Correlation Between Haptoglobin Phenotypes and Infectious and Other Complications in Cystic Fibrosis Patients

Completed

• Conditions: Cystic Fibrosis
• Interventions: Other: no intervention

• Registration Number: NCT01806025
• Lead Sponsor: Carmel Medical Center

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.

Study Timeline

• Study First Submitted: 2013-03-05
• Study First Submitted QC: 2013-03-06
• Study First Posted: 2013-03-07
• Study Start: 2013-04
• Primary Completion: 2015-01
• Study Completion: 2015-01
• Status Verified: 2016-07
• Last Update Submitted: 2016-07-27
• Last Update Posted: 2016-07-28

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 142

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Cystic Fibrosis Patients	no intervention	Patients with Cystic Fibrosis with two known severe (class I and class II) mutations

Primary Outcome Measures

Name	Time	Method
FEV1	best in last 6 months	Lung function as determined by spirometry FEV1 (% expected), normalized by age

Secondary Outcome Measures

Name	Time	Method
Number of days with antibiotics per year of follow up	one year	number of days the patient received antibiotics
Number Hospitalizations per year	one year	events of hospitalization
Number of antibiotic courses per year of follow up	one year	number of courses of antibiotics the patient received in the last year
Presence of CF related diabetes	five years	presence of CF related diabetes and HbA1C for diabetic patients.
Colonization with bacteria	one year	colonization of the following bacteria: Pseudomonas aeruginosa (mucoid and non mucoid), Staph aureus (MSSA and MRSA), Hemophilus influenza, Burkholderia Cepacia complex

Trial Locations

Locations (1)

Carmel Medical Center; 🇮🇱 Haifa, Israel