Common variable immunodeficiency (CVID) is the commonest primary immunodeficiency disease characterized by defective antibody production and various degrees of T cell numbers abnormality or impaired proliferation to mitogens. Clinical features include recurrent bacterial sinopulmonary and gastrointestinal infections. Autoimmunity is very common in CVID, occurring in approximately 25% of the patients particularly with autoimmune thrombocytopenia, hemolytic anemia, inflammatory bowel disease, and rheumatoid arthritis. Persistent antigen stimulation, secondary to a defective eradication of pathogens followed by a compensatory exaggerated chronic inflammatory response, is the primary cause leading to autoimmunity. Here we describe a girl with CVID in whom a chronic liver disease mimicking autoimmune hepatitis developed after hepatitis C virus infection. The immunosuppressive treatment with cyclosporine A proved effective in reversing liver disease. 相似文献
Ischemia is a leading causes of morbidity in giant cell arteritis (GCA). We studied circulating platelets and leukocytes in patients with GCA and with polymyalgia rheumatica. Normal healthy donors (>60 a) served as controls. Patients had a significantly greater fraction of platelets expressing P-selectin, of platelet–Nph and platelet–Mo aggregates, and of Nph and Mo expressing tissue factor. These differences were correlated with the percentage of platelets expressing P-selectin and were not influenced by clinical features or by systemic inflammation. Activated circulating leukocytes and platelets could contribute to indolent vessel inflammation and possibly to thromboembolic events in patients with systemic large vessel vasculitis. 相似文献
Neurological Sciences - The speed of information processing is one of the most reliable indices of cognitive efficiency. The most common way to evaluate this ability is to assess reaction times... 相似文献
Peak width of skeletonized mean diffusivity (PSMD) is a new MRI marker, which has shown clinical relevance in some neurological conditions and, in preliminary data, in multiple sclerosis (MS). We aimed here to investigate, in a group of relapsing-remitting MS (RRMS) patients, the relationship between PSMD and cognitive performances, in comparison with other MRI measures. RRMS patients (n = 60) and normal controls (n = 15) underwent a 3 T MRI examination. MRI-based white matter (WM) lesion volume, microstructural integrity (assessed with Tract-Based Spatial Statistics of diffusion tensor imaging [DTI] images) and brain volumes (i.e., total brain, grey matter [GM] and WM) were computed. In addition, PSMD was calculated through “skeletonization” of WM tracts and diffusion histograms. Cognition was evaluated with Rao’s Brief Repeatable Battery (BRB), which incorporated tests of verbal and visual memory, attention, concentration, information processing speed and verbal fluency. PSMD closely correlated with symbol digit modalities test (SDMT) (r = −0.70, p < 0.001) and, to a lesser extent, with verbal and visual memory tests. Multiple regression analysis showed that PSMD explained SDMT variance (R2 = 0.54, p < 0.001) more than other MRI measures. Results point out the relevance of microstructural damage, as assessed by PSMD, as a reliable marker of cognition in MS, especially in explaining dysfunction in information processing speed.
Clinical Oral Investigations - To evaluate yearly tooth loss rate (TLR) in periodontitis patients with different periodontal risk levels who had complied or not complied with supportive periodontal... 相似文献
The aim of our study is to compare patent foramen ovale (PFO) closure versus medical treatment and antiplatelet versus anticoagulant therapy in patients with cryptogenic stroke (CS) and PFO. We conducted a systematic review and meta-analysis with trial sequential analysis (TSA) of randomized trials. Primary outcomes are stroke or transient ischemic attack (TIA) and all-cause mortality. Secondary outcomes are peripheral embolism, bleeding, serious adverse events, myocardial infarction and atrial dysrhythmias. We performed an intention to treat meta-analysis with a random-effects model. We include six trials (3677 patients, mean age 47.3 years, 55.8% men). PFO closure is associated with a lower recurrence of stroke or TIA at a mean follow-up of 3.88 years compared to medical therapy [risk ratio (RR) 0.55, 95% CI 0.38–0.81; I2?=?40%]. The TSA confirms this result. No difference is found in mortality (RR 0.74, 95% CI 0.35–1.60; I2?=?0%), while PFO closure is associated with a higher incidence of atrial dysrhythmias (RR 4.55, 95% CI 2.16–9.60; I2?=?25%). The rate of the other outcomes is not different among the two groups. The comparison between anticoagulant and antiplatelet therapy shows no difference in terms of stroke recurrence, mortality and bleeding. There is conclusive evidence that PFO closure reduces the recurrence of stroke or TIA in patients younger than 60 years of age with CS. More data are warranted to assess the consequences of the increase in atrial dysrhythmias and the advantage of PFO closure over anticoagulants. 相似文献
