title: Lack of Association Between Haptoglobin Phenotype and Cystic Fibrosis Outcomes.
abstract: Haptoglobin (Hp), a heme-Iron chelator, has different isoforms which are associated with variable tendency toward infections: Hp 1-1, Hp 2-1, and Hp 2-2. Cystic fibrosis (CF) outcomes are variable and influenced by genetic and environmental factors. The aim of this study was to determine whether Hp phenotype influenced disease severity in CF. One hundred forty-two CF patients from two centers were analyzed for Haptoglobin phenotype using gel electrophoresis of hemoglobin enriched serum. Clinical and microbiological data including bacterial colonization status, lung function, presence of CF-related diabetes and liver disease, rate of exacerbation, and mortality were compared between Hp phenotype groups. We found a trend toward less mucoid PA among Hp 2-2 (20.4 %) compared with Hp 1-1 and Hp 2-1 individuals (33.3 %), p = 0.317. Hp 2-2 individuals also had less antibiotic courses, and lower inflammatory markers without statistical significance. Haptoglobin phenotype is unlikely to be an important modifier of CF phenotype.
authors.affiliation: CF Center, Carmel Medical Center, Haifa, Israel. michalsh@tx.technion.ac.il.|Queens University Belfast, Belfast, UK. michalsh@tx.technion.ac.il.|The B. Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel. michalsh@tx.technion.ac.il.|Pulnonary Institute, Carmel Medical Center, 7 Michal St., 3463209, Haifa, Israel. michalsh@tx.technion.ac.il.
authors.forename: Michal
authors.initials: M
authors.lastname: Shteinberg
authors.affiliation: CF Center, Carmel Medical Center, Haifa, Israel.
authors.forename: Joseph
authors.initials: J
authors.lastname: Rivlin
authors.affiliation: CF Center, Carmel Medical Center, Haifa, Israel.
authors.forename: Michal
authors.initials: M
authors.lastname: Gur
authors.affiliation: CF Center, Carmel Medical Center, Haifa, Israel.
authors.forename: Muriel
authors.initials: M
authors.lastname: Konopnicki
authors.affiliation: CF Center, Carmel Medical Center, Haifa, Israel.
authors.forename: Nili
authors.initials: N
authors.lastname: Stein
authors.affiliation: Queens University Belfast, Belfast, UK.
authors.forename: Michael M
authors.initials: MM
authors.lastname: Tunney
authors.affiliation: Queens University Belfast, Belfast, UK.
authors.forename: J Stuart
authors.initials: JS
authors.lastname: Elborn
authors.affiliation: Queens University Belfast, Belfast, UK.
authors.forename: Damian G
authors.initials: DG
authors.lastname: Downey
authors.affiliation: Queens University Belfast, Belfast, UK.
authors.forename: Elinor
authors.initials: E
authors.lastname: Johnston
authors.affiliation: The B. Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.
authors.forename: Hadar
authors.initials: H
authors.lastname: Shalom
authors.affiliation: The B. Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.
authors.forename: Andrew
authors.initials: A
authors.lastname: Levy
mesh_terms: D000293:Adolescent; D000328:Adult; D000483:Alleles; D002353:Carrier State / Q000378:metabolism*; D002648:Child; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000378:metabolism / Q000382:microbiology* / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D006242:Haptoglobins / Q000235:genetics*; D006454:Hemoglobins / Q000378:metabolism; D006579:Heterozygote; D006720:Homozygote; D006760:Hospitalization; D006801:Humans; D007501:Iron / Q000097:blood; D008297:Male; D055624:Methicillin-Resistant Staphylococcus aureus; D010641:Phenotype; D011379:Prognosis; D011552:Pseudomonas Infections / Q000235:genetics*; D011550:Pseudomonas aeruginosa; D013203:Staphylococcal Infections / Q000235:genetics*; D013211:Staphylococcus aureus; D014797:Vital Capacity; D055815:Young Adult
chemical_list: C485359:HP protein, human; D006242:Haptoglobins; D006454:Hemoglobins; D007501:Iron
keywords: Cystic fibrosis; Haptoglobin; Phenotypes