cssi/SciSciGPT-SciSciNet huggingface dataset:
Invasive phaeochromocytoma presenting as a right atrial mass.

A 56-year-old man with a history of hypertension and panic attacks for 30 years presented with increasing palpitations, especially after strenuous activity, that had already lasted for 1 year. Initial assessment with an echocardiogram showed a right atrial mass. Subsequent imaging revealed a right adrenal mass extending into the renal vein, inferior vena cava, and right atrium (fi gure). We identifi ed no evidence of distant metastatic disease by 18F-fl uorodeoxyglucose PET-CT imaging. Through biochemical analysis, we confi rmed the diagnosis of phaeochromocytoma, with plasma-free normetanephrine at a concentration of 13 936 pg/mL (normal range 0–145 pg/mL) and metanephrines at a concentration of 129 pg/mL (normal range is 0–62 pg/mL). After treatment with α-blockers, a right atriotomy and venotomy of the inferior vena cava with en-bloc resection of the primary tumour, including intracaval and intraatrial segments, was done. Pathology analysis confi rmed phaeo chromocytoma (TNM stage pT3 pNX pMX) extending into the inferior vena cava and right atrium, and invading the adrenal capsule and periadrenal fat with margins free of tumour. A Pheochromocytoma of the Adrenal Gland Scaled Score of 11 suggested malignancy. Early surgical cure was confi rmed by normal biochemical testing 8 months after surgery. In view of low clinical suspicion of multiple endocrine neoplasia type 2 or neurofi bromatosis type 1, we initiated genetic testing sequentially for Von Hippel–Lindau disease, the result of which was negative. Subsequently, mutational analysis of the succinate dehydrogenase gene was planned, but the patient was lost to follow-up. This case shows a haematogenously invasive phaeo chromocytoma without evidence of distant metastases to confi rm malignancy. Although phaeochromocytomas with cavo–atrial extension are rarely described in the medical literature, the disorder is commonly seen in patients with renal cell carcinoma. About 10% of phaeochromocytomas are malignant, and malignancy is most common in patients with SDHB mutations. Surgery is seldom curative. Vascular invasion, mitotic activity, and cellular atypia might suggest aggressive tumour behaviour, but cannot defi nitively predict metastatic potential. Metastases are typically used to diagnose malignancy, but no molecular or histological markers exist to confi rm a malignant phaeochromocytoma. After 30 years of symptoms related to the phaeo chromocytoma, this patient had no metastases, despite surgical pathology fi ndings suggestive of malignancy. Early surgical remission is promising, although the patient will need lifelong monitoring for recurrence.
Coactivator ACTR/AIB1, an important cell cycle regulator, transforms human breast epithelial cells independent of estrogen receptors [Abstract #5399]

Proc Amer Assoc Cancer Res, Volume 46, 2005 5399 The transcriptional co-factor ACTR/AIB1 was initially identified as a coactivator for nuclear hormone receptors including estrogen receptors (ERs) and androgen receptor (AR). It is linked to cancers because of its gene amplification and elevated protein levels frequently detected in multiple types of human malignancy, including breast cancer, prostate cancer, and pancreatic, gastric and hepatocellular carcinomas. Our previous study has unexpectedly found that overexpression of ACTR promotes breast cancer cell proliferation and invokes anti-estrogen resistance independent ER but through its association with E2F proteins, a group of transcription factors playing key roles in control of the cell cycle gene expression. In this report, we found that ACTR is required for normal human cells to enter S phase, and that depletion of ACTR strongly inhibits the proliferation of human diploid fibroblast cells. Moreover, consistent with its critical role in cell cycle control, we found that ACTR gene transcription appears to be cell cycle regulated, which involves members of the E2F family. Interestingly, ACTR is recruited to its own promoter at G1-S transition, suggesting a positive feedback mechanism for ACTR action in control of cell cycle progression. Importantly, we found that ACTR functions to integrate cell proliferation signals with DNA replication by coordinating the expression of a specific subset of E2F target genes involved in DNA replication/licensing of DNA replication. More importantly, we found that elevated ACTR can transform non-malignant human breast epithelial cells, which are devoid of ER. Together, these findings points to the novel role of coactivators in control of cell cycle, and strongly support the notion that the ability of ACTR to act as a cell cycle regulator through E2Fs underlies its oncogenicity in human cancers.
Tomographic Reconstruction and Alignment Using Matrix Norm Minimization

Cryo electron tomography is the only imaging modality that can capture individual cellular structures in situ at nanometer resolution. By using subtomogram averaging, a technique for simultaneous alignment and clustering, high-resolution reconstructions of macromolecules can often be achieved. Two main challenges of subtomogram averaging are a low SNR and missing frequency components that can lead to distortions in random directions in the reconstructed macromolecules. To cope with these challenges, we explore formulating the subtomogram averaging problem as a low-rank matrix recovery problem subject to constraints on the misfit to the Radon measurements. The rank minimization problem is relaxed to a nuclear-norm minimization problem that simultaneously reconstructs the three-dimensional structures and recovers the alignment parameters. The resulting reconstructions using small synthetic 32 $\,\times\,$ 32 $\,\times\,$ 32 data sets are denoised and refined versions of the target structures which are clustered very accurately. However, due to the increased computational complexity, the method is currently not able to align larger data sets.
Scale- dependent effects of nonnative plant invasion on host- seeking tick abundance

