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1.
A sixty-year-old man, developed 2–5 mm sized, hyperemic, itchy papules, vesicles, erosions and crusts on hyperemic base on his chest, abdomen, back, gluteal region, and proximal sites of his upper and lower extremities. The direct and indirect immunoflurescence tests were negative. Histology revealed extensive acantholysis in the epidermis in the following forms: pemphigus vulgaris-like suprabasal acantholysis, Darier-like acantholytic dyskeratosis with corps ronds, Hailey-Hailey-like suprabasal clefts, and pemphigus foliaceus-like superficial acantholysis with spongiosis. Using systemic steroids, topical drying, and reepithelising therapy, the patient was cured. He was symptom-free the first, fourth, and thirteenth months after finishing steroid therapy. We review the literature and the new subdivision of the disease according to the histological and clinical features.  相似文献   
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An unbalanced Y;autosome translocation leading to a male with a 45,X karyotype is rare with about 30 published cases. A male with a 45,X karyotype as a result of a unique, submicroscopic, unbalanced Y;16 translocation is presented with alpha-thalassemia/mental retardation syndrome.  相似文献   
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The central role of T cells in antitumor immunity is well established. However, tumor progression, often seen in the presence of substantial lymphocytic infiltration, suggests that these T cells are not capable of mounting an effective immune response to control tumor growth. Evidence has accumulated that T lymphocytes infiltrating human neoplasms are functionally defective, incompletely activated, or anergic. Therefore, when characterizing the immune competent cells within lymphoid infiltrates of tumors, it is important to assess their activation state. We investigated the expression of two T-cell activation markers, interleukin 2 receptor alpha (CD25) and OX40 (CD134), by immunohistochemistry in primary cutaneous melanoma samples of 76 patients and analyzed it in relation to tumor stage and tumor progression (>5 years follow-up), as well as to patients' survival. We found that the degree of infiltration by CD25(+) and intratumoral OX40(+) lymphocytes showed a tendency to decrease in thicker melanomas. The frequency of samples with high numbers of peritumoral CD25(+) and OX40(+) cells was significantly lower (P = 0.0009 and P = 0.0087, respectively) in melanomas developing distant visceral metastases, compared with nonmetastatic or lymph node metastatic tumors. For both activation markers studied, high peritumoral densities were associated with longer survival by univariate analysis (P = 0.0028 and P = 0.0255 for CD25 and OX40, respectively), whereas peritumoral OX40(+) lymphocyte infiltration had an impact on survival also in multivariate analysis (P = 0.035). The results suggest that the presence of lymphocytes expressing the T-cell activation markers CD25 or OX40 shows correlation with tumor progression as well as with patients' survival in cutaneous malignant melanoma.  相似文献   
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We studied cross-cultural Hungarian adaptation and validation of FACES IV for screening adaptive versus nonadaptive family functioning. A protocol was followed including translation, tests of reliability, construct and convergent validity, standardization, testing of family types, and ratios. Two hundred forty-nine couples (498 adults) from various regions of the country participated in the study. Results indicated high item–remainder correlations and Cronbach alphas. Factor analytic data fitted well with the model indicated by the Olson studies. Most salient results were found for Cohesion, which partially overlapped Flexibility, and had high negative correlations with the Disengaged dimension. Rigid and Chaotic as well as Enmeshed were found to be independent factors. Family types of the Hungarian sample explored by cluster analysis were comparable to the U.S. sample.  相似文献   
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Physical therapy in ankylosing spondylitis (AS) is important for maintaining or improving mobility, fitness, functioning, and global health. It also plays a role in the prevention and management of structural deformities. In this study we assessed the functional status of AS patients in relation to disease duration and activity. Furthermore, in volunteering patients we analyzed the efficacy of a controlled, individualized physiotherapeutic program. Altogether, clinical data of 75 AS patients were retrospectively analyzed. Anthropometrical data, duration since diagnosis and disease activity, pain intensity, tender points, sacroiliac joint involvement determined by X-ray, functional condition, and physical activity level were recorded. Subjective, functional, and physical tests were performed. Out of the 75 patients, 10 volunteered to undergo a complex physical exercise program twice a week for 3?months. The program included 1.5?h of general posture reeducation, manual mobilization of the spine, and pelvic-, upper-, and lower-extremity exercises, stretching with joint prevention strategies and functional exercises. In AS, pain intensity recorded on a 10-cm visual analog scale (VAS), BASFI, BASDAI, modified Schober index, chest expansion and occiput-to-wall distance values showed significant correlation with disease activity. The 3-month physical therapy improved several subjective and functional parameters, and markedly reduced pain intensity and spine stiffness. A complex, individualized physical therapy program may be useful and should be introduced to AS patients in order to maintain and increase spine mobility, preserve functional capacity, decrease the pain and stiffness.  相似文献   
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Rapid nonclassical effects of 17β‐oestradiol (E2) on intracellular signalling have been identified in the basal forebrain, although the extent to which these actions may be different in males and females is unknown. Previous work has shown that E2 rapidly phosphorylates cAMP responsive element binding protein (CREB) via ΕRα in female cholinergic neurones. Using this indicator, the present study examined whether nonclassical actions of E2 occur in a sexually dimorphic manner within basal forebrain cholinergic neurones in mice. In addition, we investigated the expression and intracellular distribution of oestrogen receptor (ΕR)α in cholinergic neurones in female and male mice. Animals were gonadectomised and treated 2 weeks later with E2. The number of CREB‐expressing cholinergic neurones was not altered in any of the brain regions after E2 treatment in both males and females. However, E2 treatment rapidly (< 15 minutes) increased (P < 0.05) the number of cholinergic neurones expressing phosphorylated CREB (pCREB) in the substantia innominata and medial septum but not in the striatum in female mice. By contrast, E2 did not change pCREB expression in cholinergic neurones in male mice at any time point (15 minutes, 1 hour, 4 hours), irrespective of the neuroanatomical location. We also observed that, in females, more cholinergic neurones expressed nuclear ΕRα in all regions, whereas males showed more cholinergic neurones with cytoplasmic or both nuclear and cytoplasmic expression of ΕRα. Taken together, these results demonstrate a marked sex difference in the E2‐induced nonclassical effect and intracellular distribution of ΕRα in basal forebrain cholinergic neurones in vivo.  相似文献   
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Background: Despite the increasing popularity of video game playing, little is known about the similarities and differences between online and offline video game players. Objectives: The aims of this study were (i) to test the applicability and the measurement invariance of the previously developed Problematic Online Gaming Questionnaire (POGQ) in both online and offline gamers and to (ii) examine the differences in these groups. Methods: Video game use habits and POGQ were assessed in a sample of 1,964 (71% male) adolescent videogame players. Those gamers who played at least sometimes in an online context were considered “online gamers,” while those who played videogames exclusively offline were considered “offline gamers.” Results: Confirmatory factor analysis supported the measurement invariance across online and offline videogame players. According to the multiple indicators multiple causes (MIMIC) model, online gamers were more likely to score higher on overuse, interpersonal conflict, and social isolation subscales of the POGQ. Conclusion: The results of the present study suggest that online and offline gaming can be assessed using the same psychometric instrument. These findings open the possibility for future research studies concerning problematic video gaming to include participants who exclusively play online or offline games, or both. However, the study also identified important structural features about how online and offline gaming might contribute differently to problematic use. These results provide important information that could be utilized in parental education and the prevention program about the possible detrimental consequences of online vs. offline video gaming.  相似文献   
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