Lung function in infants with cystic fibrosis diagnosed by newborn screening

B.M. Linnane, G. L. Hall, G. Nolan, S. Brennan, S. M. Stick, Peter D. Sly, C. F. Robertson, P. J. Franklin, Stephen William Turner, S.C. Ranganathan, C.F. AREST

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Abstract

RATIONALE: Progressive lung damage in cystic fibrosis (CF) starts in infancy, and early detection may aid preventative strategies.

OBJECTIVES: To measure lung function in infants with CF diagnosed by newborn screening and describe its association with pulmonary infection and inflammation.

METHODS: Infants with CF (n = 68, 6 weeks to 30 months of age) and healthy infants without CF (n = 49) were studied. Forced vital capacity, FEV(0.5), and forced expiratory flows at 75% of exhaled vital capacity (FEF(75)) were measured using the raised-volume rapid thoracoabdominal compression technique. Forty-eight hours later, infants with CF had bronchoalveolar lavage (BAL) for assessment of pulmonary infection and inflammation.

MEASUREMENTS AND MAIN RESULTS: In the CF group, the deficit in FEV(0.5) z score increased by -0.77 (95% confidence interval, -1.14 to -0.41; P < 0.001) with each year of age. The mean FEV(0.5) z score did not differ between infants with CF and healthy control subjects less than 6 months of age (-0.06 and 0.02, respectively; P = 0.87). However, the mean FEV(0.5) z score was lower by 1.15 in infants with CF who were older than 6 months of age compared with healthy infants (P < 0.001). FVC and FEF(75) followed a similar pattern. Pulmonary infection and inflammation in BAL samples did not explain the lung function results.

CONCLUSIONS: Lung function, measured by forced expiration, is normal in infants with CF at the time of diagnosis by newborn screening but is diminished in older infants. These findings suggest that in CF the optimal timing of therapeutic interventions aimed at preserving lung function may be within the first 6 months of life.

Original languageEnglish
Pages (from-to)1238-1244
Number of pages7
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume178
Issue number12
DOIs
Publication statusPublished - 15 Dec 2008

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Cystic Fibrosis
Newborn Infant
Lung
Pneumonia
Vital Capacity
Bronchoalveolar Lavage
Infection
Healthy Volunteers
Confidence Intervals

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Linnane, B. M., Hall, G. L., Nolan, G., Brennan, S., Stick, S. M., Sly, P. D., ... AREST, C. F. (2008). Lung function in infants with cystic fibrosis diagnosed by newborn screening. American Journal of Respiratory and Critical Care Medicine, 178(12), 1238-1244. https://doi.org/10.1164/rccm.200804-551OC

Lung function in infants with cystic fibrosis diagnosed by newborn screening. / Linnane, B.M.; Hall, G. L.; Nolan, G.; Brennan, S.; Stick, S. M.; Sly, Peter D.; Robertson, C. F.; Franklin, P. J.; Turner, Stephen William; Ranganathan, S.C.; AREST, C.F.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 178, No. 12, 15.12.2008, p. 1238-1244.

Research output: Contribution to journalArticle

Linnane, BM, Hall, GL, Nolan, G, Brennan, S, Stick, SM, Sly, PD, Robertson, CF, Franklin, PJ, Turner, SW, Ranganathan, SC & AREST, CF 2008, 'Lung function in infants with cystic fibrosis diagnosed by newborn screening', American Journal of Respiratory and Critical Care Medicine, vol. 178, no. 12, pp. 1238-1244. https://doi.org/10.1164/rccm.200804-551OC
Linnane, B.M. ; Hall, G. L. ; Nolan, G. ; Brennan, S. ; Stick, S. M. ; Sly, Peter D. ; Robertson, C. F. ; Franklin, P. J. ; Turner, Stephen William ; Ranganathan, S.C. ; AREST, C.F. / Lung function in infants with cystic fibrosis diagnosed by newborn screening. In: American Journal of Respiratory and Critical Care Medicine. 2008 ; Vol. 178, No. 12. pp. 1238-1244.
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abstract = "RATIONALE: Progressive lung damage in cystic fibrosis (CF) starts in infancy, and early detection may aid preventative strategies. OBJECTIVES: To measure lung function in infants with CF diagnosed by newborn screening and describe its association with pulmonary infection and inflammation. METHODS: Infants with CF (n = 68, 6 weeks to 30 months of age) and healthy infants without CF (n = 49) were studied. Forced vital capacity, FEV(0.5), and forced expiratory flows at 75{\%} of exhaled vital capacity (FEF(75)) were measured using the raised-volume rapid thoracoabdominal compression technique. Forty-eight hours later, infants with CF had bronchoalveolar lavage (BAL) for assessment of pulmonary infection and inflammation. MEASUREMENTS AND MAIN RESULTS: In the CF group, the deficit in FEV(0.5) z score increased by -0.77 (95{\%} confidence interval, -1.14 to -0.41; P < 0.001) with each year of age. The mean FEV(0.5) z score did not differ between infants with CF and healthy control subjects less than 6 months of age (-0.06 and 0.02, respectively; P = 0.87). However, the mean FEV(0.5) z score was lower by 1.15 in infants with CF who were older than 6 months of age compared with healthy infants (P < 0.001). FVC and FEF(75) followed a similar pattern. Pulmonary infection and inflammation in BAL samples did not explain the lung function results. CONCLUSIONS: Lung function, measured by forced expiration, is normal in infants with CF at the time of diagnosis by newborn screening but is diminished in older infants. These findings suggest that in CF the optimal timing of therapeutic interventions aimed at preserving lung function may be within the first 6 months of life.",
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T1 - Lung function in infants with cystic fibrosis diagnosed by newborn screening