Nonnative, invasive shrubs can affect human disease risk through direct and indirect effects on vector populations. Multiflora rose (Rosa multiflora ) is a common invader within eastern deciduous forests where tick- borne disease (e.g., Lyme disease) rates are high. We tested whether R. multiflora in- vasion affects blacklegged tick (Ixodes scapularis) abundance and at what scale. We sampled host- seeking ticks at two spatial scales: fine scale, within R. multiflora - invaded forest fragments; and patch scale, among R. multiflora- invaded and R. multiflora - free forest fragments. At a fine scale, we trapped 2.3 times more ticks under R. multiflora compared with paired traps 25 m away from R. multiflora . At the patch scale, we trapped 3.2 times as many ticks in R. multiflora - free forests compared with R. multiflora - invaded forests. Thus, ticks are concentrated beneath R. multiflora within invaded forests, but uninvaded forests support significantly more ticks. Among all covariates tested, leaf litter volume was the best predictor of tick abun- dance; at the patch scale, R. multiflora - invaded forests had less leaf litter than uninvaded forests. We sug- gest that leaf litter availability at the patch scale plays a greater role in constraining tick abundance than the fine- scale, positive effect of invasive shrubs.
The Redistribution of Impurities Under RTP for Polysilicon Capped Silicon

Rapid Thermal Processing is being evaluated in the IC industry as a way to meet the thermal budget requirements of reduced scaling in high performance Si IC’s. As scaling is reduced and alternative processing is used, the study of low level interfacial impurities is expected to become more important. An example is presented here for the redistribution of interfacial impurities under RTP for polysilicon capped silicon similar to that proposed for shallow junction bipolar transistors.
From Captives to Slaves: Commodifying Indian Women in the Borderlands

On July 21, 1774, fray Miguel Santa Maria y Silva, the leading Franciscan missionary stationed in the mission district of Los Adaes on the border between Texas and Louisiana, reported to the Spanish viceroy in Mexico City on his trip through that region as part of a delegation seeking renewed peace with powerful Wichita and Caddo nations. In 1769, in the aftermath of the Seven Years’ War, Spain had officially established administrative control of the former French province of Louisiana, and the mission to reconfirm Wichita and Caddo alliances sought to represent the new unity of Spaniards and Frenchmen in Louisiana and Texas. Many, however, could not put aside past rivalries so easily, and Santa Maria y Silva was no exception. Rather than detail this first peace council sought by the Spanish government with leading Indian nations, the Franciscan spent page after page lamenting an “infamous traffic of the flesh” he had witnessed being carried on by Frenchmen living in and among Caddoan Indian villages along the Red River. To discredit Frenchmen, Santa Maria y Silva could have deplored the skyrocketing numbers of enslaved Africans and African Americans in Louisiana by the 1770s. Or, given the hostile relations between the Spanish government and many independent and powerful Indian nations in the lower Plains, the missionary could have bemoaned the fate of Spanish women and children from New Mexico who had been taken captive by Indians armed with guns obtained from French traders.
Sclerotherapy of Vascular Malformations

Vascular malformations are congenital lesions secondary to errors in the development of arteries, capillaries, veins, or lymphatics. The majority of these lesions are sporadic, however, a certain percentage can present with syndrome, which the treating physician should be aware of. These lesions are best treated from a multidisciplinary team comprising pediatricians, plastic surgeons, dermatologists, radiologists, otolaryngologists, and oral maxillary facial surgeons. Minimal invasive percutaneous therapies play a vital role in the treatment of vascular malformations, as a sole therapy of choice, or as an adjunct to surgery. Currently, a wide variety of embolic agents are utilized as seen in this chapter in the treatment of vascular malformations, however to date there are no published, randomized, control trials comparing the efficacy of the different agents. Further, animal and clinical studies are needed to help optimize the treatment for each type of vascular malformation.
Predictable wind power via distributed control of wind generators with integrated storage

In this paper we study a leader-follower consensus protocol and establish stability properties of its equilibrium point. Specifically, by employing singular perturbation and Lyapunov theories, we first establish asymptotic stability of the consensus protocol without time-delays. Then, we extend this result and establish time-delay-independent asymptotic stability. Finally, using the proposed protocol we develop a control methodology that can be adopted by a group of state-of-the-art (SoA) wind Double-Fed Induction Generators (DFIGs) with integrated storage to accomplish a group objective. The objective is that the WGs self-organize and control their storage devices in an equal sharing fashion such that the wind farm (WF) generates predictable power output, i.e its total power output matches a given reference. We demonstrate the effectiveness of our method via simulations on the IEEE 24-bus Reliability Test System (RTS).
Counting Women's Ballots: Female Voters from Suffrage through the New Deal

