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Nitric Oxide Bioavailability in Patients with Cystic Fibrosis
Author(s) -
Harris Ryan A,
Seigler Nichole,
Fox Brandon,
White Claire,
Brantley Lucy,
Eidson Dabney,
McKie Kathleen T
Publication year - 2013
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.27.1_supplement.1141.1
Subject(s) - cystic fibrosis , medicine , nitric oxide , lung function , cohort , endothelial dysfunction , bioavailability , gastroenterology , lung , pharmacology
Major advances in medical research and therapies have resulted in marked increases in longevity and lung function in patients with cystic fibrosis (CF); however, non‐pulmonary factors have been understudied. We recently published the presence of endothelial dysfunction in young patients with CF who exhibit normal lung function. This study tested the hypothesis that increasing NO bioavailability would improve endothelial function in patients with CF. Seventeen patients (7 boys and 10 girls) with CF (FEV1=89.5±19.4 % predicted) participated in this study. Flow‐mediated dilation (FMD) was performed at baseline and 3 hours following 5 mg/kg of Kuvan™ (BH4) dissolved in apple juice. No differences in FMD was observed following BH4; however, a significant relationship between the change in FMD following BH4 and baseline FMD was observed (r=−0.608; p=0.010). Absolute change in FMD was greater (p=0.047) in boys (0.021±0.007cm) compared to girls (0.012±0.009cm). Additionally, a statistical time x sex interaction (p=0.02) was observed, such that girls exhibited an increase in FMD (0.6±2.3%) following BH4, whereas a decrease in FMD (−2.1±2.0%) was observed in boys. These data indicate that the FMD response to BH4 in a fairly healthy cohort of patients with CF is in part dependent on baseline FMD. Supported in part by the American Heart Association, Child Health Discovery Institute and BioMarin Pharmaceutical Inc. (RAH).