Endothelin-1, vascular endothelial growth factor and systolic pulmonary artery pressure in patients with Chuvash polycythemia

Chuvash Republic Cardiac Center, Cheboksary, Chuvashia, Russia. BACKGROUND AND OBJECTIVES: Endothelin-1 has been associated with development of hypoxia-related pulmonary hypertension and vascular endothelial growth factor (VEGF) with protection from this complication. In Chuvash polycythemia, homozy...

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Bibliographic Details
Published in:Haematologica (Roma) 2006-06, Vol.91 (6), p.744-749
Main Authors: Bushuev, VI, Miasnikova, GY, Sergueeva, AI, Polyakova, LA, Okhotin, D, Gaskin, PR, Debebe, Z, Nekhai, S, Castro, OL, Prchal, JT, Gordeuk, VR
Format: Article
Language:eng ; rus
Subjects:
Altitude
Electrocardiography
Endothelin-1 - blood
Endothelin-1 - genetics
Humans
Hypertension, Pulmonary - complications
Hypoxia - etiology
Mutation
Polycythemia - blood
Polycythemia - genetics
Polycythemia - physiopathology
Polymorphism, Single Nucleotide
Pulmonary Artery - physiopathology
Reference Values
Russia
Systole
Vascular Endothelial Growth Factor A - blood
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Summary:Chuvash Republic Cardiac Center, Cheboksary, Chuvashia, Russia. BACKGROUND AND OBJECTIVES: Endothelin-1 has been associated with development of hypoxia-related pulmonary hypertension and vascular endothelial growth factor (VEGF) with protection from this complication. In Chuvash polycythemia, homozygous germline von Hippel-Lindau (VHL) 598C->T leads to up-regulation during normoxia of hypoxia inducible factor-1a and several hypoxia-controlled genes including erythropoietin and VEGF. We postulated that endothelin-1 and pulmonary artery pressure may be elevated in Chuvash polycythemia. DESIGN AND METHODS: Systolic pulmonary artery blood pressure was estimated by Doppler echocardiography and plasma concentrations of endothelin-1, VEGF and erythropoietin were determined in 14 patients with Chuvash polycythemia and 14 controls. Results. Plasma endothelin-1 (p=0.010), VEGF (p=0.022) and erythropoietin (p
ISSN:0390-6078
1592-8721