How did the first female voters cast their ballots? For almost 100 years, answers to this question have eluded scholars. Counting Women's Ballots employs new data and novel methods to provide insights into whether, how, and with what consequences women voted in the elections after suffrage. The analysis covers a larger and more diverse set of places, over a longer period of time, than has previously been possible. J. Kevin Corder and Christina Wolbrecht find that the extent to which women voted and which parties they supported varied considerably across time and place, challenging attempts to describe female voters in terms of simple generalizations. Many women adapted quickly to their new right; others did not. In some cases, women reinforced existing partisan advantages; in others, they contributed to dramatic political realignment. Counting Women's Ballots improves our understanding of the largest expansion of the American electorate during a transformative period of American history.
Molecular mechanisms of antigenic variation in Neisseria gonorrhoeae.

Neisseria gonorrhoeae is the causative agent of the sexually transmitted disease gonorrhoea. Gonorrhoea has been recorded in man throughout the medieval and modern eras and possibly since biblical times (Cheng, 1988). This long association between N. gonorrhoeae and humans can be attributed to:(1) an efficient mode of transmission; (2) a high percentage of asymptomatic carriers; (3) the rare occurrence of lethality, and (4) the ability of the bacterium to avoid an immune response. This Gram-negative diplococcus (the gonococcus) does not colonize any other ecological niche besides the human body. A consequence of this limited host range is a lack of a valid animal model. Therefore, a majority of facts concerning gonococcal pathogenesis result from in vitro studies, supported by information gained by occasional experiments in human volunteers. The focus of this chapter is to examine mechanisms used by the bacterium to evade the immune response. Selected topics on other aspects of gonococcal pathogenesis will be presented but for wider analysis the reader is referred to review articles about gonorrhoea by Cannon and Sparling (1984) or Brooks and Donegan (1985) and specifically about antigenic variation by Seifert and So (1988), Swanson and Koomey (1989), or Meyer et. al. (1990).
Application of the QCTO technique to design flat fresnel GRIN lenses

The quasi-conformal transformation optics (QCTO) technique is employed to design flat TO gradient-index (GRIN) Fresnel-type lenses. Because the targeted Fresnel lenses possess complex curvatures and contour profiles with sharp corner boundaries, the QCTO algorithm cannot be applied directly to transform them into a flat TO GRIN lens. This is due to the fact that the QCTO technique is based on a discrete coordinate transformation (DCT) strategy, which relies on the property that the local coordinates of the virtual space and physical space systems remain as near-orthogonal meshes. A novel approach is proposed here to solve the limitations of the application of QCTO to the design of Fresnel lenses, that is: (a) the Fresnel lens is first divided into several sections; (b) the QCTO method is then applied to each section of the lens with homogeneous media and curved shapes; (c) the resulting flat TO GRIN lenses sections are partially truncated on the left/right end sides, then; (d) followed by a stretching transformation along the horizontal direction to yield the same size as the original TO lens sections. Next, (e) the refractive index of the resulting flat TO GRIN lens sections are multiplied by an optimized scaling factor; (f) finally all the modified flat TO lens sections are integrated to form the final flat TO GRIN lens. The imaging performances of the Fresnel lenses and their flat TO GRIN lens counterparts are simulated by use of an in-house graphical processing unit (GPU)-based body of revolution finite-difference time-domain (BOR-FDTD) code. Numerical results demonstrate that flattened TO GRIN lenses have the same focusing properties as free-space Fresnel lens or one mounted on a substrate, for both normally and obliquely incident plane waves.
Cutaneous neurturin overexpression alters mechanical, thermal, and cold responsiveness in physiologically identified primary afferents.

GDNF family neurotrophic factors regulate the development and function of primary sensory neurons. Of these, neurturin has been shown to modulate mechanical and cooling sensitivity behaviorally. He...
Small-scale Propagation of Human iPSCs in Serum-free Conditions for Routine Immunocytochemical Characterization

There is great interest in utilizing human induced pluripotent stem cells (hiPSCs) for disease modeling and cell therapeutics due to their patient specificity and characteristic stemness. However, the pluripotency of iPSCs, which is essential to their functionality, must be confirmed before these cells can be used in such applications. While a rigorous characterization of iPSCs, through different cellular and functional assays is necessary to establish their pluripotency, routine assessment of pluripotency maintenance can be achieved more simply and effectively through immunocytochemical techniques. Here, we present a systematic protocol for culturing hiPSCs, in a scaled-down manner, to particularly facilitate the verification of their pluripotent state using immunocytochemistry. More specifically, this methodology encompasses an efficient and cost-effective means of growing iPSCs in serum-free conditions and plating them on small chamber slides or glass coverslips ideal for immunocytochemistry.
From sound to meaning

Culture and experience contribute to the process that translates a complex acoustic stimulus into an intelligible message.
Summary of the First Wisconsin Symposium on D-3He Fusion, Madison, Wisconsin, August 21–22, 1990

