This Article  • Full text  • PDF  • Video  • CME Course for This Article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (7)  • Contact me when this article is cited  Related Content  • Related letters  • Similar articles in JAMA  Topic Collections  • HIV/AIDS  • Pulmonary Diseases, Other  • Genetics  • Genetic Disorders  • Hematology/ Hematologic Malignancies  • Anemias  • Hypertension  • Infectious Diseases  • Alert me on articles by topic  Social Bookmarking   What's this?

An Increasingly Recognized Complication of Hereditary Hemolytic Anemias and HIV Infection

Christopher F. Barnett, MD, MPH; Priscilla Y. Hsue, MD; Roberto F. Machado, MD

JAMA. 2008;299(3):324-331.

Modern health care has greatly increased longevity for patients with congenital hemolytic anemias (such as sickle cell disease and thalassemia) and human immunodeficiency virus (HIV) infection. It is estimated that 10% of patients with hemoglobinopathies and 0.5% of patients with HIV infection develop moderate to severe pulmonary hypertension. Pulmonary hypertension is a relentlessly progressive disease leading to right heart failure and death. Worldwide, there are an estimated 30 million patients with sickle cell disease or thalassemia and 40 million patients with HIV disease. Considering the prevalence of pulmonary vascular disease in these populations, sickle cell disease and HIV disease may be the most common causes of pulmonary hypertension worldwide. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for these diseases are summarized. Because therapy is likely to reduce morbidity and prolong survival, efforts to screen, diagnose, and treat these patients represent a global health opportunity.

Author Affiliations: Critical Care Medicine Department, Clinical Center, and Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland (Drs Barnett and Machado); and Division of Cardiology, University of California, San Francisco (Dr Hsue).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED LETTERS

Normal Range for Pulmonary Artery Systolic Pressure
Tomaso Gnecchi-Ruscone and Fausto Rigo
JAMA. 2008;299(17):2022.
EXTRACT | FULL TEXT  

Normal Range for Pulmonary Artery Systolic Pressure—Reply
Roberto F. Machado, Priscilla Y. Hsue, and Christopher F. Barnett
JAMA. 2008;299(17):2022-2023.
EXTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Magnetic resonance assessment of pulmonary artery compliance a promising diagnostic and prognostic tool in pulmonary hypertension?
Ben-Yehuda and Barnett
J Am Coll Cardiol Img 2009;2:296-298.
FULL TEXT  

The Study to Understand the Natural History of HIV and AIDS in the Era of Effective Therapy (SUN Study)
Vellozzi et al.
Am J Epidemiol 2009;169:642-652.
ABSTRACT | FULL TEXT  

Pulmonary Complications of HIV Infection
Rosen
ACCP Pulmonary Med Brd Rev 2009;25:219-232.
FULL TEXT  

Caring for AIDS Patients in the ICU: Expanding Horizons
Masur
Chest 2009;135:1-2.
FULL TEXT  

Long-term effects of bosentan in patients with HIV-associated pulmonary arterial hypertension
Degano et al.
Eur Respir J 2009;33:92-98.
ABSTRACT | FULL TEXT  

Clinical Year in Review IV: Acute Respiratory Distress Syndrome, Radiology in the Intensive Care Unit, Nonpulmonary Critical Care, and Pulmonary Infections in the Immunocompromised Host
Ware
Proc Am Thorac Soc 2008;5:755-760.
FULL TEXT  

Normal Range for Pulmonary Artery Systolic Pressure
Gnecchi-Ruscone and Rigo
JAMA 2008;299:2022-2022.
FULL TEXT