ObjectiveTo study the clinical features and identify unique renal neoplasia subtypes and their prognostic implications in individuals with tuberous sclerosis complex (TSC).Patients and MethodsThe Mayo Clinic nephrectomy registry included 37 patients with TSC diagnosed between 1970 and 2018. Four additional patients were identified from the pathology consultation and autopsy files. All available renal tumors were further characterized using immunohistochemistry and fluorescence in situ hybridization. Clinicopathologic features and follow-up were obtained from the medical record. The American Association for Cancer Research Project GENIE registry was accessed using cBioPortal for molecular profiling of angiomyolipoma (AML).ResultsA total of 276 renal tumors from 41 patients were analyzed. Renal tumors were classified into 9 distinct morphological subtypes, with AML predominating (238 [86%]). Interestingly, all these tumors acted in a benign fashion except one renal cell carcinoma with clear cells and fibromyomatous stroma and one epithelioid AML that metastasized. Molecular profiling studies revealed that epithelioid AMLs were enriched for alterations of TP53, RB1, and ATRX. Eight patients died of direct complications of TSC, including 3 of end-stage renal disease. To date, none have died of a renal epithelial neoplasm.ConclusionThe identification of unique renal neoplasia subtypes may provide important clues to establish a diagnosis of TSC, and in the somatic setting, this finding has important implications for accurate prognostication. These tumors tend to be indolent, and only 2 of 276 tumors in our study exhibited metastatic behavior. Our results support multidisciplinary management with a focus on preservation of renal function. 相似文献
This meta-analysis focuses on the accuracy of upgrading to clinically significant prostate cancer (PCa) by multiparametric magnetic resonance imaging-targeted biopsy (MRI-TB) versus systematic biopsy (SB). We searched the Medline, Embase, Cochrane Central Register of Controlled Trials, Web of Science, Scopus, and Literatura Latino Americana em Ciências da Saúde databases through January 2020 for comparative, retrospective/prospective, paired-cohort, and randomized clinical trials with paired comparisons. The population consisted of patients with low-risk PCa in active surveillance with at least 1 index lesion on imaging. We evaluated the quality of evidence by using the Quality Assessment of Diagnostic Accuracy Studies-2 score. Group comparisons considered the differences between the area under the curve summary receiver operating characteristic curve in a 2-tailed method. We also compared the positive predictive value of the best single method (MRI-TB or SB) and the referral study test (combined biopsy, a combination of MRI-TB and SB). The meta-analysis included 6 studies enrolling 741 patients. The pooled sensitivity for the 2 groups was 0.79 (95% confidence interval, 0.74-0.83; I2 = 75%) and 0.67 (95% confidence interval, 0.63-0.74; I2 = 55.4%), respectively. The area under the curve for the MRI-TB and SB groups were 0.99 and 0.92 (P < .001), respectively. The positive predictive value for the MRI-TB and combined biopsy groups were similar. The accumulated evidence suggests better results for MRI-TB compared with SB. Therefore, use of MRI-TB alone may be preferable in patients in active surveillance harboring low-risk PCa. 相似文献
Journal of Digital Imaging - Vertebral Compression Fracture (VCF) occurs when the vertebral body partially collapses under the action of compressive forces. Non-traumatic VCFs can be secondary to... 相似文献
Introduction: Boron-containing compounds induce effects on immune responses. Such effects are interesting to the biomedical field for the development of therapeutic tools to modulate the immune system.
Areas covered: The scope of BCC use to modify immune responses is expanding, mainly with regard to inflammatory diseases. The information was organized to demonstrate the breadth of reported effects. BCCs act as modulators of innate and adaptive immunity, with the former including regulation of cluster differentiation and cytokine production. In addition, BCCs exert effects on inflammation induced by infectious and noninfectious agents, and there are also reports regarding their effects on mechanisms involving hypersensitivity and transplants. Finally, the authors discuss the beneficial effects of BCCs on pathologies involving various targets and mechanisms.
Expert opinion: Some BCCs are currently used as drugs in humans. The mechanisms by which these BCCs modulate immune responses, as well as the required structure–activity relationship for each observed mechanism of action, should be clarified. The former will allow for the development of improved immunomodulatory drugs with extensive applications in medicine. Patenting trends involve claims concerning the synthesis and actions of identified molecules with a defined profile regarding cytokines, cell differentiation, proliferation, and antibody production. 相似文献
BackgroundA plain-language summary is a short and clearly stated version of a study’s results using non-scientific vocabulary that provide many advantages for patients and clinicians in the process of shared decision-making.ObjectivesThe primary objective was to investigate the extent to which published reports of physiotherapy interventions provide plain-language summaries. We investigate as the secondary objectives if the available plain-language summaries are at a suitable reading level for a lay person and if inclusion of plain-language summaries in these reports is increasing over time and is associated with trial quality (i.e. PEDro score).Data sourcesAll 4421 randomised controlled trials (RCT), systematic reviews and clinical practice guidelines that included plain-language summaries indexed on Physiotherapy Evidence Database (PEDro) were included.Main outcome measuresProportion of published reports with plain-language summaries, Flesch Reading Ease Score (FRES) and the Flesch–Kincaid Grade Level (FKGL).ResultsThe number of published reports with a plain-language summary doubled in the last 6 years. From a total of 34,444 reports indexed on PEDro, only 4421 reports had English plain-language summaries. RCTs with plain-language summaries had higher PEDro scores than RCTs without plain-language summaries (mean difference = 0.8 points, 95%CI 0.7 to 0.8). Only 2% of reports were considered at a suitable reading level by the FKGL formula and 0.1% by the FRES formula.ConclusionsAlthough the publication of plain-language summaries is increasing over time, the current number corresponds to only 13% of all published reports. In addition the majority of plain-language summaries are written at an advanced reading level. 相似文献
To examine the association of placental levels of arsenic (As), cadmium (Cd), mercury (Hg), lead (Pb), manganese (Mn), and chromium (Cr) with birth outcomes (birth weight, length, and head circumference, low birth weight [LBW], gestational age, preterm delivery, and small for gestational age [SGA]) in mother-child pairs from the Environment and Childhood (INMA) Project in Spain.
Methods
Metal concentrations were measured in placenta tissue samples randomly selected from five INMA cohorts. Data on birth outcomes were obtained from medical records. Associations were assessed in a sub-sample of 327 mother-infant pairs by regression models adjusted for confounding factors and for all metals simultaneously. Effect modification by sex was also evaluated.
Results
Elevated placental Cd levels (>5.79 vs. <3.30?ng/g) were associated with reduced birth weight (?111.8?g, 95%CI?=??215.6; ?8.06, p-trend?=?0.01) and length (?0.62?cm, 95%CI?=??1.20; ?0.04, p-trend?=?0.02), while a 10% increase in Cd was associated with 1.21-fold increased odds (95%CI?=?1.01; 1.43) of LBW in the global sample but with 14% lower odds (95%CI?=?0.78; 0.96) of preterm delivery in males (Pinteraction?=?0.10). Detected (vs. undetected) Hg was associated with reduced head circumference (?0.49?cm, 95%CI?=??1.00; 0.03) in females (Pinteraction?=?0.03). A 10% increase in placental Mn was associated with slight increases in gestational age (0.04 weeks, 95%CI?=?0.01; 0.07) in the global sample and in head circumference (0.05?cm, 95%CI?=??0.01; 0.10) in females (Pinteraction?=?0.03). Elevated Cr levels (>99.6 vs. <56.1?ng/g) were associated with reduced birth length (?0.68?cm, 95%CI?=??1.33; ?0.04, p-trend?=?0.02) and slightly increased gestational age (0.35 weeks, 95%CI?=??0.07; 0.77, p-trend?=?0.08) in the global sample. As and Pb were detected in few placentas (27% and 13%, respectively) and were not associated with any studied birth outcome.
Conclusions
Data suggest that in utero exposure to Cd, Hg, and Cr could adversely affect fetal growth, whereas Mn and Cr appear to have a positive effect on gestational age. Given the relatively small number of subjects, sex-specific associations should be interpreted with caution. 相似文献