(1992). Summary of the First Wisconsin Symposium on D-3He Fusion, Madison, Wisconsin, August 21–22, 1990. Fusion Technology: Vol. 21, No. 4, pp. 2346-2348.
Type 2 diabetes and cognitive dysfunction in minorities: a review of the literature

Objectives: The purpose of this review was to summarize the current status of knowledge regarding cognitive dysfunction and diabetes in minorities. Literature on the interaction of cognitive dysfun...
Long-acting injectable antipsychotics update: lengthening the dosing interval and expanding the diagnostic indications

Introduction: Long-acting injectable (LAI) antipsychotics are a useful but underutilized option in the management of schizophrenia.Areas covered: This is a narrative review of newer LAI antipsychot...
Do Recent Trends in RA Surgery Reflect Success in Disease Management

Longterm outcomes are critically important for patients living with a chronic disease such as rheumatoid arthritis (RA). Because joints are replaced for endstage damage to the target organ in RA, studies that identify changes in the use of arthroplasty provide insight into changes in longterm RA outcomes. In this issue of The Journal , Young and colleagues1 studied arthroplasties to evaluate use trends for patients with RA. The investigators used data from the National Inpatient Sample (NIS), the largest US all-payer inpatient database, which contains a sample of over 1000 hospitals in the United States participating in the Healthcare Cost and Utilization Project. They assessed trends in the proportion of patients with RA among arthroplasty patients undergoing total shoulder (TSA), elbow (TEA), knee (TKA), hip (THA), and ankle (TAA) arthroplasty between 2002 and 2012. They determined the incidence of arthroplasties based on the US population, and compared the data from the first year of the study, 2002, to the final year, 2012. While studies using large databases such as the NIS have strengths and weaknesses, the large numbers contained in this database permit recognition of surgical trends and can be used to assess trends in less frequently performed procedures such as TAA or TEA. However, relevant data such as disease severity, presence of erosions, or medication use are not available, and coding errors or misclassification may occur. The investigators note, however, that because misclassification is unlikely to change over time, the effect on … Address correspondence to Dr. S.M. Goodman, 535 East 70th St., New York, New York 10021, USA. E-mail: Goodmans{at}hss.edu
Target Organ Modification in Pharmacology: Reversible Osmotic Opening of the Blood-Brain Barrier by Opening of Tight Junctions

We often consider how to modify the physical-chemical properties of a drug—its distribution, excretion, and metabolism—so as to increase its effective action at the target organ. In this paper I shall discuss how a target organ itself—the brain and blood-brain barrier system—can be modified to facilitate entry of normally excluded drugs. I shall present physiological and morphological evidence that the blood-brain barrier can be opened osmotically in a reversible manner without producing gross neurological damage to the animal.
Protein Kinase C Signaling and Expression of the Diabetic Cardiac Phenotype

The focus of this review will be recent advances in the molecular biology of protein kinase C (PKC) signaling in cardiac myocytes and the application of these novel techniques to study the pathobiology of diabetic cardiac myopathy. The PKC family of serine/threonine kinases have been implicated in a diverse array of biologic responses in health and disease. Compelling evidence has linked PKC signaling to hyperglycemia mediated cell injury. Although the cardiac myocyte has not been traditionally considered a major target cell for insulin, high ambient concentration of glucose promotes the activation of cardiac PKC isozymes, that target physiologically relevant intracellular substrates. The availability of genetically engineered mice, with targeted activation of distinct PKC isozyme (PKCe) in cardiac muscle cells and the development of selective peptide PKC modulators, provides an approach to examine PKC signaling events in the diabetic heart, and to explore the in vivo and in vitro consequences of this signaling cascade. The rapid growth of knowledge in this area is critical to the development of therapeutic strategies with the potential to arrest or reverse the progression of diabetic cardiomyopathy.
Phenotype-centric modeling for elucidation of biological design principles

A recently developed 'phenotype-centric' modeling strategy combines four innovations with the potential to advance our understanding of complex biological systems: (1) a rigorous mathematical definition of biochemical phenotypes, (2) a method for enumerating the phenotypic repertoire based on the biomolecular network architecture, (3) an integrated suite of computational algorithms for the efficient prediction of parameter values and analysis of the phenotypic repertoire, and (4) a user-focused environment for navigating the resulting space of phenotypes and identifying biologically relevant features and system design principles. These innovations will facilitate deterministic and stochastic simulations that require parameter values, will accelerate both hypothesis discrimination in systems biology and the design cycle in synthetic biology. Here we first review the fundamental definition of biochemical phenotype that enables this new modeling strategy and give an overview of the strategy using a simple system from phage λ to provide an example of a global design principle. Second, we illustrate this approach in more detail with an application to a common network architecture involving positive and negative feedback. We report system design principles related to the global tolerances of this system's phenotypes. Finally, we apply the phenotype-centric strategy to a logic network and compare the results with those obtained from a Boolean approach. Mechanistic and Boolean models have well-documented complementary advantages and disadvantages. Mechanistic models have the advantage of being biologically realistic; however, they also are limited by the large number of kinetic parameters whose values are largely unknown. Boolean models have the advantage of being parameter free; however, they also are limited by the absence of well-known physical and chemical constraints. We show that the phenotype-centric modeling strategy combines advantages of both.
A Synthetic, Small, Sulfated Agent Is a Promising Inhibitor of Chlamydia spp. Infection in vivo.

