Scientists from Johns Hopkins Children's Centre are conducting a study to identify several biomarkers for idiopathic pulmonary hypertension (IPH).
IPH is a progressive and highly lethal condition in children and adults marked by persistently elevated pressure in the artery that carries blood from the heart to the lungs.
The researchers hope that the a measurable, reliable biomarker would help follow patient's response to medication and reveal who's benefitting and who's not Johns Hopkins Children's Centre cardiologist and lead researcher Allen Everett said it will help us predict how well the patient will do and tells us if we should tweak the dose for a better effect.
Currently, the most common way to track the course of IPH is an echocardiogram - an ultrasound image of the heart and the pulmonary artery - but the test is time-consuming, expensive and subject to misinterpretation.
Initial studies have revealed several promising candidate proteins as IHP biomarkers.
Preliminary findings show that these proteins circulate at much higher levels in the blood of children with IHP than in children without the disease.
The researchers are now trying to determine if the levels of these proteins drop when children respond to treatment, but remain elevated when they don't.
If they do, the next step to further validate the reliability of these biomarkers will be testing them in nearly 150 children and adults with IPH at Johns Hopkins and at the University of Colorado.
"In order to classify these proteins as bona fide biomarkers, we also want to make sure they reliably and predictably detect changes in medication response in a larger group of patients," said Everett.