Research article summary (published 29 Jun 2008):

Pulmonary arterial hypertension and left-sided heart disease in sickle cell disease: clinical characteristics and association with soluble adhesion molecule expression.

Full Abstract

Pulmonary hypertension (PH), a risk factor for mortality in sickle cell disease (SCD), has pathologic features of both pulmonary arterial hypertension (PAH) and PH related to left-sided heart disease (LHD) suggesting a link between these two entities. We hypothesized that both are characterized by endothelial dysfunction and increased adhesion molecule expression. SCD patients and normal volunteers underwent a screening questionnaire, echocardiogram, and blood donation for preparation of platelet-poor plasma. PAH was defined as a tricuspid regurgitant jet (TRJ) velocity > or =2.5 m/sec and/or the presence of isolated right ventricular hypertrophy or decreased systolic function. LHD was defined as either left-sided systolic/diastolic dysfunction or significant valvular disease. Plasma vascular cell adhesion molecule-1 (VCAM-1), intercellular adhesion molecule-1 (ICAM-1), P- and E-selectin, nitric oxide (NO(x)), erythropoietin, and vascular endothelial growth factor (VEGF) levels were assayed by enzyme-linked immunoassay. Forty-three percent of sickle cell anemia (HbSS) and 28% of hemoglobin SC disease (HbSC) disease patients had PAH. Additionally, 10-15% of SCD patients had LHD. VCAM-1 levels were significantly increased in HbSS patients compared with HbSC patients and normal volunteers. VCAM-1 and P-selectin levels correlated positively with TRJ velocity in HbSS patients (r = 0.45, P = 0.03, r = 0.2, P = 0.05, respectively). ICAM-1, E-selectin, NO(x), erythropoietin, and VEGF levels were similar across subject groups. PH is common in SCD and, at times, due to LHD. Increased VCAM-1 and P-selectin expression was associated with TRJ elevation regardless of etiology suggesting a similar effect on endothelial gene expression and possibly providing a pathologic link between PAH and PH related to LHD in SCD.

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Author information

Author/s: Klings, Elizabeth S (ES); Anton Bland, Demedrick (D); Rosenman, Dara (D); Princeton, Stephanie (S); Odhiambo, Adam (A); Li, Guihua (G); Bernard, Sheilah A (SA); Steinberg, Martin H (MH); Farber, Harrison W (HW);

Affiliation: The Pulmonary Center, Boston University School of Medicine, Boston, Massachusetts 02118, USA. klingon(-atsign-)bu.edu

Grants: 1U54 HL 0708819 (Agency:United States NHLBI) ; HL R01 68970 (Agency:United States NHLBI) ; K 23 HL079003-01 (Agency:United States NHLBI) ; M01 RR 00533 (Agency:United States NCRR)

Journal and publication information

Publication Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

Journal: American journal of hematology (Am J Hematol), published in United States. (Language: eng)

Reference: 2008-Jul; vol 83 (issue 7) : pp 547-53

Dates: Created 2008/06/30; Completed 2008/07/18;

PMID: 18383329, status: MEDLINE (last retrieval date: 11/6/2008)

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

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MeSH headings (categories)

This article was linked to the MESH Headings shown below.

Adult Anemia, Sickle Cell - complications; epidemiology; metabolism Female Heart Diseases - complications; epidemiology; metabolism Humans

Hypertension, Pulmonary - complications; epidemiology; metabolism Male P-Selectin - metabolism Solubility Vascular Cell Adhesion Molecule-1 - metabolism

Associated Chemicals: P-Selectin (0) ; Vascular Cell Adhesion Molecule-1 (0)

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