Conditions such as muscular dystrophies (MDs) that affect both cardiac and skeletal muscles would benefit from therapeutic strategies that enable regeneration of both of these striated muscle types. Protocols have been developed to promote induced pluripotent stem cells (iPSCs) to differentiate toward cardiac or skeletal muscle; however, there are currently no strategies to simultaneously target both muscle types. Tissues exhibit specific epigenetic alterations; therefore, source-related lineage biases have the potential to improve iPSC-driven multilineage differentiation. Here, we determined that differential myogenic propensity influences the commitment of isogenic iPSCs and a specifically isolated pool of mesodermal iPSC-derived progenitors (MiPs) toward the striated muscle lineages. Differential myogenic propensity did not influence pluripotency, but did selectively enhance chimerism of MiP-derived tissue in both fetal and adult skeletal muscle. When injected into dystrophic mice, MiPs engrafted and repaired both skeletal and cardiac muscle, reducing functional defects. Similarly, engraftment into dystrophic mice of canine MiPs from dystrophic dogs that had undergone TALEN-mediated correction of the MD-associated mutation also resulted in functional striatal muscle regeneration. Moreover, human MiPs exhibited the same capacity for the dual differentiation observed in murine and canine MiPs. The findings of this study suggest that MiPs should be further explored for combined therapy of cardiac and skeletal muscles. 相似文献
AIM: To evaluate whether lymph node pick up by separate stations could be an indicator of patients submitted to appropriate surgical treatment. METHODS: One thousand two hundred and three consecutive gastric cancer patients submitted to radical resection in 7 general hospitals and for whom no information was available on the extension of lymphatic dissection were included in this retrospective study. RESULTS: Patients were divided into 2 groups: group A, where the stomach specimen was directly formalinfixed and sent to the pathologist, and group B, where lymph nodes were picked up after surgery and fixed for separate stations. Sixty-two point three percent of group A patients showed 16 retrieved lymph nodes compared to 19.4% of group B(P 0.0001). Group B(separate stations) patients had significantly higher survival rates than those in group A [46.1 mo(95%CI: 36.5-56.0) vs 27.7 mo(95%CI: 21.3-31.9); P = 0.0001], independently of T or N stage. In multivariate analysis, group A also showed a higher risk of death than group B(HR = 1.24; 95%CI: 1.05-1.46).CONCLUSION: Separate lymphatic station dissection increases the number of retrieved nodes, leads to better tumor staging, and permits verification of the surgical dissection. The number of dissected stations could potentially be used as an index to evaluate the quality of treatment received. 相似文献
The frequency and timing of meals may affect cardiovascular health (CVH) outcomes, but large-scale epidemiological studies are lacking. The aim of this study was to understand the relationship between eating time interval and frequency, and measures of ideal CVH in the Kardiovize Brno cohort study, a random urban sample population in Central Europe.
Methods and Results
1659 members of the Kardiovize Brno 2030 cohort were included in a cross-sectional study (mean age = 46.86 years; 44.6% male). Exposure variables were eating time interval and frequency, and skipping meals. Primary outcomes were indices of CVH, including body mass index, diet, physical activity, smoking, blood pressure, glucose and cholesterol, and the composite CVH score. Cluster analysis and binary logistic regression analysis were used to evaluate eating habits and the association between variables.After adjustment for well-known risk factors, subjects who skipped breakfast or the afternoon snack had a higher risk of poor CVH (OR = 1.613; 95%CI = 1.121–2.320; p = 0.010; OR = 1.409; 95%CI = 1.110–1.788; p = 0.005, respectively). Moreover, we identified three clusters of individuals based on eating habits; from cluster 1 to cluster 3, eating time interval and frequency increased and this was associated with increases in CVH score from 8.70 (SEM = 0.10) in cluster 1, and 9.06 (SEM = 0.08) in cluster 2 to 9.42 (SEM = 0.09) in cluster 3 (p-trend = 0.019).
Conclusions
Our findings suggest that skipping breakfast or the afternoon snack are risk factors for poor CVH, while higher eating time interval and frequency may promote ideal CVH. 相似文献