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Previous Volume 350:560-569 February 5, 2004 Number 6 Next

	Full Text PDF PDA Full Text PowerPoint Slide Set Editorial by Strandvik, B. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited E-mail When Letters Appear PubMed Citation

ABSTRACT

Background Patients with cystic fibrosis have altered levels of plasma fatty acids. We previously demonstrated that arachidonic acid levels are increased and docosahexaenoic acid levels are decreased in affected tissues from cystic fibrosis–knockout mice. In this study we determined whether humans with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene have a similar fatty acid defect in tissues expressing CFTR.

Methods Fatty acids from nasal- and rectal-biopsy specimens, nasal epithelial scrapings, and plasma were analyzed from 38 subjects with cystic fibrosis and compared with results in 13 obligate heterozygotes, 24 healthy controls, 11 subjects with inflammatory bowel disease, 9 subjects with upper respiratory tract infection, and 16 subjects with asthma.

Results The ratio of arachidonic to docosahexaenoic acid was increased in mucosal and submucosal nasal-biopsy specimens (P<0.001) and rectal-biopsy specimens (P=0.009) from subjects with cystic fibrosis and pancreatic sufficiency and subjects with cystic fibrosis and pancreatic insufficiency, as compared with values in healthy control subjects. In nasal tissue, this change reflected an increase in arachidonic acid levels and a decrease in docosahexaenoic acid levels. In cells from nasal mucosa, the ratio of arachidonic to docosahexaenoic acid was increased in subjects with cystic fibrosis (P<0.001), as compared with healthy controls, with values in obligate heterozygotes intermediate between these two groups (P<0.001). The ratio was not increased in subjects with inflammatory bowel disease. Subjects with asthma and those with upper respiratory tract infection had values intermediate between those in subjects with cystic fibrosis and those in healthy control subjects.

Conclusions These data indicate that alterations in fatty acids similar to those in cystic fibrosis–knockout mice are present in CFTR-expressing tissue from subjects with cystic fibrosis.

Source Information

From the Departments of Medicine (S.D.F., P.G.B., M.M.Z., J.C.S., M.O., I.K.H., D.A.W., A.G., M.M.R.) and Obstetrics and Gynecology (M.O.), Beth Israel Deaconess Medical Center and Harvard Medical School, Boston; the Division of Laboratory Medicine, Massachusetts General Hospital and Harvard Medical School, Boston (M.L.); Instituto de Infertilidad Masculina, Centro de Infertilidad Masculina Androgen, Hospital San Rafael, La Coruña, Spain (J.G.A.); and the Department of Pediatrics, UMass Memorial Health Care, University of Massachusetts Medical School, Worcester (B.P.O.).

Address reprint requests to Dr. Freedman at Beth Israel Deaconess Medical Center, Dana 552, 330 Brookline Ave., Boston, MA 02215, or at sfreedma{at}caregroup.harvard.edu.

Full Text of this Article

Related Letters:

Eicosanoids in Cystic Fibrosis
Kida Y., Steger B., Colvin H. P., Laposata M., O'Sullivan B. P., Freedman S. D.
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N Engl J Med 2004; 350:2000-2001, May 6, 2004. Correspondence

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