New progress in pulmonary hypertension research

07.16.2013

Source: Doctor Tipster

July 16, 2013

Researchers at the University of Lincoln in colaboration with those from the University of Cambridge, King’s College London and Papworth Hospital, made new advances in the research regarding pulmonary hypertension. They were able to isolate the cells associated with the disease: blood outgrowth endothelial cells (BOEC).

Pulmonary hypertension is defined by a pressure greater than 25 mmHg in the pulmonary artery at rest. In pulmonary hypertension symptoms appear and worsen gradually so that in advanced stages can lead to severe heart failure and even death. There are several classifications and types of pulmonary hypertension: arterial, venous, familial, hypoxic, idiopathic etc. It should be noted that this disorder is associated with a number of diseases such as left heart disease, pulmonary embolism, chronic obstructive pulmonary disease, scleroderma and other collagen diseases etc.

Nobody knows what causes this disorder but it is known that in patients with pulmonary hypertension  vasoconstriction of the pulmonary arteries occurs. In addition, there is a proliferation of the endothelium of blood vessels that further narrows their caliber which lead to pulmonary hypertension. The symptoms are nonspecific, this is why the disease is difficult to diagnose at the beginning. Patients can have shortness of breath, heart pain, palpitations, rapid pulse, swelling in the legs, hemoptysis etc.

PAH

A team of researchers led by academic Dr Rajiv Machado conducted a study that investigated genetically identical twins who had both genetic marker for pulmonary hypertension. What is worth mentioning is that only one of them developed the disease that required a heart and lung transplant. The research team was able to investigate the origin of blood outgrowth endothelial cells (BOEC). BOEC, which are stem cells, are considered possible candidates for vascular regenerative therapy. However, there is not much known about these cells, specially about their origin. The study of twins led to the discovery of a marker showing that those BOEC (stem cells) were very unlikely to come from the heart or lungs.

Dr Machado said that even when circulating BOECs were grown in the new heart and lungs, they still expressed the mutation. This means that these cells have been produced elsewhere in the body. He added that the discovery of the origin of these cells is important because it may serve as a proxy for the fundamental sciences and to understand what is happening in our circulatory system. “If we can in one fell swoop remove two organs as being contributory then we are another step closer to knowing where these cells come from”, he said.

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