Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort.
BACKGROUND
The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young children with cystic fibrosis (CF).


OBJECTIVE
To describe the baseline characteristics of the cohort and evaluate associations between potential risk factors and nutritional and respiratory characteristics at enrollment. We hypothesized that distinct demographic and environmental risk factors could be identified for poorer nutritional status and lung function at enrollment.


METHODS
During 2004-2006, 1,700 children with CF were enrolled at 59 US CF centers. Children <or=12 years were eligible if they had no prior Pa infection (Pa-Never) or, if prior isolation of Pa from respiratory cultures, at least a 2-year history of Pa negative cultures (Pa-Past).


RESULTS
One thousand one hundred seventeen participants (65.7%) were Pa-Never and 583 (34.3%) Pa-Past. Pa-never patients had a lower proportion of CFTR genotypes with both mutations in functional classes I, II, or III), higher lung function and less respiratory symptoms. Diagnosis after newborn or prenatal screening was associated with significantly higher mean weight, height, and FEV(1) at enrollment, while maternal smoking during pregnancy appeared to worsen these parameters.


CONCLUSIONS
Children in this cohort with a remote history of Pa infection had a higher proportion of CFTR genotypes associated with severely reduced CFTR function as well as lower lung function and more respiratory symptoms than those without prior Pa infection. These observed differences in respiratory indices may reflect the impact of prior Pa airway infection and/or of CFTR genotype or other genetic factors predisposing both to earlier Pa acquisition and more severe lung disease. Key characteristics associated with nutritional and pulmonary status at enrollment included diagnosis after prenatal or neonatal screening (protective) and in utero cigarette exposure (harmful).
Rosenfeld
Margaret
M
Department of Pediatrics, Seattle Children's Hospital and University of Washington, Seattle, Washington, USA. margaret.rosenfeld@seattlechildrens.org
Emerson
Julia
J
McNamara
Sharon
S
Joubran
Kelli
K
Retsch-Bogart
George
G
Graff
Gavin R
GR
Gutierrez
Hector H
HH
Kanga
Jamshed F
JF
Lahiri
Thomas
T
Noyes
Blake
B
Ramsey
Bonnie
B
Ren
Clement L
CL
Schechter
Michael
M
Morgan
Wayne
W
Gibson
Ronald L
RL
D002353:Carrier State / Q000382:microbiology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000235:genetics*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D009752:Nutritional Status; D011552:Pseudomonas Infections / Q000235:genetics*; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification*; D012141:Respiratory Tract Infections / Q000235:genetics*; D013183:Sputum / Q000382:microbiology
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator