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71.
Summary. Background: A pulmonary embolism (PE) is thought to be associated with atrial fibrillation (AF). Nevertheless, this association is based on weak data. Objectives: To assess whether the presence of AF influences the clinical probability of PE in a cohort of patients with suspected PE and to confirm the association between PE and AF. Patients/methods: We retrospectively analyzed the data from two trials that included 2449 consecutive patients admitted for a clinically suspected PE. An electrocardiography (ECG) was systematically performed and a PE was diagnosed by computer tomography (CT). The prevalence of AF among patients with or without a PE was compared in a multivariate logistic regression model. Results: The prevalence of PE was 22.8% (519/2272) in patients without AF and 18.8% (25/133) in patients with AF (P = 0.28). After adjustment for confounding factors, AF did not significantly modify the probability of PE (odds ratio [OR] 0.68, 95% confidence interval [CI] 0.42–1.11). However, when PE suspicion was based on new‐onset dyspnea, AF significantly decreased the probability of PE (OR 0.47, 95% CI 0.26–0.84). If isolated chest pain without dyspnea was the presenting complaint, AF tended to increase the probability of PE (OR 2.42, 95% CI 0.97–6.07). Conclusions: Overall, the presence of AF does not increase the probability of PE when this diagnosis is suspected. Nevertheless, when PE suspicion is based on new‐onset dyspnea, AF significantly decreases the probability of PE, as AF may mimic its clinical presentation. However, in patients with chest pain alone, AF tends to increase PE probability.  相似文献   
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Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months.   The most striking change within the bone marrow was the presence of many pigment-laden histiocytes which had the typical morphology of sea-blue histiocytes seen in the so-called idiopathic sea-blue histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis.  相似文献   
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Loss-of-function variants in ANKRD11 were identified as the cause of KBG syndrome, an autosomal dominant syndrome with specific dental, neurobehavioural, craniofacial and skeletal anomalies. We present the largest cohort of KBG syndrome cases confirmed by ANKRD11 variants reported so far, consisting of 20 patients from 13 families. Sixteen patients were molecularly diagnosed by Sanger sequencing of ANKRD11, one familial case and three sporadic patients were diagnosed through whole-exome sequencing and one patient was identified through genomewide array analysis. All patients were evaluated by a clinical geneticist. Detailed orofacial phenotyping, including orthodontic evaluation, intra-oral photographs and orthopantomograms, was performed in 10 patients and revealed besides the hallmark feature of macrodontia of central upper incisors, several additional dental anomalies as oligodontia, talon cusps and macrodontia of other teeth. Three-dimensional (3D) stereophotogrammetry was performed in 14 patients and 3D analysis of patients compared with controls showed consistent facial dysmorphisms comprising a bulbous nasal tip, upturned nose with a broad base and a round or triangular face. Many patients exhibited neurobehavioural problems, such as autism spectrum disorder or hyperactivity. One-third of patients presented with (conductive) hearing loss. Congenital heart defects, velopharyngeal insufficiency and hip anomalies were less frequent. On the basis of our observations, we recommend cardiac assessment in children and regular hearing tests in all individuals with a molecular diagnosis of KBG syndrome. As ANKRD11 is a relatively common gene in which sequence variants have been identified in individuals with neurodevelopmental disorders, it seems an important contributor to the aetiology of both sporadic and familial cases.  相似文献   
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OBJECTIVE: To assess the effectiveness of a non-pharmacological intervention in patients with asthma on conventional therapy including inhaled corticosteroid. DESIGN: A randomised controlled trial of the Buteyko technique in a group of adults with asthma. The control group was trained by a physiotherapist in breathing and relaxation techniques. SETTING: A single centre associated with a University-based asthma programme. MAIN OUTCOME MEASURE: Asthma control, defined by a composite score based on the Canadian asthma consensus report 6 months after completion of the intervention. RESULTS: Both groups showed substantial and similar improvement and a high proportion with asthma control 6 months after completion of the intervention. In the Buteyko group the proportion with asthma control increased from 40% to 79% and in the control group from 44% to 72%. In addition the Buteyko group had significantly reduced their inhaled corticosteroid therapy compared with the control group (p=0.02). None of the other differences between the groups at 6 months were significant. CONCLUSIONS: Six months after completion of the interventions, a large majority of subjects in each group displayed control of their asthma with the additional benefit of reduction in inhaled corticosteroid use in the Buteyko group. The Buteyko technique, an established and widely recognised intervention, or an intensive programme delivered by a chest physiotherapist appear to provide additional benefit for adult patients with asthma who are being treated with inhaled corticosteroid.  相似文献   
78.
Using an experimental model of hepatic Echinococcus multilocularis infection in C57BL/6J mice, intraperitoneal administration of 0.8 μg of recombinant IL-12 to mice with an established infection was shown to reduce the parasite burden as soon as two weeks after the end of treatment. At that time, in vitro Echinococcus multilocularis -induced spleen T cell proliferative responses as well as IFN-γ and IL-5 production were higher in IL-12 treated mice than in untreated mice. Administration of 0.8 μg of IL-12 at the time of infection was shown to be without effect on the parasite establishment. However, this treatment greatly inhibited the subsequent metacestode development. Indeed, ten weeks after infection, it induced a complete healing in 37.5% of mice. At that time, the development of metastases was inhibited in 68.75% of IL-12-treated mice. This reduction of parasite burden was mainly associated with a strong proliferation of spleen cells to E. multilocularis antigen and with a high IFN-γ production. Altogether, our results show that IL-12 is of crucial importance in inhibiting the larval growth after the metacestode establishment in the liver and suggest that this cytokine could be of potential value in the treatment of human alveolar echinococcosis .  相似文献   
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Context

Studies on multiple dimensions of the symptom experience of patients with gastrointestinal cancers are extremely limited.

Objective

Purpose was to evaluate for changes over time in the occurrence, severity, and distress of seven common symptoms in these patients.

Methods

Patients completed Memorial Symptom Assessment Scale, six times over two cycles of chemotherapy (CTX). Changes over time in occurrence, severity, and distress of pain, lack of energy, nausea, feeling drowsy, difficulty sleeping, and change in the way food tastes were evaluated using multilevel regression analyses. In the conditional models, effects of treatment group (i.e., with or without targeted therapy), age, number of metastatic sites, time from cancer diagnosis, number of prior cancer treatments, cancer diagnosis, and CTX regimen on enrollment levels, as well as the trajectories of symptom occurrence, severity, and distress were evaluated.

Results

Although the occurrence rates for pain, lack of energy, feeling drowsy, difficulty sleeping, and change in the way food tastes declined over the two cycles of CTX, nausea and numbness/tingling in hands/feet had more complex patterns of occurrence. Severity and distress ratings for the seven symptoms varied across the two cycles of CTX.

Conclusions

Demographic and clinical characteristics associated with differences in enrollment levels as well as changes over time in occurrence, severity, and distress of these seven common symptoms were highly variable. These findings can be used to identify patients who are at higher risk for more severe and distressing symptoms during CTX and to enable the initiation of preemptive symptom management interventions.  相似文献   
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