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Cellular, pharmacological, and biophysical evaluation of explanted lungs from a patient with sickle cell disease and severe pulmonary arterial hypertension.

Abstract：

:Pulmonary hypertension is recognized as a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). We now report benchtop phenotyping from the explanted lungs of the first successful lung transplant in SCD. Pulmonary artery smooth muscle cells (PASMCs) cultured from the explanted lungs were analyzed for proliferate capacity, superoxide (O2 (•-)) production, and changes in key pulmonary arterial hypertension (PAH)-associated molecules and compared with non-PAH PASMCs. Upregulation of several pathologic processes persisted in culture in SCD lung PASMCs in spite of cell passage. SCD lung PASMCs showed growth factor- and serum-independent proliferation, upregulation of matrix genes, and increased O2 (•-) production compared with control cells. Histologic analysis of SCD-associated PAH arteries demonstrated increased and ectopically located extracellular matrix deposition and degradation of elastin fibers. Biomechanical analysis of these vessels confirmed increased arterial stiffening and loss of elasticity. Functional analysis of distal fifth-order pulmonary arteries from these lungs demonstrated increased vasoconstriction to an α1-adrenergic receptor agonist and concurrent loss of both endothelial-dependent and endothelial-independent vasodilation compared with normal pulmonary arteries. This is the first study to evaluate the molecular, cellular, functional, and mechanical changes in end-stage SCD-associated PAH.

journal_name

Pulm Circ

journal_title

Pulmonary circulation

authors

Rogers NM,Yao M,Sembrat J,George MP,Knupp H,Ross M,Sharifi-Sanjani M,Milosevic J,St Croix C,Rajkumar R,Frid MG,Hunter KS,Mazzaro L,Novelli EM,Stenmark KR,Gladwin MT,Ahmad F,Champion HC,Isenberg JS

doi

10.1086/674754

subject

Has Abstract

pub_date

2013-12-01 00:00:00

pages

936-51

issue

4

eissn

2045-8932

issn

2045-8940

pii

PC2013012

journal_volume

3

pub_type

杂志文章

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