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Previous Volume 359:2254-2265 November 20, 2008 Number 21 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.

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The inheritance of two copies of a mutant β-globin gene, one from each parent, is the underlying cause of sickle cell disease. The mutation, GAGGTG, substitutes valine for glutamic acid at position 6 in the β-globin chain of hemoglobin A, resulting in a hemoglobin called hemoglobin S.^1,2,3 Sickle cell disease is one of the most common autosomal recessive disorders in the world. Approximately 8% of black Americans are heterozygous and have the sickle cell trait, whereas approximately 1 in 600 is homozygous and has sickle cell disease. In certain areas of sub-Saharan Africa, an estimated 40 to 60% of . . . [Full Text of this Article]

Phenotypes of Sickle Cell Disease

Vaso-Occlusion

The Acute Chest Syndrome

Causes of the Acute Chest Syndrome

            Pulmonary Infection

            Fat Emboli

            Pulmonary Infarction

Clinical Aspects of the Acute Chest Syndrome

Hemolysis, Endothelial-Cell Dysfunction, and Vasculopathy

Catabolism of Hemoglobin

Hemolysis

            Effect on Nitric Oxide

            Effect on Arginine

The Hypercoagulable State

Pulmonary Hypertension in Sickle Cell Disease

Echocardiography

Brain Natriuretic Peptide

Cardiac Catheterization

Other Mechanisms of Pulmonary Hypertension

Alternative Hypotheses

Conclusions

Source Information

From the Division of Pulmonary, Allergy, and Critical Care Medicine and the Hemostasis and Vascular Biology Research Institute, University of Pittsburgh, Pittsburgh, (M.T.G.); and Children's Hospital and Research Center at Oakland, Oakland, CA (E.V.).

Address reprint requests to Dr. Gladwin at the Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, NW 168 Montefiore Hospital, 354 Fifth Ave., Pittsburgh, PA, 15213, or at gladwinmt@upmc.edu.

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