Построение 3D-модели зубчатого колеса в приложении GearTeq

The article discusses the process of constructing and simulating the gear in the universal CAD application GearTeq. This application allows you tocreate 3D models of cog wheels almost any gear. An overview of such systems is made and the relevance and ease of use of this application is shown. The main objects that can be processed by the GearTeq system are listed. The interface, process of work and transfer of application information to the software complex SolidWorks is shown. Additional features of the application have been analyzed. The main features of the program are given. The mechanism of modeling of a profile of a tooth of a cogwheel is told. An example of creating a bevel gear in GearTeq and transferring its data to the CAD system of SolidWorks is considered. The main advantages of using the application in the design are shown and general conclusions on its application are made.В статье рассмотрен процесс построения и моделирование зубчатого колеса в универсальном CAD приложении GearTeq. Данное приложение позволяет создавать 3D модели зубчатых колёс практически любой зубчатой передачи. Сделан обзор подобных систем и показана актуальность и простота использования данного приложения. Перечислены основные объекты, которые может обрабатывать система GearTeq. Показан интерфейс, процесс работы и перенос информации приложения в программный комплекс SolidWorks. Разобраны и проанализированы дополнительные возможности приложения. Приведены основные особенности работы программы. Рассказан механизм моделирования профиля зуба зубчатого колеса. Рассмотрен пример создания конического зубчатого колеса в GearTeq и перенос его данных в CAD систему SolidWorks. Показаны главные преимущества использования приложения при проектировании и сделаны общие выводы по его применению

Mapping the differences in care for 5,000 Spinal Muscular Atrophy patients, a survey of 24 national registries in North America, Australasia and Europe

Spinal muscular atrophy (SMA) is an autosomal recessive genetic disorder characterised by the degeneration of motor neurons and progressive muscle weakness. It is caused by homozygous deletions in the survival motor neuron gene on chromosome 5. SMA shows a wide range of clinical severity, with SMA type I patients often dying before 2\ua0years of age, whereas type III patients experience less severe clinical manifestations and can have a normal life span. Here, we describe the design, setup and utilisation of the TREAT-NMD national SMA patient registries characterised by a small, but fully standardised set of registry items and by genetic confirmation in all patients. We analyse a selection of clinical items from the SMA registries in order to provide a snapshot of the clinical data stratified by SMA subtype, and compare these results with published recommendations on standards of care. Our study included 5,068 SMA patients in 25 countries. A total of 615 patients were ventilated, either invasively (178) or non-invasively (437), 439 received tube feeding and 455 had had scoliosis surgery. Some of these interventions were not available to patients in all countries, but differences were also noted among high-income countries with comparable wealth and health care systems. This study provides the basis for further research, such as quality of life in ventilated SMA patients, and will inform clinical trial planning