Correction of the genetic lesion leading to X-linked SCID with first-generation retroviral vectors has been associated with a 25% risk of acute leukemia. A self-inactivating retrovirus appears to ...retain therapeutic efficacy, with no leukemia yet observed.
X-linked severe combined immunodeficiency (SCID-X1) is caused by mutations in the gene encoding the interleukin-2 receptor γ chain (
IL2RG
) that result in a lack of response to common γ-chain (γc)–dependent cytokines, an absence of T-cell and natural killer (NK)–cell development, and impairment of B-cell function.
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Death from community-acquired or opportunistic infection usually occurs before 1 year of age unless allogeneic hematopoietic stem-cell transplantation (HSCT) is performed. The immunologic defect in children with SCID-X1 obviates the requirement for a preparative regimen before transplantation.
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Standard allogeneic HSCT with matched-sibling donors is associated with an 85 to 90% overall . . .
In rural communities, primary care providers continue to provide mental health services, and about 70% of children and adolescents identified to have a psychiatric disorder never receive treatment. A ...telehealth model for providing integrated mental health services in a school-based health clinic has the potential to increase access to specialized care for the most vulnerable youths. This column provides an overview of the strategies used to implement and integrate such a model in West Virginia. Operationalization, barriers, challenges, and judicious resource use are discussed. Appropriate reimbursement for services and state-specific legislation to ensure consistent revenue to sustain the program are considered.
Reticular dysgenesis is a human severe combined immunodeficiency that is primarily characterized by profound neutropenia and lymphopenia. The condition is caused by mutations in the adenylate kinase ...2 (AK2) gene, resulting in the loss of mitochondrial AK2 protein expression. AK2 regulates the homeostasis of mitochondrial adenine nucleotides (ADP, ATP and AMP) by catalyzing the transfer of high-energy phosphate. Our present results demonstrate that AK2-knocked-down progenitor cells have poor proliferative and survival capacities and are blocked in their differentiation toward lymphoid and granulocyte lineages. We also observed that AK2 deficiency impaired mitochondrial function in general and oxidative phosphorylation in particular - showing that AK2 is critical in the control of energy metabolism. Loss of AK2 disrupts this regulation and leads to a profound block in lymphoid and myeloid cell differentiation.
This article develops the foundations for a theory of interpersonal trust-building based on relational signalling theory (RST). RST is based on the assumptions that rationality is bounded through ...framing, that preferences are partially determined by altruism (through a distinction between foreground and background goals), and that an individual's action is influenced by the normative context in which he or she operates. The focus is on interpersonal trust in work relations within organizations. Interpersonal trust-building is construed as an interactive process in which both individuals learn about each other's trustworthiness in different situations. Four conditions for the building of interpersonal trust within organizations are (1) the suspension of all opportunistic behaviour, or the removal of distrust; (2) exchange of positive relational signals; (3) avoiding negative relational signals, i.e., dealing with trouble; and (4) the stimulation of frame resonance, or the introduction of trust-enhancing organizational policies. The proposed theory can explain important characteristics of trust in organizational contexts, such as the interactive nature of trust, the learning required to build trust, the role of psychological mechanisms (such as attributions and perceptions) in decisions to trust, the limits to trust, asymmetries between trust and distrust and the context-dependency of trust. PUBLICATION ABSTRACT
We report the measurement of the beam-vector and tensor asymmetries A_{ed}^{V} and A_{d}^{T} in quasielastic (eover →,e^{'}p) electrodisintegration of the deuteron at the MIT-Bates Linear Accelerator ...Center up to missing momentum of 500 MeV/c. Data were collected simultaneously over a momentum transfer range 0.1<Q^{2}<0.5 (GeV/c)^{2} with the Bates Large Acceptance Spectrometer Toroid using an internal deuterium gas target polarized sequentially in both vector and tensor states. The data are compared with calculations. The beam-vector asymmetry A_{ed}^{V} is found to be directly sensitive to the D-wave component of the deuteron and has a zero crossing at a missing momentum of about 320 MeV/c, as predicted. The tensor asymmetry A_{d}^{T} at large missing momentum is found to be dominated by the influence of the tensor force in the neutron-proton final-state interaction. The new data provide a strong constraint on theoretical models.
In this article, we review the distrust literature and explore whether the universal sequence for trust as outlined by Dietz (2011) and Dietz and Den Hartog (2006) is also true for distrust. Compared ...to trust, there is much less research on distrust, although the field has been rapidly developing in recent years. We argue that it is time to explore a universal sequence for distrust to take stock of current knowledge and to focus the empirical and conceptual research. There is sufficient evidence to suggest that such a universal sequence is a valuable framework for distrust research. This analytical exercise also forces us to identify tacit assumptions that frame and guide much of the current distrust (and trust) research. In so doing, we identify two main areas that require more attention: 1) the definition of distrust and its relation to trust and 2) the universal sequences and their dynamics. These findings lead to avenues for further research.
We report a precision measurement of the deuteron tensor analyzing powers T(20) and T(21) at the MIT-Bates Linear Accelerator Center. Data were collected simultaneously over a momentum transfer range ...Q=2.15-4.50 fm(-1) with the Bates Large Acceptance Spectrometer Toroid using a highly polarized deuterium internal gas target. The data are in excellent agreement with calculations in a framework of effective field theory. The deuteron charge monopole and quadrupole form factors G(C) and G(Q) were separated with improved precision, and the location of the first node of G(C) was confirmed at Q=4.19±0.05 fm(-1). The new data provide a strong constraint on theoretical models in a momentum transfer range covering the minimum of T(20) and the first node of G(C).
The mean square polarizability radii of the proton have been measured for the first time in a virtual-Compton-scattering experiment performed at the MIT-Bates out-of-plane scattering facility. ...Response functions and polarizabilities obtained from a dispersion analysis of the data at Q2 = 0.057 GeV2/c2 are in agreement with O(p3) heavy baryon chiral perturbation theory. The data support the dominance of mesonic effects in the polarizabilities.
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