Chlamydia is the most frequently reported sexually transmitted bacteria causing 2.9 million infections annually in the USA. Diagnosis, treatment, and sequelae of chlamydial disease cost billions of dollars each year in the U.S. alone. Considering that a heparin sulfate-like cell surface receptor is involved in Chlamydia infections, we reasoned that sulfated and sulfonated mimics of heparin sulfate would be useful in topical prophylactic prevention of Chlamydia. In this study, we tested a small, synthetic sulfated agent SPGG and three synthetic sulfonated polymers PSS and SPS with average MWs in the range of 11 to 1000 kDa for inhibition against Chlamydia. Infection of HeLa cells with C. muridarum or C. trachomatis in the presence of increasing concentrations of SPGG or sulfonated polymers were quantified by immunofluorescence of Chlamydia inclusions. To determine whether in vitro pre-treatment of SPGG inhibits infection of C. muridarum, HeLa monolayers were incubated with SPGG-containing media, and then infected with Chlamydia. Our in vitro results show that SPGG pre-treatment inhibits Chlamydia infection in a dose-dependent manner. In addition, we further determined if SPGG treatment has an inhibitory effect during infection, therefore cell monolayers were infected with C. muridarum in the concurrent presence of SPGG. Our results show that SPGG inhibits C. muridarum infection with an IC50 at 10 μg/ml levels. We also tested the inhibitory effect of synthetic polymers PSS and SPS against Chlamydia and found inhibition of C. muridarum and C. trachomatis infections with IC50 ranging from 0.3 – 0.8 μg/ml. SPGG, PSS and SPS inhibit formation of Chlamydia inclusions in a concentration-dependent manner. For evaluation of in vivo efficacy of the most effective agent in blocking C. muridarum, SPGG, we intravaginally pre-treated mice with SPGG before infection with C. muridarum. Cervical swabs were collected post-infection to quantify Chlamydia inclusions in vitro. Our in vivo data show that the SPGG-treated group has a statistically significant reduction of infection compared to the no-treatment control. Overall, our results show that SPGG could serve as a promising topical inhibitor for preventing Chlamydia infection.
Identification, Pathogenicity, and Spore Trapping of Colletotrichum karstii Associated with Twig and Shoot Dieback in California

Colletotrichum Corda, 1831 species are well-documented pathogens of citrus that are associated with leaf and fruit anthracnose diseases. However, their role in twig and shoot dieback diseases of citrus has recently become more prominent. Recent surveys of orchards in the Central Valley of California have revealed C. gloeosporioides and a previously undocumented species, C. karstii, to be associated with twig and shoot dieback. Pathogenicity tests using clementine (cv. 4B) indicated that both C. karstii and C. gloeosporioides are capable of producing lesions following inoculation of citrus stems. Pathogenicity tests also revealed C. karstii to be the most aggressive fungal species producing the longest lesions after 15 months. The majority of spores trapped during this study were trapped during or closely following a precipitation event with the majority of spores being trapped from January through May. These findings confirm C. karstii as a new pathogen of citrus in California.
Altruism, the free rider problem and group size

This paper is a survey of recent theoretical work on the influence of group size, perceived effectiveness or noticeability of one's behavior on a group member's altruistic responses. Experiments on this problem are analyzed and evaluated, and problems for future research are outlined.
Infrared Spectroscopic Observation of the Group 13 Metal Hydroxides, M(OH)1,2,3 (M =Al, Ga, In, and Tl) and HAl(OH)2

Reactions of laser-ablated Al, Ga, In, and Tl atoms with H2O2 and with H2 + O2 mixtures diluted in argon give new absorptions in the O−H and M−O stretching and O−H bending regions, which are assigned to the metal mono-, di-, and trihydroxide molecules. Isotopic substitutions (D2O2, 18O2, 16,18O2, HD, and D2) confirm the assignments, and DFT calculations reproduce the experimental results. Infrared spectra for the Al(OH)(OD) molecule verify the calculated C2v structure. The trihydroxide molecules increase on annealing from the spontaneous reaction with a second H2O2 molecule. Aluminum atom reactions with the H2 + O2 mixtures favor the HAl(OH)2 product, suggesting that AlH3 generated by UV irradiation combines with O2 to form HAl(OH)2.
Expansion of B4GALT7 linkeropathy phenotype to include perinatal lethal skeletal dysplasia.

