Clinical trial NCT01806025: Correlation Between Haptoglobin Phenotypes and Infectious and Other Complications in Cystic Fibrosis Patients
Official title: Correlation Between Haptoglobin Phenotypes and Infectious and Other Complications in Cystic Fibrosis Patients
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Cystic Fibrosis
Keywords: Cystic Fibrosis
Haptoglobin
Lead sponsor: Carmel Medical Center
Sponsor class: OTHER
First posted: 2013-03-07
Enrollment: 142
Sex: ALL
Maximum age (years): 60
Standard ages: CHILD
ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: RETROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patients diagnosed with CF

Brief summary: 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.

Detailed description: 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.

Eligibility criteria: Inclusion Criteria:

Patients diagnosed with CF according to diagnostic criteria , between the ages of 0 and 50, who are themselves, or their parents or guardians, able to give informed consent.

Two known severe (class I , II and III) mutations

Exclusion Criteria:

none

Primary outcomes:
measure: FEV1
timeFrame: best in last 6 months
Secondary outcomes:
measure: Number of antibiotic courses per year of follow up
timeFrame: one year
measure: Number of days with antibiotics per year of follow up
timeFrame: one year
measure: Number Hospitalizations per year
timeFrame: one year
measure: Colonization with bacteria
timeFrame: one year
measure: Presence of CF related diabetes
timeFrame: five years
Other outcomes:
measure: Haptoglobin phenotype
timeFrame: one visit
Locations:
city: Haifa
country: Israel
facility: Carmel Medical Center
Study officials:
affiliation: Pulmonology Institute and CF Center, Carmel Medical Center
name: Michal Shteinberg, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Shteinberg M, Rivlin J, Gur M, Konopnicki M, Stein N, Tunney MM, Elborn JS, Downey DG, Johnston E, Shalom H, Levy A. Lack of Association Between Haptoglobin Phenotype and Cystic Fibrosis Outcomes. Lung. 2015 Dec;193(6):1017-21. doi: 10.1007/s00408-015-9801-z. Epub 2015 Sep 14.