Metastasising chordoma to the mandible from a rare vertebral site: the first reported case.

Chordoma is a rare tumor, arising from notochord remnants, which usually occurs in the axial skeleton and rarely metastasizes. Although there have been 3 previous reports of metastatic disease to the facial bones from sacrococcygeal chordoma, this is the first to describe spread to the mandible from a vertebral primary chordoma.     

MR findings in iatrogenic Gerbode defect

Congenital or acquired communication between left ventricle and right atrium is rare and can be easily overlooked as an eccentric tricuspid regurgitation jet. MRI is the ideal modality for accurate diagnosis, providing details for surgical planning about the location and size of the defect.     

Barriers to Effective Drug Treatment for Brain Metastases: A Multifactorial Problem in the Delivery of Precision Medicine

The treatment of metastatic lesions in the brain represents a serious unmet medical need in the field of neuro-oncology. Even though many effective compounds have demonstrated success in treating peripheral (non-CNS) tumors with targeted agents, one aspect of this lack of success in the brain may be related to poor delivery of otherwise effective compounds. Many factors can influence the brain delivery of these agents, but one key barrier is a heterogeneously “leaky” BBB that expresses efflux transporters that limit the BBB permeability for many targeted agents. Future success in therapeutics for brain metastases must take into account the adequate delivery of “active, free drug” to the target, and may include combinations of targeted drugs that are appropriate to address each individual patient’s tumor type. This review discusses some issues that are pertinent to precision medicine for brain metastases, using specific examples of tumor types that have a high incidence of brain metastases.     

Prevalence of Moderate or Severe Left Ventricular Diastolic Dysfunction in Persons With Suspected Myocardial Ischemia With and Without an Abnormal Adenosine or Exercise Sestamibi Stress Test or Coronary Revascularization

We investigated in 335 patients (mean age-63 year) with suspected myocardial ischemia the prevalence of moderate or severe left ventricular diastolic dysfunction (LVDD) in patients with an abnormal adenosine or exercise sestamibi stress test (SST) or prior coronary revascularization and in patients with a normal SST and no prior coronary revascularization. Moderate or severe LVDD was present in 117 of 142 patients (82%) with an abnormal SST or prior coronary revascularization and in 111 of 193 patients (58%) with a normal SST and no prior coronary revascularization (p < 0.001). Moderate or severe LVDD was present in 34 of 38 patients (89%) with an abnormal SST or prior coronary revascularization and an abnormal left ventricular ejection fraction (LVEF) and in 4 of 8 patients (50%) with a normal SST and no prior coronary revascularization and an abnormal LVEF (p < 0.01).     

Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.     

Characterization of Extracellular Signal-Regulated Kinase 5 Levels in Human Umbilical Vein Endothelial Cells Exposed to Disturbed and Uniform Flow

Extracellular signal-regulated kinase 5 (ERK5) has been reported to regulate endothelial cell integrity and protect from vascular dysfunction under continuous laminar flow. However, the effect of flow on ERK5 levels has not been determined. Confluent human umbilical vein endothelial cells (HUVECs) were seeded on fibronectin coated glass slides and serum starved for 2 hours with 1% fetal bovine serum (FBS). HUVECs were then exposed to to and fro flow (TFF), pulsatile forward flow (PFF), or continuous laminar flow (CLF) in a parallel plate flow chamber for up to 2 hours. At the end of experiment, cell lysates were prepared and immunoblotted with antibodies to total ERK5. Both CLF and TFF exhibited a decrease in ERK5 after levels after 2-hour exposure. However, the level of ERK5 for PFF remained the same. Disturbed, but not uniform pulsatile, flow decreases ERK5 levels in HUVECs.     

Carotid intima-media thickness among human immunodeficiency virus-infected patients without coronary calcium

Subjects infected with human immunodeficiency virus (HIV) have increased risk for atherosclerosis. Carotid artery intima-media thickness (IMT) assessed using ultrasound and coronary artery calcium (CAC) detected using computed tomography predict cardiovascular risk in the general population; however, their usefulness and comparability in patients with HIV are less well defined. The purpose of this study was to compare IMT and CAC in the detection of atherosclerosis in subjects with HIV. CAC and IMT were measured in 253 HIV-infected and 58 uninfected adults. Associations among HIV-related factors, traditional risk factors, and CAC and IMT were evaluated. The distribution of IMT among subjects with and without CAC was compared. Among the patients with HIV, 37% had detectable CAC compared to 28% of controls (p = 0.19); 16% of the patients with HIV had CAC >100 compared to 5% of controls (p = 0.03). With either detectable or undetectable CAC, HIV-infected subjects had higher IMT compared to controls (1.02 ± 0.34 vs 0.78 ± 0.12 mm, p <0.0001), even after adjustment for traditional risk factors. Among those with undetectable CAC, 34% of patients with HIV had markedly increased IMT (≥1 mm) compared to no controls (p <0.0001). HIV-related factors were associated with IMT but not with CAC. In conclusion, patients with HIV and controls had similar rates of detectable CAC, while absolute CAC scores were modestly higher in the HIV group. Conversely, carotid IMT detected advanced subclinical atherosclerosis in patients with HIV even in the absence of CAC. Thus, with HIV, IMT is associated with disease-related factors and may be a more sensitive indicator of subclinical atherosclerosis than CAC.