1.1127. [DOI] [PubMed] [Google Scholar]
  15. Hardy K. A., Wolfson M. R., Schidlow D. V., Shaffer T. H. Mechanics and energetics of breathing in newly diagnosed infants with cystic fibrosis: effect of combined bronchodilator and chest physical therapy. Pediatr Pulmonol. 1989;6(2):103–108. doi: 10.1002/ppul.1950060209. [DOI] [PubMed] [Google Scholar]
  16. Hordvik N. L., König P., Hamory B., Cooperstock M., Kreutz C., Gayer D., Barbero G. Effects of acute viral respiratory tract infections in patients with cystic fibrosis. Pediatr Pulmonol. 1989;7(4):217–222. doi: 10.1002/ppul.1950070406. [DOI] [PubMed] [Google Scholar]
  17. Kerem E., Corey M., Gold R., Levison H. Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa. J Pediatr. 1990 May;116(5):714–719. doi: 10.1016/s0022-3476(05)82653-8. [DOI] [PubMed] [Google Scholar]
  18. Loening-Baucke V. A., Mischler E., Myers M. G. A placebo-controlled trial of cephalexin therapy in the ambulatory management of patients with cystic fibrosis. J Pediatr. 1979 Oct;95(4):630–637. doi: 10.1016/s0022-3476(79)80785-4. [DOI] [PubMed] [Google Scholar]
  19. Pattishall E. N. Longitudinal response of pulmonary function to bronchodilators in cystic fibrosis. Pediatr Pulmonol. 1990;9(2):80–85. doi: 10.1002/ppul.1950090204. [DOI] [PubMed] [Google Scholar]
  20. Phelan P. D., Gracey M., Williams H. E., Anderson C. M. Ventilatory function in infants with cystic fibrosis. Physiological assessment of halation therapy. Arch Dis Child. 1969 Jun;44(235):393–400. doi: 10.1136/adc.44.235.393. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Ramsey B. W., Astley S. J., Aitken M. L., Burke W., Colin A. A., Dorkin H. L., Eisenberg J. D., Gibson R. L., Harwood I. R., Schidlow D. V. Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis. Am Rev Respir Dis. 1993 Jul;148(1):145–151. doi: 10.1164/ajrccm/148.1.145. [DOI] [PubMed] [Google Scholar]
  22. Rosenberg S. M., Schramm C. M. Predictive value of pulmonary function testing during pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol. 1993 Oct;16(4):227–235. doi: 10.1002/ppul.1950160404. [DOI] [PubMed] [Google Scholar]
  23. Solymar L., Aronsson P. H., Bake B., Bjure J. Nitrogen single breath test, flow-volume curves and spirometry in healthy children, 7-18 years of age. Eur J Respir Dis. 1980 Oct;61(5):275–286. [PubMed] [Google Scholar]
  24. Steinkamp G., Tümmler B., Gappa M., Albus A., Potel J., Döring G., von der Hardt H. Long-term tobramycin aerosol therapy in cystic fibrosis. Pediatr Pulmonol. 1989;6(2):91–98. doi: 10.1002/ppul.1950060207. [DOI] [PubMed] [Google Scholar]
  25. Stocks J., Levy N. M., Godfrey S. A new apparatus for the accurate measurement of airway resistance in infancy. J Appl Physiol Respir Environ Exerc Physiol. 1977 Jul;43(1):155–159. doi: 10.1152/jappl.1977.43.1.155. [DOI] [PubMed] [Google Scholar]
  26. Taussig L. M., Landau L. I., Godfrey S., Arad I. Determinants of forced expiratory flows in newborn infants. J Appl Physiol Respir Environ Exerc Physiol. 1982 Nov;53(5):1220–1227. doi: 10.1152/jappl.1982.53.5.1220. [DOI] [PubMed] [Google Scholar]
  27. Tepper R. S., Hiatt P. W., Eigen H., Smith J. Total respiratory system compliance in asymptomatic infants with cystic fibrosis. Am Rev Respir Dis. 1987 May;135(5):1075–1079. doi: 10.1164/arrd.1987.135.5.1075. [DOI] [PubMed] [Google Scholar]
  28. Tepper R. S., Hiatt P., Eigen H., Scott P., Grosfeld J., Cohen M. Infants with cystic fibrosis: pulmonary function at diagnosis. Pediatr Pulmonol. 1988;5(1):15–18. doi: 10.1002/ppul.1950050105. [DOI] [PubMed] [Google Scholar]
  29. Turner D. J., Lanteri C. J., LeSouef P. N., Sly P. D. Improved detection of abnormal respiratory function using forced expiration from raised lung volume in infants with cystic fibrosis. Eur Respir J. 1994 Nov;7(11):1995–1999. [PubMed] [Google Scholar]
  30. Turner D. J., Morgan S. E., Landau L. I., LeSouëf P. N. Methodological aspects of flow-volume studies in infants. Pediatr Pulmonol. 1990;8(4):289–293. doi: 10.1002/ppul.1950080414. [DOI] [PubMed] [Google Scholar]
  31. Weaver L. T., Green M. R., Nicholson K., Mills J., Heeley M. E., Kuzemko J. A., Austin S., Gregory G. A., Dux A. E., Davis J. A. Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period. Arch Dis Child. 1994 Feb;70(2):84–89. doi: 10.1136/adc.70.2.84. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Wong Y. C., Beardsmore C. S., Silverman M. Pulmonary sequelae of neonatal respiratory distress in very low birthweight infants: a clinical and physiological study. Arch Dis Child. 1982 Jun;57(6):418–424. doi: 10.1136/adc.57.6.418. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Zapletal A., Paul T., Samanek M. Pulmonary elasticity in children and adolescents. J Appl Physiol. 1976 Jun;40(6):953–961. doi: 10.1152/jappl.1976.40.6.953. [DOI] [PubMed] [Google Scholar]
  34. Zapletal A., Samanek M., Paul T. Upstream and total airway conductance in children and adolescents. Bull Eur Physiopathol Respir. 1982 Jan-Feb;18(1):31–37. [PubMed] [Google Scholar]

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