Article on PFT's in 3-6yo

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American Journal of Respiratory and Critical Care Medicine

July 1, 2002 (Volume 166, Number 1)

Spirometry in 3- to 6-Year-Old Children With Cystic Fibrosis

Marostica PJC, Weist AD, Eigen H, et al.

American Journal of Respiratory and Critical Care Medicine.

2002;166(1):67-71

Introduction

Limited pulmonary function data exist for children with cystic fibrosis

(CF) who are 3 to 6 years of age. Early quantitative assessment could

allow for early intervention before irreversible lung damage occurs. The

current study was designed to evaluate the spectrum of airway disease in

clinically stable children with CF in this age group. In addition, a

subset of children who had lung function assessment as infants allowed

an evaluation of whether there was any relationship between lung

measurements from infancy and preschool.

Methods

Children with CF who were clinically stable at their baseline and

between 3 and 6 years of age were recruited. After coaching, spirometry

was performed in the upright position.[1] Total training and testing

time was limited to 20 minutes. Data were included only for children

able to perform a least 2 technically acceptable maneuvers. History

obtained included genotype, colonization with Pseudomonas aeruginosa,

spirometric assessments performed during infancy,[2,3] and Brasfield

scoring of the most recent chest radiograph.[4] The z scores were

calculated for forced vital capacity (FVC), forced expiratory volume in

1 second (FEV1), FVC/FEV1, and forced expiratory flow between 25% and

75% expired volume (FEF25-75) using previously published normative data

for children in this age group.[1]

Results

Technically acceptable spirometry was available from 33 of 38 children

enrolled. The coefficients of variation (CVs) for FVC, FEV1, FEV1/FVC,

and FEF25-75 were 2.2%, 2.4%, 2.0%, and 7.7%, respectively. There was no

difference in variability related to age. Abnormal results for FVC,

FEV1, FEF27-75, and FEV1/FVC were present in 24%, 27%, 17%, and 10% of

patients, respectively. When genotype was compared, subjects homozygous

for the delta-F508 mutation had significantly lower z scores for FVC (P

< .01) and FEV1 (P < .05) vs heterozygotes. There was a positive

correlation between Brasfield score and FVC and FEV1 z scores (r2 = .26,

P < .01 and r2 = .24, P < .01, respectively).

Measurements obtained during infancy were available for 14 subjects.

There was a significant correlation in z score values of FEF25-75 (r =

..72, P = .002). For subjects with normal values, there was no

significant interval change. However, for 4 subjects with abnormal z

scores in infancy, there was a significant increase between infancy and

early childhood (P < .05).

Discussion

The current study demonstrates that 3- to 6-year-old children with CF

can be successfully assessed with spirometry and that they have lower

pulmonary function than a previously studied healthy population. In

addition, measurement of the flow-volume curve can potentially be used

in the longitudinal assessment of lung function from infancy.

The authors noted that the variability in spirometric measurements in

this study is comparable to that seen in a larger group of healthy

preschool children.[1,5] However, a smaller percentage of CF vs healthy

children were able to perform 3 reproducible maneuvers.

The finding of lower FVC and FEV1 z scores in delta-F508 homozygous vs

heterozygous subjects is consistent with other studies.[6] In contrast,

unlike studies of older CF subjects,[7-9] colonization with P aeruginosa

did not correlate with lung function. The authors speculated that this

finding may be related to the younger age and lower colonization rate of

subjects in the current study.

The authors concluded by noting that spirometry in preschool children

can quantify lung function, can detect the present of airflow

obstruction, and has the potential for providing longitudinal assessment

in CF patients from infancy to adulthood.

Becki

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