Medical and Non-medical Costs of Sickle Cell Disease and Treatments from a US Perspective: A Systematic Review and Landscape Analysis.

Background: Sickle cell disease (SCD) is a complex genetic disorder that manifests in infancy and progresses throughout life in the form of acute and chronic complications. As the upfront costs of potentially curative, genetic therapies will likely be high, an assessment and comprehensive characterization of the medical and non-medical cost burden will inform future decision making.

Objective: We sought to systematically summarize the existing literature surrounding SCD medical and non-medical costs.

Emergency procedures on the descending thoracic aorta in the endovascular era.

Background: Thoracic endovascular aortic repair (TEVAR), initially developed for the treatment of degenerative aneurysms of the descending thoracic aorta, has been applied to the entire spectrum of descending thoracic aortic pathology in both the elective and emergent settings. This single center study evaluates the effectiveness of TEVAR for the treatment of acute surgical emergencies involving the descending thoracic aorta, including traumatic aortic disruption (TAD), ruptured descending thoracic aneurysm (RDTA), and acute complicated Type B dissection (cTBD).

Methods: A retrospective review of the medical records of all patients undergoing emergent TEVAR at the University of Mississippi Medical Center between August 2007 and November 2010 was undertaken.

Is sexual selection driving diversification of the bioluminescent ponyfishes (Teleostei: Leiognathidae)?

Sexual selection may facilitate genetic isolation among populations and result in increased rates of diversification. As a mechanism driving diversification, sexual selection has been invoked and upheld in numerous empirical studies across disparate taxa, including birds, plants and spiders. In this study, we investigate the potential impact of sexual selection on the tempo and mode of ponyfish evolution.

Double-barrel technique for endovascular exclusion of an aortic arch aneurysm without sternotomy.

Purpose: To report the use of commercially available stents and a stent-graft in coaxial orientation to extend the proximal limits of endografting within the aortic arch.

Case Report: A 70-year-old man was found to have an asymptomatic 7-cm saccular aortic arch aneurysm, extending distally from the origin of the left carotid artery and involving the left subclavian artery; there was only 11 mm between the innominate artery orifice and the aneurysm. The patient was deemed to be high risk for open surgical repair due to a history of 2 prior sternotomies and the requirement for hypothermic circulatory arrest.

Contemporary standards for the diagnosis and treatment of heparin-induced thrombocytopenia (HIT).

Background: Heparin binding to platelet factor 4 (PF4) generates a new antigenic epitope. In an unpredictable fashion, as many as approximately 17% of patients treated with unfractionated heparin (UFH) and approximately 8% treated with low-molecular-weight heparin (LMWH) subsequently develop the anti-heparin-PF4 antibodies that mediate heparin-induced thrombocytopenia and thrombosis (HIT). Very few of those patients with circulating anti-heparin-PF4 antibodies, however, progress to develop clinical HIT (referred to previously as Type II HIT).

Slower onset of low shear stress leads to less neointimal thickening in experimental vein grafts.

Vein grafts respond to low flow and shear stress (tau(w)) by generating thicker walls and smaller lumens through the processes of neointimal hyperplasia and remodeling. Clinically, however, vein grafts with obviously low tau(w), such as those distal to high-grade proximal obstructions, are not infrequently found to be widely patent and pliable. One possible explanation for this phenomenon may be that vein grafts remodel more favorably in response to changes in shear that occur gradually over time compared to abruptly.

Endovascular solutions to complications of open aortic repair.

Open repair of abdominal aortic aneurysms (AAAs) or occlusive disease can be complicated by pseudoaneurysm formation and aneurysmal dilatation of native vessels. Reports of reoperation for these new lesions have a mortality rate of 5-17% electively, and 24-88% if ruptured. These complications are commonly several years after initial repair, and progression of other comorbidities can further complicate a repeat exploration.

Ureteroarterial fistula with ruptured anastomotic pseudoaneurysm: successful management with vascular exclusion, extra-anatomic bypass and nephrectomy--a case report.

Ureteroarterial fistula is a rare but life-threatening condition most often arising as a consequence of combined vascular and urologic pathology. Only about 70 cases are reported in the English literature. Principles of repair include complete vascular isolation, extra-anatomic bypass, and urinary stream diversion away from major vascular conduits.

Gene therapy for the extension of vein graft patency: a review.

The mainstay of treatment for long-segment small-vessel chronic occlusive disease not amenable to endovascular intervention remains surgical bypass grafting using autologous vein. The procedure is largely successful and the immediate operative results almost always favorable. However, the lifespan of a given vein graft is highly variable, and less than 50% will remain primarily patent after 5 years.

