Duchenne muscular dystrophy (DMD) is a degenerative disease primarily affecting voluntary muscles with secondary consequences on heart and breathing muscles. DMD is an X-linked recessive disease that ...results in the loss of dystrophin, a key muscle protein. Inflammation can play different roles in DMD; it can be a secondary response to muscle degeneration, a primary cause of degeneration, or can contribute to the disease progression. Several immunosuppressants have been used with the aim to reduce the inflammation associated with DMD. Most recently, myoblast transplantation has shown the possibility to restore the dystrophin lack in the DMD patientʼs muscle fibers and this evidence has emphasized the importance of the use of immunosuppressants and the necessity of studying them and their secondary effects. The aim of this review is to analyze the main immunosuppressants drugs starting from the mdx mice experiments and concluding with the most recent human clinical studies.
Although data from parent-implemented Naturalistic Developmental Behavioral Interventions have shown positive effects on decreasing core symptoms of autism, there has been limited examination of the ...effectiveness of Naturalistic Developmental Behavioral Interventions in community settings. In addition, parent perspectives of their involvement in parent-implemented early intervention programs have not been well studied. Using both qualitative and quantitative data to examine parent perspectives and the perceived feasibility of parent training by community providers, 13 families were followed as they received training in the Naturalistic Developmental Behavioral Intervention, Project ImPACT. Data indicate that parent training by community providers is feasible and well received, and parents find value in participating in intervention and perceive benefit for their children. Recommendations for adaptation of program elements and future research are discussed.
Community-acquired pneumonia (CAP) is amongst the leading causes of death worldwide. As inflammatory markers, cytokines can predict outcomes, if interpreted together with clinical data and scoring ...systems such as CURB-65, CRB, and Acute Physiology and Chronic Health Evaluation II (APACHE II). The aim of this study was to determine the impact of inflammatory biomarkers on the early mortality of hospitalized CAP patients. Twenty-seven CAP patients needing hospitalization were enrolled for the study and samples of interleukin-1 (IL-1) and interleukin-6 (IL-6), tumor necrosis factor alpha (TNF-α), C-reactive protein (CRP), and homocystein were collected at the time of admission (day 1) as well as on the seventh day of the treatment. There was a significant reduction in the levels of IL-6 between the first and the second collections. Median IL-6 values decreased from 24 pg/mL (day 1) to 8 pg/mL (day 7) (P=0.016). The median levels of TNF-α were higher in patients: i) with acute kidney injury (AKI) (P=0.045), ii) requiring mechanical ventilation (P=0.040), iii) with short hospital stays (P=0.009), iv) admitted to the intensive care unit (ICU) (P=0.040), v) who died early (P=0.003), and vi) with worse CRB scores (P=0.013). In summary, IL-6 and TNF-α levels were associated with early mortality of CAP patients. Longer admission levels demonstrated greater likelihood of early death and overall mortality, necessity of mechanical ventilation, and AKI.
The rate of scientific discovery can be accelerated through computation and visualization. This acceleration results from the synergy of expertise, computing tools, and hardware for enabling ...high-performance computation, information science, and visualization that is provided by a team of computation and visualization scientists collaborating in a peer-to-peer effort with the research scientists. In the context of this discussion, high performance refers to capabilities beyond the current state of the art in desktop computing. To be effective in this arena, a team comprising a critical mass of talent, parallel computing techniques, visualization algorithms, advanced visualization hardware, and a recurring investment is required to stay beyond the desktop capabilities. This article describes, through examples, how the Scientific Applications and Visualization Group (SAVG) at NIST has utilized high performance parallel computing and visualization to accelerate condensate modeling, (2) fluid flow in porous materials and in other complex geometries, (3) flows in suspensions, (4) x-ray absorption, (5) dielectric breakdown modeling, and (6) dendritic growth in alloys.
Objective To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Study design Patients enrolled in a PFAPA ...registry were contacted and surveyed. Results Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up ( P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days ( P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses. Conclusions In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.
Platelets contain exceptionally high levels of pp60c-src and, thus, provide a convenient system for investigating the physiological
function of this protein-tyrosine kinase. We have employed chemical ...cross-linking of myristylated amino-terminal peptides
of pp60c-src to platelet membranes in order to identify platelet membrane components that interact with pp60c-src to regulate
or mediate its activity. We detected specific binding of radioiodinated peptides to platelet membrane proteins of 32, 50,
92, and 105 kDa. The 32-kDa protein may be related to the putative src receptor component recently identified in fibroblast
membranes. The most reactive platelet protein, however, is the 50-kDa protein, which is either absent or nonreactive in fibroblast
membranes. Binding of src peptides to this protein was saturable, and we estimate the presence of approximately 1 x 10(6)
of the 50-kDa binding sites per platelet. The specificity of the peptide binding to the 50- and 32-kDa platelet proteins was
analyzed by competition with different peptides. The binding sites displayed an absolute requirement for an N-myristoyl moiety
and a strong preference for pp60c-src amino-terminal sequences. The identification of these src-interacting proteins may help
to decipher the biochemical pathways in which platelet pp60c-src is involved.
Entanglement between the constituents of a quantum system is an essential resource in the implementation of many quantum processes and algorithms. Indeed, universal quantum computation is possible by ...measuring individual qubits that constitute highly entangled "cluster states." In this work, it is shown that the unique gapped ground state of noninteracting fermions hopping on a specially prepared lattice is equivalent to a cluster state, where the entanglement between qubits results from fermionic indistinguishability and antisymmetry. A deterministic strategy for universal measurement-based quantum computation with this resource is described. Because most matter is composed of fermions, these results suggest that resources for quantum information processing might be generic in nature.
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