The function of CX3CR1 receptors on circulating patrolling monocytes and other leukocytes determine the severity of emphysema in patients with ZZ genotype alpha-1-antitrypsin deficiency

The function of CX3CR1 receptors on circulating patrolling monocytes and other leukocytes determine the severity of emphysema in patients with ZZ genotype alpha-1-antitrypsin deficiency - CX3CR1 functionality in patients with alpha-1-antitrypsin deficiency

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON49069

Enrollment

Registered

2020-07-23

Start date

2020-09-07

Completion date

Unknown

Last updated

2024-04-15

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

hereditary deficiency of the protein alpha-1-antitrypsin stretched lungs

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: In order to be eligible for PBMC sampling in this study, a subject must meet all of the following criteria: * Known ZZ-AATD genotype of study patient. * Age between 30 and 75. * Spouses with normal AAT genotype, as determined by a PCR kit: AlphaKit-Quickscreen (see section 6.3, study procedures in study protocol). * Spouses must have normal gas transfer values of the lungs.

Exclusion criteria

Exclusion criteria: A potential subject who meets any of the following criteria will be excluded from participation in this study: * ZZ-AATD patient or spouse are current smoker. * Patients or spouses who had any type of infection in the previous 3 months. * Patients or spouses who are treated for any type of cancer. * Any co-morbidity that in the opinion of the investigator may interfere with the interpretation of the primary outcome parameter of the study.

Design outcomes

Primary

Measure	Time frame
To identify the variability in the circulation of the number of patrolling monocytes and their expression of CX3CR1 obtained from adult ZZ-AATD patients compared to spouse controls.	—

Secondary

Measure	Time frame
1) To identify if low expression of CX3CR1 on PBMC in blood is associated with the severity of emphysema in subjects with ZZ-AATD. (2) To identify if expression of chemokine receptors other than CX3CR1 on PBMC in blood is associated with the severity of emphysema associated with the severity of emphysema in subjects with ZZ-AATD. (3) to identify if damage of pulmonary microvascular endothelial cells (pMVECs) reflected by the level of plasma E-selectin EMP ( Endothelial Micro Particles, which are von-Willebrand negative) is correlated with any of the results identified in secondary objective nr 2.	—

Measure

Time frame

1) To identify if low expression of CX3CR1 on PBMC in blood is associated with the severity of emphysema in subjects with ZZ-AATD. (2) To identify if expression of chemokine receptors other than CX3CR1 on PBMC in blood is associated with the severity of emphysema associated with the severity of emphysema in subjects with ZZ-AATD. (3) to identify if damage of pulmonary microvascular endothelial cells (pMVECs) reflected by the level of plasma E-selectin EMP ( Endothelial Micro Particles, which are von-Willebrand negative) is correlated with any of the results identified in secondary objective nr 2.

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Countries

Netherlands

Outcome results