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Primary pulmonary hypertension: the pressure rises for a gene
  1. J R Thomson1,
  2. R C Trembath1
  1. 1Division of Medical Genetics, Adrian Building, University of Leicester, Leicester LE1 7RH, UK
  1. Dr Thomson jrt10{at}leicester.ac.uk

Abstract

Primary pulmonary hypertension (PPH) represents the end stage of a disruption of pulmonary vascular integrity, of unknown cause. Although PPH is associated with several systemic disorders, there have hitherto been few clues as to the aetiological factors responsible for the pathogenesis of this condition. As an example of the application of modern molecular genetics and positional cloning, this leader desribes the range of studies currently under way, which aim to find the gene that underlies PPH, and summarises the implications of the identification of such a gene.

  • pulmonary hypertension
  • genetics
  • chromosome 2q33

https://doi.org/10.1136/jcp.53.12.899

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    Footnotes

    • ↵* The International PPH Consortium: RC Trembath, Division of Medical Genetics, University of Leicester, Leicester LE1 7RH, UK; WC Nichols, Division of Human Genetics, Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati OH 45229, USA; T Faroud, Department of Medical and Molecular Genetics, Indiana University School of Medicine, 975 W. Walnut Street IB-155, Indianapolis, IN 46202–5251, USA; JE Loyd, JA Phillips III, JH Newman, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA.