82 research outputs found

    Improved upper limb function in non-ambulant children with SMA type 2 and 3 during nusinersen treatment: a prospective 3-years SMArtCARE registry study

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    Background The development and approval of disease modifying treatments have dramatically changed disease progression in patients with spinal muscular atrophy (SMA). Nusinersen was approved in Europe in 2017 for the treatment of SMA patients irrespective of age and disease severity. Most data on therapeutic efficacy are available for the infantile-onset SMA. For patients with SMA type 2 and type 3, there is still a lack of sufficient evidence and long-term experience for nusinersen treatment. Here, we report data from the SMArtCARE registry of non-ambulant children with SMA type 2 and typen 3 under nusinersen treatment with a follow-up period of up to 38 months. Methods SMArtCARE is a disease-specific registry with data on patients with SMA irrespective of age, treatment regime or disease severity. Data are collected during routine patient visits as real-world outcome data. This analysis included all non-ambulant patients with SMA type 2 or 3 below 18 years of age before initiation of treatment. Primary outcomes were changes in motor function evaluated with the Hammersmith Functional Motor Scale Expanded (HFMSE) and the Revised Upper Limb Module (RULM). Results Data from 256 non-ambulant, pediatric patients with SMA were included in the data analysis. Improvements in motor function were more prominent in upper limb: 32.4% of patients experienced clinically meaningful improvements in RULM and 24.6% in HFMSE. 8.6% of patients gained a new motor milestone, whereas no motor milestones were lost. Only 4.3% of patients showed a clinically meaningful worsening in HFMSE and 1.2% in RULM score. Conclusion Our results demonstrate clinically meaningful improvements or stabilization of disease progression in non-ambulant, pediatric patients with SMA under nusinersen treatment. Changes were most evident in upper limb function and were observed continuously over the follow-up period. Our data confirm clinical trial data, while providing longer follow-up, an increased number of treated patients, and a wider range of age and disease severity

    Friderici Godoholdi Schoenii De personarum in Euripidis Bacchabus habitu scenico commentatio

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    FRIDERICI GODOHOLDI SCHOENII DE PERSONARUM IN EURIPIDIS BACCHABUS HABITU SCENICO COMMENTATIO Friderici Godoholdi Schoenii De personarum in Euripidis Bacchabus habitu scenico commentatio ( - ) Einband ( - ) Titelseite ([1]r) Caput I. Praefatio. ([1]) Caput II. De Bacchi persona. (8) Caput III. De relicuo Bacchi ornatu. (20) Caput IV. De Penthei persona et habitu. (35) Caput V. De Agaua (46) Caput VI. De Cudmo et Tiresia. (49) Caput VII. De nuntiorum et famulantium personis. (60) Caput VIII. De choricarum personarum partibus et numero. (67) Caput IX. De apparatu bacchico in universum deque instituenda hac disputatione. (76) Caput X. De ea apparatus parte, quae ad corporis ornatum cultumque pertinet. (78) Caput XI. De instrumentis bacchantium musicis. (121) Caput XII. De Baccharum apud Euripidem habitu scenico. (130) Index scriptorum. (157) Index rerum. (159) Corrigenda et addenda. (165

    Modelling of the preheating process of filament tows

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    Filament winding of powder impregnated fibres has been applied to produce composite rings. Important steps in the process are the preheating of the tow within an infrared (IR) preheater. The paper provides results of modelling the heating process of the tow within the preheater. The calculations are focused on the evaluation of the temperature not only at the surface of the tow but also across the whole diameter, i.e. the skin-core effect is investigated
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