125 resultados para Idiopathic Generalized Epilepsies


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Despite long-standing calls for patient-focused research on individuals with generalized anxiety spectrum disorder there is little systematized knowledge about the in-session behaviors of these patients. The primary objective of this study was to describe of in-session trajectories of the patients' level of explication (as an indicator of an elaborated exposure of negative emotionality) and the patients' focus on their own resources and how these trajectories are associated with post-treatment outcome. In respect to GAD patients, a high level of explication might be seen as an indicator of successful exposure of avoided negative emotionality during therapy sessions. Observers made minute-by-minute ratings of 1100 minutes of video of 20 patients-therapists dyads. The results indicated that a higher level of explication generally observed at a later stage during the therapy sessions and the patients' focus on competencies at an early stage was highly associated with positive therapy outcome at assessment at post treatment, independent of pretreatment distress, rapid response of well-being and symptom reduction, as well as the therapists' professional experience and therapy lengths. These results will be discussed under the perspective of emotion regulation of patients and therapist's counterregulation. It is assumed that GAD-Patients are especially skilled in masking difficult emotions. Explication level and emotion regulation are important variables for this patient group but there's relation to outcome is different.

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Abstract Several monogenic defects have been reported to be associated with idiopathic short stature. Focusing on growth hormone receptor (GHR)-gene alterations, the heterozygosity of the same gene defect may be associated with a range of growth deficits. We found a heterozygous mutation (V144I) within exon 6 of the GHR gene in a patient with a low level of insulin-like growth factor I (IGF-I), normal level of GH, and severe short stature. Despite the lack of statistical difference, an overall tendency for reduced wt-GH-induction of GHR activation and Jak/Stat signalling in cells transiently expressing GHR-V144I alone or co-expressing wt-GHR compared to cells expressing only wt-GHR was found when GH doses were increased. Our results suggest that, although GHR sequence variants are responsible for some functional alterations commonly observed in children with idiopathic short stature, these changes may not explain all the height deficits observed in these subjects.

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Idiopathic chylothorax and limb edema was diagnosed in two 2-year-old male whippet siblings. The fact that the 2 related animals developed similar clinical signs at a young age may suggest a congenital or hereditary etiology.

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Acute generalized exanthematous pustulosis (AGEP) and generalized pustular psoriasis (GPP) are rare pustular skin disorders with systemic involvement. IL-17A/F is a proinflammatory cytokine involved in various neutrophilic inflammatory disorders. Here we show that IL-17A/F is highly expressed by innate immune cells such as neutrophils and mast cells in both AGEP and GPP.

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INTRODUCTION An accurate description of the biomechanical behavior of the spine is crucial for the planning of scoliotic surgical correction as well as for the understanding of degenerative spine disorders. The current clinical assessments of spinal mechanics such as side-bending or fulcrum-bending tests rely on the displacement of the spine observed during motion of the patient. Since these tests focused solely on the spinal kinematics without considering mechanical loads, no quantification of the mechanical flexibility of the spine can be provided. METHODS A spinal suspension test (SST) has been developed to simultaneously monitor the force applied on the spine and the induced vertebral displacements. The system relies on cervical elevation of the patient and orthogonal radiographic images are used to measure the position of the vertebras. The system has been used to quantify the spinal flexibility on five AIS patients. RESULTS Based on the SST, the overall spinal flexibility varied between 0.3 °/Nm for the patient with the stiffer curve and 2 °/Nm for the less rigid curve. A linear correlation was observed between the overall spinal flexibility and the change in Cobb angle. In addition, the segmental flexibility calculated for five segments around the apex was 0.13 ± 0.07 °/Nm, which is similar to intra-operative stiffness measurements previously published. CONCLUSIONS In summary, the SST seems suitable to provide pre-operative information on the complex functional behavior and stiffness of spinal segments under physiological loading conditions. Such tools will become increasingly important in the future due to the ever-increasing complexity of the surgical instrumentation and procedures.

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We present a general method for inserting proofs in Frege systems for classical logic that produces systems that can internalize their own proofs.