51 research outputs found

    Prognostic value of clinical and MRI features in the screening of lipomatous lesions

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    BACKGROUND AND OBJECTIVES: Differentiation of lipomatous tumors mostly requires diagnostic biopsy but is essential to decide for the most adequate therapy. This study aims to investigate the prognostic value of available clinical and radiological features with regard to malignancy of the lesion, recurrence and survival. METHODS: In this retrospective cohort study, 104 patients with a biopsy-proven lipomatous tumor between 2010 and 2015 and a minimum clinical follow-up of two years were enrolled. Next to clinical features (age, gender, location of the lesion, histopathologic diagnosis, stage of disease, time to recurrence and death), MRI parameters were recorded retrospectively and blinded to the histological diagnosis. RESULTS: Malignant lipomatous tumors were associated with location in the lower extremities and MRI features like thick septation (>2 mm), presence of a non-adipose mass, foci of high T2/STIR signal and contrast agent enhancement. A non-adipose mass was a predictor for recurrence and inferior overall survival, while lesions with high T2/STIR signal showed higher risk of recurrence only. In combination, clinical and radiological features (lower extremities, septation > 2 mm, existence of non-adipose mass, contrast enhancement, and foci of high T2/STIR signal) predicted a malignant lipomatous tumor with an accuracy of 0.941 (95% CI of 0.899-0.983; 87% sensitivity, 86% specificity). CONCLUSION: Localization and characteristic MR features predict malignancy in most lipomatous lesions. Non-adipose masses are a poor prognostic factor, being associated with tumor recurrence and disease-related death

    Severe communication delays are independent of seizure burden and persist despite contemporary treatments in SCN1A + Dravet syndrome: Insights from the ENVISION natural history study

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    Objective: Dravet syndrome (DS) is a developmental and epileptic encephalopathy characterized by high seizure burden, treatment‐resistant epilepsy, and developmental stagnation. Family members rate communication deficits among the most impactful disease manifestations. We evaluated seizure burden and language/communication development in children with DS. Methods: ENVISION was a prospective, observational study evaluating children with DS associated with SCN1A pathogenic variants (SCN1A+ DS) enrolled at age ≤5 years. Seizure burden and antiseizure medications were assessed every 3 months and communication and language every 6 months with the Bayley Scales of Infant and Toddler Development 3rd edition and the parent‐reported Vineland Adaptive Behavior Scales 3rd edition. We report data from the first year of observation, including analyses stratified by age at Baseline: 0:6–2:0 years:months (Y:M; youngest), 2:1–3:6 Y:M (middle), and 3:7–5:0 Y:M (oldest). Results: Between December 2020 and March 2023, 58 children with DS enrolled at 16 sites internationally. Median follow‐up was 17.5 months (range = .0–24.0), with 54 of 58 (93.1%) followed for at least 6 months and 51 of 58 (87.9%) for 12 months. Monthly countable seizure frequency (MCSF) increased with age (median [minimum–maximum] = 1.0 in the youngest [1.0–70.0] and middle [1.0–242.0] age groups and 4.5 [.0–2647.0] in the oldest age group), and remained high, despite use of currently approved antiseizure medications. Language/communication delays were observed early, and developmental stagnation occurred after age 2 years with both instruments. In predictive modeling, chronologic age was the only significant covariate of seizure frequency (effect size = .52, p = .024). MCSF, number of antiseizure medications, age at first seizure, and convulsive status epilepticus were not predictors of language/communication raw scores. Significance: In infants and young children with SCN1A+ DS, language/communication delay and stagnation were independent of seizure burden. Our findings emphasize that the optimal therapeutic window to prevent language/communication delay is before 3 years of age

    Indentation failure of foam cored sandwich structure subjected to elevated temperatures

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    This research explores the detrimental effect of elevated temperatures on local indentation failure of polymer foam cored sandwich structures with laminated glass fibre reinforced epoxy face sheets. A simple analytical model to predict the critical indentation failure load at elevated temperatures is presented and validated against experimental observations. For this purpose, a sandwich beam loaded in three-point bending with an induced through-thickness elevated temperature profile,was studied experimentally and by means of analytical and finite element models. In the experiment, the through-thickness temperature gradient was induced with an infrared lamp pointing on the top face sheet, while the local displacement and strain fields near the applied point load were recorded by digital image correlation. The analytical model proposed, which accounts for temperature degraded/reduced foam core properties superimposes the local response, approximated by the classical Winkler foundation model, and the global response, obtained by sandwich beam theory. The comparative study has shown that the critical load causing core crushing failure reduces significantly with elevated temperatures by as much as 50 % at an elevated temperature of 90 °C. It is shown that the simple analytical model can predict the local deflections and core stresses of foam cored composite sandwich structures subjected to simultaneous localised mechanical loading and elevated temperatures. Thus, the analytical model can be used as a preliminary design tool to determine the critical core crushing load at elevated temperatures
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