Proteoglycans have a core polypeptide connected to glycosaminoglycans (GAGs) via a common tetrasaccharide linker region. Defects in enzymes that synthesize the linker result in a group of autosomal recessive conditions called “linkeropathies”. Disease manifests with skeletal and connective tissue features, including short stature, hyperextensible skin, and joint hypermobility. We report a family with three affected pregnancies showing short limbs, cystic hygroma, and perinatal death. Two spontaneously aborted; one survived 1 day after term delivery, and had short limbs, bell-shaped thorax, 11 ribs, absent thumbs, and cleft palate. Exome sequencing of the proband and one affected fetus identified compound heterozygous missense variants, NM_007255.3: c.808C>T (p.(Arg270Cys)) and NM_007255.3: c.398A>G (p.(Gln133Arg)), in B4GALT7, a gene required for GAG linker biosynthesis. Homozygosity for p.(Arg270Cys), associated with partial loss of B4GALT7 function, causes Larsen of Reunion Island syndrome (LRS), however no previous studies have linked p.(Gln133Arg) to disease. The p.(Gln133Arg) and p.(Arg270Cys) variants were transfected into CHO pgsB-618 cells. High protein expression of p.(Gln133Arg) was found, with mislocalization, compared to p.(Arg270Cys) that had a normal Golgi-like pattern. The p.(Gln133Arg) had almost no enzyme activity and little production of heparan sulfate GAGs, while p.(Arg270Cys) only had 17% of wild-type activity. These findings expand the phenotype of B4GALT7-related linkeropathies to include lethal skeletal dysplasia due to more severe loss of function.
A note on the robustness of quantile treatment effect estimands

Abstract This note examines the robustness of two quantile treatment effect estimands to a perturbation away from the common effect assumption. The first estimand Q Y 1 − Y 0 ( τ ) is the τ -quantile of the difference between the potential outcomes and the second estimand Q Y 1 ( τ ) − Q Y 0 ( τ ) is the difference between the τ -quantiles of the potential outcomes. To this end, this note provides a simple “trembling hand” example whereby the treatment effect deviates from the common effect β for only one individual in a large population. As a result, each estimand deviates from β , and may have the opposite sign than β , in a distinct range of τ . In general, this perturbation leads Q Y 1 − Y 0 ( ⋅ ) to differ from β more severely but only in a small and extreme range of τ whereas it leads Q Y 1 ( ⋅ ) − Q Y 0 ( ⋅ ) to differ from β less severely but over a substantial and central range of τ . This example suggests that researchers should carefully evaluate estimates or bounds for Q Y 1 − Y 0 ( τ ) and especially Q Y 1 ( τ ) − Q Y 0 ( τ ) over a large range of τ .
Health System Pharmacist Protocol for Myocarditis Monitoring in Clozapine-Naïve Inpatients-Two Years' Experience.

Objective:To describe the implementation and efficacy of a pharmacist-driven clozapine myocarditis monitoring protocol including 2 cases of clozapine-induced myocarditis identified using this proto...
The Noble Gases

Excluding 3He, the rare-gas nuclei amenable to NMR investigation are 21Ne, 83Kr, 129Xe, and 131Xe. Of these only Kr and Xe form compounds that are stable under ordinary conditions, and only 129Xe NMR spectra have been observed in chemical environments other than the pure element, although Kr compounds have been characterized by 19F NMR spectroscopy.(1) Thus, we will divide this chapter into two parts: (1) 129Xe NMR studies of bonding and structure of xenon compounds, and (2) 3He, 21Ne, 83Kr, 129Xe, and 131Xe NMR studies of nonspecific intermolecular interactions.
Effectiveness of the Convention on the Rights of the Child in realization of the right to a remedy for Child victims of violence in Africa.

Abstract Background In spite of the progress achieved over the years since the adoption of the CRC, violence against children (VAC) is still widespread in the African continent. Although some progress has been made in addressing violence through the implementation of the CRC and its use in the courts, there are still gaps particularly in ensuring access to remedies for child victims of violence. Participants and setting The strongest factors that impede measures to eliminate VAC in Africa are its broad societal acceptance and weak remedial tools to deal with the issue, including access to justice and assistance to child victims. The article analyses the CRC’s implementation and influence on national courts' jurisprudence on VAC in South Africa, Nigeria and Kenya. Objective The objective of the article' is to provide evidence of the positive impact of the CRC on the right to a remedy for child victims of violence in selected African states, while highlighting existing gaps. Methods Evidence is provided through the CRC reporting process, and the impact of the CRC on national courts in Kenya, Nigeria and South Africa. Results Facts and cases of redress for child victims of violence in the three states, as well as roles and paths that the stakeholders undertake, reflect the strength, value, role and influence of the CRC on the recent progress in these states. The law and legal advocacy in the selected African states have proved to be a powerful tool for children. Conclusions There is a need for more evidence and more research on the topic in each African state, so that real impact of the CRC on national remedial activities, as well as its relatedness to the level and types of violence against children is properly evaluated.
Design and Fabrication of AlGaAs-based 1.8eV Schottky Solar Cell

