Cystic Fibrosis is a genetic disease with variable severity, and a predisposition for lung infection. Usually severity is determined by the class of CF mutations, but even among patients with the same severity of mutations there is a variation of the severity of CF. Haptoglobin has several types (phenotypes), one of them was found to be related to infectious complications. In this study the investigators aim to find a correlation between Haptoglobin phenotypes in patients with CF and frequency of infectious complications. To this end the investigators will collect serum from CF patients, and determine their Haptoglobin protein phenotype. The investigators will correlate Haptoglobin phenotype to retrospectively gathered data on infectious complications.
Cystic Fibrosis is a genetic disease with variable severity, and a predisposition for lung infection. The severity of the disease is determined by genetic factors (type of mutation), environmental factors (exposure to bacteria) and behavioral (adherence with therapy). Even among patients with the same severity of mutations there is a variation of the severity of CF. Haptoglobin is a protein responsible for collecting Iron from senescent Red Blood Cells. There are two genes of Haptoglobin, numbered 1 and 2, and combinations between the two genes create three forms of proteins: 1-1, 1-2, and 2-2. The 1-1 Phenotype was found to be associated with a predisposition to infection. In this study the investigators aim to find a correlation between Haptoglobin phenotypes in patients with CF and frequency of infectious complications. To this end the investigators will collect serum from CF patients, and determine their Haptoglobin protein phenotype by gel- electrophoresis. The investigators will correlate Haptoglobin phenotype to retrospectively gathered data on infectious complications. FEV1- Forced Expiratory Volume in 1 second.
Study Type
OBSERVATIONAL
Enrollment
142
Carmel Medical Center
Haifa, Israel, Israel
FEV1
Lung function as determined by spirometry FEV1 (% expected), normalized by age
Time frame: best in last 6 months
Number of antibiotic courses per year of follow up
number of courses of antibiotics the patient received in the last year
Time frame: one year
Number of days with antibiotics per year of follow up
number of days the patient received antibiotics
Time frame: one year
Number Hospitalizations per year
events of hospitalization
Time frame: one year
Colonization with bacteria
colonization of the following bacteria: Pseudomonas aeruginosa (mucoid and non mucoid), Staph aureus (MSSA and MRSA), Hemophilus influenza, Burkholderia Cepacia complex
Time frame: one year
Presence of CF related diabetes
presence of CF related diabetes and HbA1C for diabetic patients.
Time frame: five years
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