AU - Linnane, B.M.

AU - Hall, G. L.

AU - Nolan, G.

AU - Brennan, S.

AU - Stick, S. M.

AU - Sly, Peter D.

AU - Robertson, C. F.

AU - Franklin, P. J.

AU - Turner, Stephen William

AU - Ranganathan, S.C.

AU - AREST, C.F.

PY - 2008/12/15

Y1 - 2008/12/15

N2 - RATIONALE: Progressive lung damage in cystic fibrosis (CF) starts in infancy, and early detection may aid preventative strategies. OBJECTIVES: To measure lung function in infants with CF diagnosed by newborn screening and describe its association with pulmonary infection and inflammation. METHODS: Infants with CF (n = 68, 6 weeks to 30 months of age) and healthy infants without CF (n = 49) were studied. Forced vital capacity, FEV(0.5), and forced expiratory flows at 75% of exhaled vital capacity (FEF(75)) were measured using the raised-volume rapid thoracoabdominal compression technique. Forty-eight hours later, infants with CF had bronchoalveolar lavage (BAL) for assessment of pulmonary infection and inflammation. MEASUREMENTS AND MAIN RESULTS: In the CF group, the deficit in FEV(0.5) z score increased by -0.77 (95% confidence interval, -1.14 to -0.41; P < 0.001) with each year of age. The mean FEV(0.5) z score did not differ between infants with CF and healthy control subjects less than 6 months of age (-0.06 and 0.02, respectively; P = 0.87). However, the mean FEV(0.5) z score was lower by 1.15 in infants with CF who were older than 6 months of age compared with healthy infants (P < 0.001). FVC and FEF(75) followed a similar pattern. Pulmonary infection and inflammation in BAL samples did not explain the lung function results. CONCLUSIONS: Lung function, measured by forced expiration, is normal in infants with CF at the time of diagnosis by newborn screening but is diminished in older infants. These findings suggest that in CF the optimal timing of therapeutic interventions aimed at preserving lung function may be within the first 6 months of life.

AB - RATIONALE: Progressive lung damage in cystic fibrosis (CF) starts in infancy, and early detection may aid preventative strategies. OBJECTIVES: To measure lung function in infants with CF diagnosed by newborn screening and describe its association with pulmonary infection and inflammation. METHODS: Infants with CF (n = 68, 6 weeks to 30 months of age) and healthy infants without CF (n = 49) were studied. Forced vital capacity, FEV(0.5), and forced expiratory flows at 75% of exhaled vital capacity (FEF(75)) were measured using the raised-volume rapid thoracoabdominal compression technique. Forty-eight hours later, infants with CF had bronchoalveolar lavage (BAL) for assessment of pulmonary infection and inflammation. MEASUREMENTS AND MAIN RESULTS: In the CF group, the deficit in FEV(0.5) z score increased by -0.77 (95% confidence interval, -1.14 to -0.41; P < 0.001) with each year of age. The mean FEV(0.5) z score did not differ between infants with CF and healthy control subjects less than 6 months of age (-0.06 and 0.02, respectively; P = 0.87). However, the mean FEV(0.5) z score was lower by 1.15 in infants with CF who were older than 6 months of age compared with healthy infants (P < 0.001). FVC and FEF(75) followed a similar pattern. Pulmonary infection and inflammation in BAL samples did not explain the lung function results. CONCLUSIONS: Lung function, measured by forced expiration, is normal in infants with CF at the time of diagnosis by newborn screening but is diminished in older infants. These findings suggest that in CF the optimal timing of therapeutic interventions aimed at preserving lung function may be within the first 6 months of life.

U2 - 10.1164/rccm.200804-551OC

DO - 10.1164/rccm.200804-551OC

M3 - Article

VL - 178

SP - 1238

EP - 1244

JO - American Journal of Respiratory and Critical Care Medicine

JF - American Journal of Respiratory and Critical Care Medicine

SN - 1073-449X

IS - 12

ER -