Interesting CF Article

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American Journal of Respiratory and Critical Care Medicine Vol 167. pp.

390-394, (2003)

© 2003 American Thoracic Society

Original Article

Endothelial Nitric Oxide Synthase Variants in Cystic Fibrosis Lung

Disease

Hartmut Grasemann, Karin Storm van's Gravesande, Rainer Büscher, Nicola

Knauer, S. Silverman, Lyle J. Palmer, M. Drazen and Felix

Ratjen Children's Hospital, University of Essen, Essen, Germany;

Department of Medicine and Channing Laboratory, Brigham and Women's

Hospital, Harvard Medical School, Boston, Massachusetts; and Department

of Epidemiology and Biostatistics, Case Western Reserve University,

Cleveland, Ohio Correspondence: Correspondence and requests for reprints

should be addressed to Dr. Hartmut Grasemann, Children's Hospital,

University of Essen, Hufeland Str. 55, D-45122 Essen, Germany.

E-mail:

hartmutg@...

Variants in the genes encoding for the nitric oxide

synthases may act as disease modifier loci in cystic fibrosis, affecting

both an individual's nitric oxide level and pulmonary function. In this

study, the 894G/T variant in exon 7 of the endothelial nitric oxide

synthase gene was related to exhaled nitric oxide and pulmonary function

in 70 cystic fibrosis patients who were aged 14.8 ± 6.9 years (mean ±

SD), with a FEV1 of 69.4 ± 24.8% predicted. Although there was no

association between endothelial nitric oxide synthase genotypes and

exhaled nitric oxide in males, nitric oxide levels were significantly

higher in female cystic fibrosis patients with an 894T mutant allele,

compared with female patients homozygous for the 894G wild-type allele

(7.0 ± 4.4 versus 3.6 ± 1.9 parts per billion, p = 0.02). Furthermore, in

female patients, colonization of airways with Pseudomonas aeruginosa was

significantly (p < 0.05) less frequent when carrying an 894T mutant

allele as compared with wild type. These data suggest that the 894T

variant in the endothelial nitric oxide synthase gene is associated with

increased airway nitric oxide formation in female cystic fibrosis

patients, possibly affecting colonization of airways with P. aeruginosa.

Key Words: endothelial nitric oxide synthase • exhaled nitric oxide •

cystic fibrosis • Pseudomonas aeruginosa

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