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61.
Neutropenia is an almost constant feature of Chediak-Higashi syndrome (CHS). There is evidence for a central mechanism of neutropenia. Ultrastructural studies of the bone marrow from a child with CHS showed marked autophagia phenomena within myeloid precursor cells and mature neutrophils. Autophagic vacuoles were randomly distributed in the cytoplasm of the cells from the granulocytic series and some of them contained giant granules which thus appeared particularly resistant to the autophagic process. The vital cellular damage through endophagocytosis suggests the possibility of intramedullary destruction as an explanation for neutropenia.  相似文献   
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Repetitive electrical stimulation of wrist extensor muscles in rat was combined with quantitative 14C-deoxyglucose autoradiography to study sensory systems functionally activated during forelimb movement. Metabolism increased ipsilaterally in the wrist extensors, the dorsal horn of the cervical spinal cord, the cuneate nucleus and cerebellar hemisphere. The metabolic activation in cerebellum occurred in cortex surrounding the primary fissure anteriorly (lobules simplex and V), and the prepyramidal fissure posteriorly (lobules paramedian and copula pyramis). Metabolism was increased in both granule cell and molecular layers and was uniform throughout the zone of activation. Hindlimb stimulation primarily activated the medial aspect of copula pyramis, demonstrating the somatotopic specificity of changes. Forelimb stimulation also activated contralateral sites in the dorsal accessory nucleus of the inferior olive, ventrobasal thalamus, and SI and SII in cortex. Studies of the relationship between the magnitude of the response and the frequency of the stimulation revealed a positive correlation in muscle, dorsal horn and cuneate nucleus. Other activated sites only showed a significant change at the highest rates of stimulation. Comparison of the pattern of metabolic activation during forelimb movements induced centrally (Collins et al., 1986) with the pattern induced peripherally revealed overlap primarily in the paramedian zone of anterior and posterior cerebellum, and the granular cortex of SI and SII. These studies suggest that forelimb movement initiated centrally would have considerable influence on feedback sensation from the moving limb in these sites.  相似文献   
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In the organ transplantation area the application of the evidence-based medicine (EBM) methods may be limited by several, heterogeneous conditions, eg, mandatory laws and protocols, logistic concerns, as well as donor/recipient matching. In this report we have described the results of a first EBM-oriented course for surgeons and health personnel in a regional transplantation center under the Italian Continuing Medical Education (CME) program. The course was formally approved for 25 credits. It included a maximum of 70 medical and nursing staff registrants; 50 of the spots were reserved for our transplantation center. The course was scheduled in 10 sessions from June to November 2010. Each session was composed of 2 phases: (1) first, computer-assisted education including slide presentations (2 hours); and (2) subsequent discussion led by experts (1.5 hours). The registered participants were expected to be able to correctly answer a multiple-choice, 10-question questionnaire at the end of each session. The majority of the participants considered the course relevant to their need to be updated and effective to improve their clinical skills. The requirements to obtain credits by the Italian CME program for live events were overall presence ≥80% and correct responses in the postsession questionnaires ≥70%. However, among the initially registered participants 31.5% failed at least one of these requirements. The main reason for failure was exceeding the maximum number of absences. Paradoxically, the absences were largely caused by the simultaneous execution of surgical/medical transplantation procedures. For professional figures engaged in complex medical activities, the Italian CME program should consider different threshold limits for the maximum number of absences allowed at live events.  相似文献   
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Imbalance in Th1 and Th2 subsets and their derived cytokines seems to be involved in the immune abnormalities underlying UC and CD. CD30 is a member of the tumour necrosis factor/nerve growth receptor superfamily expressed on T cells producing Th2 cytokines and released as a soluble form. In this study high levels of soluble CD30 were found in sera of UC patients independently of disease activity. Furthermore, increased titres of soluble CD30 molecule were shown, in the same patients, by mitogen-stimulated cultures of peripheral blood mononuclear cells. Our data seem to indicate that an activation of Th2 immune response is involved in the pathogenesis of UC, but not of CD. Furthermore, this finding indicates that serum soluble CD30 measurement may be helpful for differentiating these two forms of inflammatory bowel disease.  相似文献   
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