high blood pressure in the lungs Pulmonary Hypertension Pulmonary arterial hypertension
Conditions
Interventions
Sponsors
Universitair Medisch Centrum Groningen
Eligibility
Age
2 Years to 17 Years
Inclusion criteria
Inclusion criteria: PAH group: 1. PAH diagnosis, confirmed by right heart catheterization 2. aged 2 to 18 years 3. informed consent depending on the age, by the child and/or the legal guardian/parent. Control group: 1. No diagnosis of PAH, (signs of) heart failure, conditions of pulmonary vascular disease or conditions associated with pulmonary vascular remodeling, cardiac arrythmia, cardiomyopathy, congenital heart disease, infection, inflammation or immunological comorbidities. 2. aged 2 to 18 years 3. informed consent depending on the age and competency, by the child and/or the legal guardian/parent.
Exclusion criteria
Exclusion criteria: A potential subject either from the control or the PAH group who meets any of the following criteria will be excluded from participation in this study: 1. a medical history of immunologic or chronic inflammatory conditions 2. signs/symptoms of an active infection within 1 week prior to blood withdrawal, indicated by anamnesis and/or CRP >10 mg/L or monocytes >10% of white blood cell count.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The primary objective is to determine whether TET2 mRNA expression in peripheral blood mononuclear cells (PBMCs) is reduced and serum levels of IL-1β and IL-18 are elevated in pediatric PAH patients when compared to age- and sex-matched controls. | — |
Secondary
| Measure | Time frame |
|---|---|
| - Investigate whether TET2 expression in PBMCs associates with disease severity markers used in the clinic during standard follow-up in pediatric PAH patients. Currently, the clinical markers used during standard follow-up include: - Serum N-terminal prohormone of brain natriuretic peptide (NT-pro-BNP), a marker for right ventricular dilatation - WHO functional class, a 4-point scale where all patients are assigned a severity class by the physician - Tricuspid Annular Plane Systolic Excursion (TAPSE), which is measured by echocardiography and is considered the gold standard for assessment of right ventricular function - Investigate whether the serum level of IL-1β associates with NT-pro-BNP, WHO functional class, and TAPSE in patients with pediatric PAH. - Investigate whether the serum level of IL-18 associates with NT-pro-BNP, WHO functional class, and TAPSE in patients with pediatric PAH. - Investigate whether serum IL-1β and IL-18 levels inversely correlate with the TET2 expression in PBMCs in patients with pediatric PAH. | — |
Countries
Netherlands
Contacts
Public ContactR Weldeghebreal
Universitair Medisch Centrum Groningen
Outcome results
None listed