Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHL(R200W) mutation (Chuvash polycythemia).

@article{Sable2012PulmonaryAP,
  title={Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHL(R200W) mutation (Chuvash polycythemia).},
  author={Craig Andrew Sable and Zakari Yusufu Aliyu and Niti Dham and Mehdi S Nouraie and Vandana Sachdev and Stanislav Sidenko and Galina Y. Miasnikova and Lydia A. Polyakova and Adelina I. Sergueeva and Daniel J. Okhotin and Vladimir Bushuev and Alan T Remaley and Xiaomei Niu and Oswaldo L. Castro and Mark T Gladwin and Gregory J. Kato and Josef T Prchal and Victor R Gordeuk},
  journal={Haematologica},
  year={2012},
  volume={97 2},
  pages={193-200}
}
BACKGROUND Patients with Chuvash polycythemia, (homozygosity for the R200W mutation in the von Hippel Lindau gene (VHL)), have elevated levels of hypoxia inducible factors HIF-1 and HIF-2, often become iron-deficient secondary to phlebotomy, and have elevated estimated pulmonary artery pressure by echocardiography. The objectives of this study were to provide a comprehensive echocardiographic assessment of cardiovascular physiology and to identify clinical, hematologic and cardiovascular risk… CONTINUE READING

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