In this paper, Schottky junction solar cells fully based on Al 0.3 Ga 0.7 As with optical band gap energy of 1.8 eV are designed, fabricated, and characterized. In solar cells based on AlGaAs/GaAs heterostructure, AlGaAs is mostly used as window or electron barrier layer, while GaAs is used as active photon absorbing layer. This work is among the first reports of AlGaAsbased Schottky solar cells. AlGaAs/GaAs heterojunction grown using molecular beam epitaxy (MBE) is used for fabrication of photovoltaic devices. Photovoltaic properties of solar cells are reported, along with experimental details of device fabrication and characterization. The optimized bandgap energy of 1.8eV makes this structure suitable for use in III-V//Si wide-bandgap multi-junction solar cells.
The Reemergence of Ketamine for Treatment in Critically Ill Adults.

Objectives To assess the evidence and discuss the risks and clinical relevance of ketamine for the treatment of various disease states impacting the adult critically ill population. Data sources A literature review was performed using PubMed evaluating primary literature published until August 2018. Study selection Case reports, observational studies (cohort, case-control), and randomized controlled trials involving patients 18 years and older in a nonperioperative setting using either IV or intramuscular ketamine were included for analysis. Uses of ketamine discussed focused on critically ill patients in the ICU and emergency department settings. Data extraction Included studies were evaluated for dosing, outcomes, and adverse effects of ketamine. For each study, the design, population, intervention, investigated outcomes, and results were assessed. Data synthesis The evidence was organized according to use of ketamine, which included pain, sedation, status asthmaticus, alcohol withdrawal syndrome, status epilepticus, and acute behavioral psychologic disturbances. Evaluation of the evidence was based on the included primary literature along with any related guideline recommendations. Conclusions Ketamine has suggested potential benefit in several disease states impacting critically ill patients including pain, alcohol withdrawal syndrome, status epilepticus, and acute agitation. Further supporting evidence is needed to validate its use in the setting of critical illness.
Such a Long Journey: Peacebuilding After Genocide in Rwanda

Until its 1994 genocide, Rwanda was among the world’s most obscure countries: a tiny dot on the map of Africa, rarely studied, even more rarely in the news. Today, no country in Africa divides opinion among scholars and commentators as fiercely as Rwanda. A development success, rising from the ashes of mass ethnic slaughter? Or a case of autocratic recidivism, masked by a bogus narrative of national unity? This chapter breaks Rwanda’s highly contested peacebuilding into four main parts—military, society, economy, and youth & women—to put some distance between its tangible gains and failings, on one hand, and the presumed aims and personality of President Paul Kagame, on the other. It finds that Rwanda is a complex—but by no means secure—success.
Five-dimensional quantitative low-dose Multitasking dynamic contrast- enhanced MRI: Preliminary study on breast cancer.

PURPOSE To develop a low-dose Multitasking DCE technique (LD-MT-DCE) for breast imaging, enabling dynamic T1 mapping-based quantitative characterization of tumor blood flow and vascular properties with whole-breast coverage, a spatial resolution of 0.9 × 0.9 × 1.1 mm3 , and a temporal resolution of 1.4 seconds using a 20% gadolinium dose (0.02 mmol/kg). METHODS Magnetic resonance Multitasking was used to reconstruct 5D images with three spatial dimensions, one T1 recovery dimension for dynamic T1 quantification, and one DCE dimension for contrast kinetics. Kinetic parameters Fp , vp , Ktrans , and ve were estimated from dynamic T1 maps using the two-compartment exchange model. The LD-MT-DCE repeatability and agreement against standard-dose MT-DCE were evaluated in 20 healthy subjects. In 7 patients with triple-negative breast cancer, LD-MT-DCE image quality and diagnostic results were compared with that of standard-dose clinical DCE in the same imaging session. One-way unbalanced analysis of variance with Tukey test was performed to evaluate the statistical significance of the kinetic parameters between control and patient groups. RESULTS The LD-MT-DCE technique was repeatable, agreed with standard-dose MT-DCE, and showed excellent image quality. The diagnosis using LD-MT-DCE matched well with clinical results. The values of Fp , vp , and Ktrans were significantly different between malignant tumors and normal breast tissue (P < .001, < .001, and < .001, respectively), and between malignant and benign tumors (P = .020, .003, and < .001, respectively). CONCLUSION The LD-MT-DCE technique was repeatable and showed excellent image quality and equivalent diagnosis compared with standard-dose clinical DCE. The estimated kinetic parameters were capable of differentiating between normal breast tissue and benign and malignant tumors.
Covalent Organic Frameworks as Electrode Materials for Rechargeable Batteries

