Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis

[Guest guest]

Longitudinal evaluation of bronchopulmonary disease in children with cystic

fibrosis

Online ISSN: 1099-0496 Print ISSN: 8755-6863

Pediatric Pulmonology

Volume 36, Issue 3, 2003. Pages: 230-240

Published Online: 4 Aug 2003

Copyright © 2003 Wiley-Liss, Inc.

Longitudinal evaluation of bronchopulmonary disease in

children with cystic fibrosis

Philip M. Farrell, MD, PhD 1 *, Zhanhai Li, PhD 1 2,

R. Kosorok, PhD 1 2, Anita Laxova, BS 1,

G. Green, MD 1, Jannette , MD, MEd

3, Hui-Chuan Lai, PhD 4, M Makholm, MT 1,

J. Rock, MD 1, Mark L. Splaingard, MD 5

1Department of Pediatrics, University of Wisconsin,

Madison, Wisconsin

2Department of Biostatistics/Medical Informatics,

University of Wisconsin, Madison, Wisconsin

3Department of Radiology, University of Wisconsin,

Madison, Wisconsin

4Department of Nutritional Sciences, University of

Wisconsin, Madison, Wisconsin

5Department of Pediatrics, Medical College of

Wisconsin, Milwaukee, Wisconsin

email: Philip M. Farrell (pmfarrel@...)

*Correspondence to Philip M. Farrell, Department of

Pediatrics, University of Wisconsin Medical School,

Room 1217 MSC, 1300 University Ave., Madison, WI

53706-1532.

Funded by:

National Institutes of Health; Grant Number: DK

34108, M01 RR03186

Cystic Fibrosis Foundation; Grant Number: A001-5-01

Keywords

cystic fibrosis . lung . infection . pseudomas .

epidemiology . bronchopheumonia . pulmonary function

Abstract

Children with cystic fibrosis (CF) develop

bronchopulmonary disease at variable ages. Determining

the epidemiology of chronic lung disease and

quantifying its severity, however, have been difficult

in infants and young children. As part of the

Wisconsin CF Neonatal Screening Project, we were

presented with an ideal opportunity to assess

longitudinally the evolution of symptoms, signs, and

quantitative measures of CF respiratory disease. After

newborn screening test results led to early

recognition, 64 patients diagnosed at a median age of

6.71 weeks were enrolled and studied systematically at

a median age of 11.3 years to obtain clinical

information, chest radiographs, and pulmonary function

tests. Our observations revealed that a frequent cough

by history is evident by 10.5 months of age in half

the patients. Quantitative chest radiology (CXR

scoring) demonstrated that potentially irreversible

abnormalities are present in half the children by 2

years. The severity of Wisconsin and Brasfield CXR

scores increased in association with respiratory

infections. Longitudinal progression of Wisconsin CXR

scores was related to age (P < 0.001), pancreatic

insufficiency (P = 0.005), and respiratory secretion

cultures positive for Staphylococus aureas (P =

0.039). In contrast, serial spirometry showed limited

sensitivity, as did lung volume determinations;

neither was satisfactory as repeated measures with

acceptable quality control until after 7 years of age.

Time to event analyses revealed that half the patients

had % predicted FEF25-75 and FEV1/FVC values greater

than 80% until 10.7 and 9.9 years, respectively. We

conclude that of the methods evaluated, quantitative

chest radiology is currently the best procedure for

frequent assessment of bronchopulmonary disease in CF,

and that radiographic progression is evident in

approximately 85% of patients by 5 years of age. Our

results also suggest that bronchiectasis and other

radiographic evidence of chronic infection are

apparent prior to airways obstruction in young CF

patients.

Pediatr Pulmonol. 2003; 36:230-240. © 2003 Wiley-Liss,

Inc.

----------------------------------------------------------------------------

----

Received: 4 October 2002; Accepted: 24 March 2003