Modulation of vascular remodeling induced by a brief intraluminal exposure to the recombinant R7020 strain of Herpes simplex-1.

Objective: Vascular remodeling in response to injury or low shear stress (or both) is characterized by neointimal hyperplasia and luminal contraction. When profound, the response leads to restenosis after percutaneous endovascular intervention as well as to de novo stenosis in vein grafts. It has recently been reported that exposure of vein patches to neurovirulence-attenuated Herpes simplex virus-1 (HSV-1) decreases neointimal hyperplasia and increases luminal area.

Limb salvage after infrainguinal bypass graft failure.

Objective: The purpose of this study was to examine the outcome of patients in whom an infrainguinal bypass graft failed.

Methods: This was a retrospective analysis of consecutive patients undergoing infrainguinal bypass grafting in a single institution over 8 years.

Results: Six hundred thirty-one infrainguinal bypass grafts were placed in 578 limbs in 503 patients during the study period.

Catheter-directed thrombolysis for deep venous thrombosis.

Venous thromboembolism (VTE) represents a significant clinical problem, affecting patients of all age groups, nationalities, and socioeconomic strata. Despite its prevalence, the paradigms for care are largely centered around primary or secondary prophylaxis, with less emphasis on actual treatment of the thrombus. With the recent rapid development of advanced endovascular techniques, it is now feasible to dissolve many thrombi using catheter-directed thrombolysis (CDT), and favorable clinical experience has been reported in over 600 patients.

The changing face of abdominal aortic aneurysm management.

In summary, endovascular therapy represents a new and exiting paradigm in the treatment of abdominal aortic aneurysms. Its detractors have now largely been silenced, and a working knowledge of the devices and techniques is essential for all surgeons who care for patients with aneurysms. The first 25,000 stent-graft procedures have been attended by significant risks of implantation and endoleak, but the patients' acceptance of the technique has been heard loud and clear.

Theoretical hydraulic consequences of vein graft taper.

Objective: Internal diameter is a strong predictor of patency of infrainguinal vein grafts. However, most vein grafts are tapered, with variable diameter along their length. It is unknown which diameter is most important in determining graft resistive properties, that is, its mean diameter, minimum diameter, or some geometric combination thereof.

Sustained inhibition of experimental neointimal hyperplasia with a genetically modified herpes simplex virus.

Objective: Reported herein is a potential strategy for sustained smooth muscle cell (SMC) inhibition with a virulence-attenuated herpes simplex virus (HSV). Experiments were conducted in vitro to demonstrate selective SMC cytotoxicity and in vivo to demonstrate reduced neointimal hyperplasia (NIH) in a clinically relevant animal model.

Methods: In vitro: Cultured human umbilical artery smooth muscle cells (UASMC) and venous endothelial cells (HUVEC) were exposed to varying multiplicities of infection (MOI) of a gamma(1)34.

Ergotism associated with HIV antiviral protease inhibitor therapy.

Ergotism is a rare condition of acute vasospasm found classically in young and middle-aged women taking ergot alkaloid agents to treat migraine headache. We report the case of a young man with human immunodeficiency virus (HIV) positivity and describe the drug interaction between protease inhibitors and ergot alkaloid agents, which most likely predisposed to development of ergot toxicity. The HIV-positive population receiving antiviral therapy may be an under-recognized group at risk for ergotism through decreased hepatic metabolism of ergot preparations.

Long-term outcome of infrainguinal bypass grafting in patients with serologically proven hypercoagulability.

Objective: The purpose of this study was to test the hypothesis that the long-term outcome of infrainguinal bypass grafting in patients with congenital or acquired hypercoagulability is inferior to the results in patients without documented clotting disorders.

Methods: The study was a retrospective analysis of consecutive patients from January 1994 to January 2001.

Results: Five hundred eighty-two infrainguinal bypass grafts were created in 456 patients.

Differential mechanical activation of mitogen-activated protein kinases in intact human blood vessels.

Background: Vascular tissue remodels in response to a variety of hemodynamic factors, often transduced through activation of mitogen-activated protein kinases such as extracellular signal-related kinase (ERK1/2) and c-jun N-terminal kinase (JNK). This study tests the hypothesis that these kinases are involved in mechanical signal transduction in intact human arteries and veins.

Methods: Unused portions of human saphenous vein and radial artery were obtained fresh at the time of peripheral or coronary bypass.