Covalent organic frameworks (COFs) are an advanced class of crystalline porous polymers that have garnered significant interest due to their tunable properties and robust molecular architectures. As a result, COFs with energy-storage properties are of particular interest to the field of rechargeable battery electrode materials. However, investigation into COFs as candidates for energy-storage materials is still in its infancy. This review will highlight methods used to fabricate COFs used as electrode materials and discuss the factors that prove critical for their production. A collection of known COF-based energy-storage systems will be featured. In addition, the ability to utilize the storage properties of COFs for systems beyond traditional Li-ion batteries will be addressed. An outlook will address the current progress and remaining challenges facing the field to ultimately expand the scope of their applications.
The economic aftermath of Hurricanes Harvey and Irma: The role of federal aid

Abstract This paper empirically examines the economic impacts of Hurricanes Harvey and Irma in 2017 and the role of federal aid in the recovery process. Data from 90 disaster counties in Texas and Florida reveal a strong relationship between direct economic damages and localized measures of storm intensity. The impact of the storms on local unemployment and employment dissipated within one year. Along with individual communities’ inherent resilience capacity, federal policy responses to economic losses played a role in supporting local recovery across the two disaster regions. Regressions with spatial effects show that following a hurricane strike, employment and wages moved in the opposite directions between a disaster county and its neighboring counties.
Qualitative insights on how adult e-cigarette users describe quantity of e-cigarettes used - PATH Study 2018.

Abstract The purpose of this qualitative study was to gain a deeper understanding of how adult e-cigarette users describe quantity of e-cigarettes used. Data for this analysis came from a qualitative study of U.S. adult dual e-cigarette and cigarette users and former cigarette smokers aged 18 years and older. Eligible respondents from Wave 4 (2016–2017) of the Population Assessment of Tobacco and Health (PATH) Study responded to a brief web questionnaire and participated in an in-depth telephone interview (n = 112) between March and August 2018. Using the respondent’s native terminology for their e-cigarette device, interviewers asked respondents to describe in their own words the quantity of e-cigarettes used. Using NVivo software, interview transcripts were coded and analyzed to identify themes and patterns. Respondents described quantity used in three different ways: number of times and/or puffs; device-specific terms (i.e., replacement of disposable devices, cartridges/pods; use of e-liquid); and perceived equivalence to a quantity of traditional cigarettes. The most commonly reported approach across all device types and levels of device proficiency, although with varying ease and specificity, was the number of times and/or puffs taken in a day. Several respondents used multiple approaches to describe quantity. E-cigarette users use a variety of approaches to describe quantity of e-cigarette used, contributing to challenges developing standardized survey measures. The variety of approaches should be taken into consideration along with device type and other contextual factors such as device proficiency when developing survey questions.
Trend Based Asset Flow in Technical Analysis and Securities Marketing

This article generalizes the asset flow model of the dynamics of equity prices to multiple groups of investors with distinct strategies and assessments of value. Applications include the closed-end fund puzzle, government privatizations, and marketing of initial and secondary offerings of equities. The generalized model is used to provide a theoretical foundation for the practice of technical analysis, in which price history and patterns are examined in order to obtain an indication of future prices. The asset flow model, which is an extension of price adjustment due to disequilibria, tracks the finite assets of each group and involves a preference function that is governed by the price trend in addition to the fundamental value of the equity. The system, which consists of a system of ordinary differential equations, uses four parameters that characterize the extent to which investors' preferences are governed by trend versus fundamental value, and the time scales associated with each motivation. The evolution toward equilibrium is found to be much more complex than the monotonic change that is implied by standard price theories. Finally, the time scale for the return to equilibrium, a concept crucial to securities marketing, is considered in a precise quantitative context. The asset flow approach provides a unified explanation for many of the basic patterns of technical analysis and market phenomena. In particular, the origin of a typical bubble can be explained on the basis of trend-based investors entering a market hitherto dominated by value-based investors. Thus, a channel of increasing prices yields to a breakout from a trend line. The formation of triangle patterns in prices has its origin in the emergence of a second group of investors, with a different assessment of value, that offers fresh supply or demand near a particular price. The article also considers applications to the marketing of securities, consumer preferences which are logically influenced by the popular trend (e.g., VHS versus Beta or long-distance telephone companies) and (particularly three-way) elections.
How Online Tests Contribute to the Support System for People With Cognitive and Mental Disabilities

Roughly 1 in 3 people around the world are affected by cognitive or mental disabilities at some point in their lives, yet people often face a variety of barriers when seeking support and receiving diagnosis from healthcare professionals. While prior work found that people with such disabilities assess themselves using online tests and assessments, it remains unknown whether and how effectively these tests fill gaps in healthcare and general support systems. To find out, we interviewed 17 adults with cognitive or mental disabilities about their motivation for and experience using online tests. We learned that online tests act as an important resource that address the shortcomings in support systems for people with professionally diagnosed or suspected cognitive or mental disabilities. In particular, online tests can lower barriers to a professional diagnosis, provide valuable information about the nuances of a disability, and support people in forming a disability identity – an invaluable step towards a positive acceptance of oneself. Our results also uncovered challenges and risks that prevent people with known or suspected health conditions from fully taking advantage of online tests. Based on these findings, we discuss how online tests can be better leveraged to support people with cognitive or mental disabilities before and after professional diagnosis.