FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 345:367-371 August 2, 2001 Number 5 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text PDF Purchase this article Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited Related Article by Newman, J. H. Related Article by Trembath, R. C. PubMed Citation

Primary pulmonary hypertension is a devastating disease that is invariably fatal without definitive therapy. The disorder is more appropriately called a syndrome, since it is characterized by a set of clinical and pathophysiological features common to a variety of underlying causes. The proposed causes of primary pulmonary hypertension range widely, from environmental (e.g., hypoxia) to autoimmune (e.g., systemic lupus erythematosus) to drug related (e.g., dexfenfluramine use). Although genetic determinants no doubt indirectly influence the propensity for primary pulmonary hypertension to develop in response to these factors, a heritable form of primary pulmonary hypertension, familial primary pulmonary hypertension, has also been . . . [Full Text of this Article]

References

This article has been cited by other articles:

• Raj, J. U., Aliferis, C., Caprioli, R. M., Cowley, A. W. Jr., Davies, P. F., Duncan, M. W., Erle, D. J., Erzurum, S. C., Finn, P. W., Ischiropoulos, H., Kaminski, N., Kleeberger, S. R., Leikauf, G. D., Loyd, J. E., Martin, T. R., Matalon, S., Moore, J. H., Quackenbush, J., Sabo-Attwood, T., Shapiro, S. D., Schnitzer, J. E., Schwartz, D. A., Schwiebert, L. M., Sheppard, D., Ware, L. B., Weiss, S. T., Whitsett, J. A., Wurfel, M. M., Matthay, M. A. (2007). Genomics and proteomics of lung disease: conference summary. Am. J. Physiol. Lung Cell. Mol. Physiol. 293: L45-L51 [Full Text]  
• Beghe, B., Bazzan, E., Baraldo, S., Calabrese, F., Rea, F., Loy, M., Maestrelli, P., Zuin, R., Fabbri, L. M., Saetta, M. (2006). Transforming growth factor-{beta} type II receptor in pulmonary arteries of patients with very severe COPD. Eur Respir J 28: 556-562 [Abstract] [Full Text]  
• Callebert, J., Esteve, J. M., Herve, P., Peoc'h, K., Tournois, C., Drouet, L., Launay, J. M., Maroteaux, L. (2006). Evidence for a Control of Plasma Serotonin Levels by 5-Hydroxytryptamine2B Receptors in Mice. J. Pharmacol. Exp. Ther. 317: 724-731 [Abstract] [Full Text]  
• Yu, P. B., Beppu, H., Kawai, N., Li, E., Bloch, K. D. (2005). Bone Morphogenetic Protein (BMP) Type II Receptor Deletion Reveals BMP Ligand-specific Gain of Signaling in Pulmonary Artery Smooth Muscle Cells. J. Biol. Chem. 280: 24443-24450 [Abstract] [Full Text]  
• Satoh, T., Kimura, K., Okano, Y., Hirakata, M., Kawakami, Y., Kuwana, M. (2005). Lack of circulating autoantibodies to bone morphogenetic protein receptor-II or activin receptor-like kinase 1 in mixed connective tissue disease patients with pulmonary arterial hypertension. Rheumatology (Oxford) 44: 192-196 [Abstract] [Full Text]  
• Richter, A., Yeager, M. E., Zaiman, A., Cool, C. D., Voelkel, N. F., Tuder, R. M. (2004). Impaired Transforming Growth Factor-{beta} Signaling in Idiopathic Pulmonary Arterial Hypertension. Am. J. Respir. Crit. Care Med. 170: 1340-1348 [Abstract] [Full Text]  
• Farber, H. W., Loscalzo, J. (2004). Pulmonary Arterial Hypertension. NEJM 351: 1655-1665 [Full Text]  
• Napoli, C., Loscalzo, J. (2004). Nitric Oxide and Other Novel Therapies for Pulmonary Hypertension. J CARDIOVASC PHARMACOL THER 9: 1-8 [Abstract]  
• Ahn, J., Glaser, D., Puzas, J. E. (2003). Gene Therapy and the Future of Orthopaedics. JBJS 85: 965-966 [Full Text]  
• van den Driesche, S., Mummery, C. L., Westermann, C. J.J. (2003). Hereditary hemorrhagic telangiectasia: an update on transforming growth factor {beta} signaling in vasculogenesis and angiogenesis. Cardiovasc Res 58: 20-31 [Abstract] [Full Text]  
• Chang, H., Brown, C. W., Matzuk, M. M. (2002). Genetic Analysis of the Mammalian Transforming Growth Factor-{beta} Superfamily. Endocr. Rev. 23: 787-823 [Abstract] [Full Text]  
• Eddahibi, S., Morrell, N., d'Ortho, M-P., Naeije, R., Adnot, S. (2002). Pathobiology of pulmonary arterial hypertension. Eur Respir J 20: 1559-1572 [Abstract] [Full Text]  
• Chatterjee, K., De Marco, T., Alpert, J. S. (2002). Pulmonary Hypertension: Hemodynamic Diagnosis and Management. Arch Intern Med 162: 1925-1933 [Abstract] [Full Text]  
• Humbert, M., Trembath, R.C. (2002). Genetics of pulmonary hypertension: from bench to bedside. Eur Respir J 20: 741-749 [Abstract] [Full Text]  
• Harris, S.E., Chand, A.L., Winship, I.M., Gersak, K., Aittomaki, K., Shelling, A.N. (2002). Identification of novel mutations in FOXL2 associated with premature ovarian failure. Mol Hum Reprod 8: 729-733 [Abstract] [Full Text]  
• Hoeper, M. M., Galie, N., Simonneau, G., Rubin, L. J. (2002). New Treatments for Pulmonary Arterial Hypertension. Am. J. Respir. Crit. Care Med. 165: 1209-1216 [Full Text]