Bone mineral density in patients with inherited bone marrow failure syndromes.

BackgroundPatients with inherited bone marrow failure syndromes (IBMFS) may have several risk factors for low bone mineral density (BMD). We aimed to evaluate the prevalence of low BMD in IBMFS and determine the associated risk factors.MethodsPatients with IBMFS with at least one dual-energy X-ray absorptiometry (DXA) scan were evaluated. Diagnosis of each IBMFS, Fanconi anemia (FA), dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome was confirmed by syndrome-specific tests. Data were gathered on age, height, and clinical history. DXA scans were completed at the lumbar spine, femoral neck, and forearm. BMD was adjusted for height (HAZ) in children (age ≤20 years). Low BMD was defined as a BMD Z-score and HAZ ≤-2 in adults and children, respectively, in addition to patients currently on bisphosphonate therapy.ResultsNine of thirty-five adults (26%) and eleven of forty children (27%) had low BMD. Adults with FA had significantly lower BMD Z-scores than those with other diagnoses; however, HAZ did not vary significantly in children by diagnosis. Risk factors included hypogonadism, iron overload, and glucocorticoid use.ConclusionsAdults and children with IBMFS have high prevalence of low BMD. Prompt recognition of risk factors and management are essential to optimize bone health

Achieving enhanced gain in photorefractive polymers by eliminating electron contributions using large bias fields

Photorefractive polymers have been extensively studied for over two decades and have found applications in holographic displays and optical image processing. The complexity of these materials arises from multiple charge contributions, for example, leading to the formation of competing photorefractive gratings. It has been recently shown that in a photorefractive polymer at relatively moderate applied electric fields the primary charge carriers (holes) establish an initial grating, followed by a subsequent competing grating (electrons) resulting in a decreased two-beam coupling and diffraction efficiencies. In this paper, it is shown that with relatively large sustainable bias fields, the two-beam coupling efficiency is enhanced owing to a decreased electron contribution. These results also explain the cause of dielectric breakdown experienced under large bias fields. Our conclusions are supported by self-pumped transient two-beam coupling and photocurrent measurements as a function of applied bias fields at different wavelengths

Ursinus College Alumni Journal, November 1966

\u2770 is here • And \u2770 says: Centennial Fund, roll on! • From the President • For Alumni Association leaders, Homecoming Day was some work and plenty of play • An Ursinus specialty: Shaping the future physician • New placement service for alumni • Huck Finn\u27s dilemma is ours • The paradox of urbia • The theatre of the absurd emerges in Latin America • An age of reforming in liberal education: Trends at Ursinus compare with reforms proposed at Columbia University • Sporting scene: Women undefeated in hockey; Soccer; Cross country; Football • Campus clippings: Founders\u27 Day; Sigma Xi Club; Research at Ursinus; Student teachers; The Agency; Mr. Rue remembers; On army duty; Evening school grows; Neighbors come to dinner; Lindback scholar; Parents Day; Job opportunity; \u2767 Ruby underway • Class notebook • Weddings • Births • In memoriamhttps://digitalcommons.ursinus.edu/alumnijournal/1085/thumbnail.jp

Multiplexed, High Density Electrophysiology with Nanofabricated Neural Probes

Extracellular electrode arrays can reveal the neuronal network correlates of behavior with single-cell, single-spike, and sub-millisecond resolution. However, implantable electrodes are inherently invasive, and efforts to scale up the number and density of recording sites must compromise on device size in order to connect the electrodes. Here, we report on silicon-based neural probes employing nanofabricated, high-density electrical leads. Furthermore, we address the challenge of reading out multichannel data with an application-specific integrated circuit (ASIC) performing signal amplification, band-pass filtering, and multiplexing functions. We demonstrate high spatial resolution extracellular measurements with a fully integrated, low noise 64-channel system weighing just 330 mg. The on-chip multiplexers make possible recordings with substantially fewer external wires than the number of input channels. By combining nanofabricated probes with ASICs we have implemented a system for performing large-scale, high-density electrophysiology in small, freely behaving animals that is both minimally invasive and highly scalable

Pi‐29

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/110026/1/cptclpt200664.pd

The Pseudomonas aeruginosa Efflux Pump MexGHI-OpmD Transports a Natural Phenazine that Controls Gene Expression and Biofilm Development

Redox-cycling compounds, including endogenously produced phenazine antibiotics, induce expression of the efflux pump MexGHI-OpmD in the opportunistic pathogen Pseudomonas aeruginosa Previous studies of P. aeruginosa virulence, physiology, and biofilm development have focused on the blue phenazine pyocyanin and the yellow phenazine-1-carboxylic acid (PCA). In P. aeruginosa phenazine biosynthesis, conversion of PCA to pyocyanin is presumed to proceed through the intermediate 5-methylphenazine-1-carboxylate (5-Me-PCA), a reactive compound that has eluded detection in most laboratory samples. Here, we apply electrochemical methods to directly detect 5-Me-PCA and find that it is transported by MexGHI-OpmD in P. aeruginosa strain PA14 planktonic and biofilm cells. We also show that 5-Me-PCA is sufficient to fully induce MexGHI-OpmD expression and that it is required for wild-type colony biofilm morphogenesis. These physiological effects are consistent with the high redox potential of 5-Me-PCA, which distinguishes it from other well-studied P. aeruginosa phenazines. Our observations highlight the importance of this compound, which was previously overlooked due to the challenges associated with its detection, in the context of P. aeruginosa gene expression and multicellular behavior. This study constitutes a unique demonstration of efflux-based self-resistance, controlled by a simple circuit, in a Gram-negative pathogen

Time Dynamics of Self-Pumped Reflection Gratings in a Photorefractive Polymer

The time dynamics of self-pumped reflection gratings in a commonly used photorefractive polymer PDCST:PVK:ECZ-BBP:C60 with no additional electron sources or traps is investigated. While holes are normally the mobile charges and responsible for grating formation, our experimental observations, analyzed using multi-exponential fitting curves, show evidence of electrons in addition to holes as charge carriers, particularly above an applied field of 40 V/μm. The dependence of effective carrier mobilities on the applied electric field, deduced from experimental results, show stronger field dependence of electron mobility at high electric fields. At an applied field of 70 V/μm, electron and hole mobilities become approximately equal, and the contribution of electrons on grating formation becomes significant

Maximal care considerations when treating patients with end-stage heart failure: ethical and procedural quandaries in management of the very sick

Deciding who should receive maximal technological treatment options and who should not represents an ethical, moral, psychological and medico-legal challenge for health care providers. Especially in patients with chronic heart failure, the ethical and medico-legal issues associated with providing maximal possible care or withholding the same are coming to the forefront. Procedures, such as cardiac transplantation, have strict criteria for adequate candidacy. These criteria for subsequent listing are based on clinical outcome data but also reflect the reality of organ shortage. Lack of compliance and non-adherence to lifestyle changes represent relative contraindications to heart transplant candidacy. Mechanical circulatory support therapy using ventricular assist devices is becoming a more prominent therapeutic option for patients with end-stage heart failure who are not candidates for transplantation, which also requires strict criteria to enable beneficial outcome for the patient. Physicians need to critically reflect that in many cases, the patient’s best interest might not always mean pursuing maximal technological options available. This article reflects on the multitude of critical issues that health care providers have to face while caring for patients with end-stage heart failure