Correlation Between Haptoglobin Phenotypes and Infectious and Other Complications in Cystic Fibrosis Patients (Hp-in-CF)

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.

Study Overview

• Status: Completed
• Conditions: Cystic Fibrosis
• Intervention / Treatment: Other: no intervention

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 Type: Observational
• Enrollment (Actual): 142

Contacts and Locations

Study Locations

• Israel
  • Haifa, Israel, 3436209
    • Carmel Medical Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: No older than 60 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients diagnosed with CF

Description

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 Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Cystic Fibrosis Patients; Patients with Cystic Fibrosis with two known severe (class I and class II) mutations	Other: no intervention

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
FEV1	Lung function as determined by spirometry FEV1 (% expected), normalized by age	best in last 6 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Number of antibiotic courses per year of follow up	number of courses of antibiotics the patient received in the last year	one year
Number of days with antibiotics per year of follow up	number of days the patient received antibiotics	one year
Number Hospitalizations per year	events of hospitalization	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	one year
Presence of CF related diabetes	presence of CF related diabetes and HbA1C for diabetic patients.	five years

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Haptoglobin phenotype	Haptoglobin phenotype in serum will be determined by gel electrophoresis	one visit

Collaborators and Investigators

• Sponsor: Carmel Medical Center
• Investigators: Principal Investigator: Michal Shteinberg, MD, Pulmonology Institute and CF Center, Carmel Medical Center

Publications and helpful links

General Publications

• 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.

Study record dates

Study Major Dates

• Study Start: April 1, 2013
• Primary Completion (Actual): January 1, 2015
• Study Completion (Actual): January 1, 2015

Study Registration Dates

• First Submitted: March 5, 2013
• First Submitted That Met QC Criteria: March 6, 2013
• First Posted (Estimate): March 7, 2013

Study Record Updates

• Last Update Posted (Estimate): July 28, 2016
• Last Update Submitted That Met QC Criteria: July 27, 2016
• Last Verified: July 1, 2016

More Information

Terms related to this study

• Keywords: Cystic Fibrosis; Haptoglobin
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Pancreatic Diseases; Fibrosis; Cystic Fibrosis
• Other Study ID Numbers: CMC-12-0093-CTIL

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO