Global Outcome Assessment Life-long after stroke in young adults initiative-the GOAL initiative : study protocol and rationale of a multicentre retrospective individual patient data meta-analysis

Introduction Worldwide, 2 million patients aged 18-50 years suffer a stroke each year, and this number is increasing. Knowledge about global distribution of risk factors and aetiologies, and information about prognosis and optimal secondary prevention in young stroke patients are limited. This limits evidence-based treatment and hampers the provision of appropriate information regarding the causes of stroke, risk factors and prognosis of young stroke patients. Methods and analysis The Global Outcome Assessment Life-long after stroke in young adults (GOAL) initiative aims to perform a global individual patient data meta-analysis with existing data from young stroke cohorts worldwide. All patients aged 18-50 years with ischaemic stroke or intracerebral haemorrhage will be included. Outcomes will be the distribution of stroke aetiology and (vascular) risk factors, functional outcome after stroke, risk of recurrent vascular events and death and finally the use of secondary prevention. Subgroup analyses will be made based on age, gender, aetiology, ethnicity and climate of residence.Peer reviewe

Performance of the infrared array camera (IRAC) for SIRTF during instrument integration and test

The Infrared Array Camera (IRAC) is one of three focal plane instruments in the Space Infrared Telescope Facility (SIRTF). IRAC is a four-channel camera that obtains simultaneous images at 3.6, 4.5, 5.8, and 8 microns. Two adjacent 5.12x5.12 arcmin fields of view in the SIRTF focal plane are viewed by the four channels in pairs (3.6 and 5.8 microns; 4.5 and 8 microns). All four detector arrays in the camera are 256x256 pixels in size, with the two shorter wavelength channels using InSb and the two longer wavelength channels using Si:As IBC detectors. We describe here the results of the instrument functional and calibration tests completed at Ball Aerospace during the integration with the cryogenic telescope assembly, and provide updated estimates of the in-flight sensitivity and performance of IRAC in SIRTF

Case Reports1. A Late Presentation of Loeys-Dietz Syndrome: Beware of TGFβ Receptor Mutations in Benign Joint Hypermobility

Background: Thoracic aortic aneurysms (TAA) and dissections are not uncommon causes of sudden death in young adults. Loeys-Dietz syndrome (LDS) is a rare, recently described, autosomal dominant, connective tissue disease characterized by aggressive arterial aneurysms, resulting from mutations in the transforming growth factor beta (TGFβ) receptor genes TGFBR1 and TGFBR2. Mean age at death is 26.1 years, most often due to aortic dissection. We report an unusually late presentation of LDS, diagnosed following elective surgery in a female with a long history of joint hypermobility. Methods: A 51-year-old Caucasian lady complained of chest pain and headache following a dural leak from spinal anaesthesia for an elective ankle arthroscopy. CT scan and echocardiography demonstrated a dilated aortic root and significant aortic regurgitation. MRA demonstrated aortic tortuosity, an infrarenal aortic aneurysm and aneurysms in the left renal and right internal mammary arteries. She underwent aortic root repair and aortic valve replacement. She had a background of long-standing joint pains secondary to hypermobility, easy bruising, unusual fracture susceptibility and mild bronchiectasis. She had one healthy child age 32, after which she suffered a uterine prolapse. Examination revealed mild Marfanoid features. Uvula, skin and ophthalmological examination was normal. Results: Fibrillin-1 testing for Marfan syndrome (MFS) was negative. Detection of a c.1270G > C (p.Gly424Arg) TGFBR2 mutation confirmed the diagnosis of LDS. Losartan was started for vascular protection. Conclusions: LDS is a severe inherited vasculopathy that usually presents in childhood. It is characterized by aortic root dilatation and ascending aneurysms. There is a higher risk of aortic dissection compared with MFS. Clinical features overlap with MFS and Ehlers Danlos syndrome Type IV, but differentiating dysmorphogenic features include ocular hypertelorism, bifid uvula and cleft palate. Echocardiography and MRA or CT scanning from head to pelvis is recommended to establish the extent of vascular involvement. Management involves early surgical intervention, including early valve-sparing aortic root replacement, genetic counselling and close monitoring in pregnancy. Despite being caused by loss of function mutations in either TGFβ receptor, paradoxical activation of TGFβ signalling is seen, suggesting that TGFβ antagonism may confer disease modifying effects similar to those observed in MFS. TGFβ antagonism can be achieved with angiotensin antagonists, such as Losartan, which is able to delay aortic aneurysm development in preclinical models and in patients with MFS. Our case emphasizes the importance of timely recognition of vasculopathy syndromes in patients with hypermobility and the need for early surgical intervention. It also highlights their heterogeneity and the potential for late presentation. Disclosures: The authors have declared no conflicts of interes

Navigating the Problem Space of Academic Work

Despite an increasing focus on the quality of teaching in postsecondary institutions, little research exists that examines how faculty actually plan their courses in real-world settings. In this study the idea of the “problem space” from cognitive science is used to examine how faculty construct mental representations for the task of planning undergraduate courses. Using data from free lists and retrospective interviews, I report the factors that most shape the planning space and subsequent strategies and curricular artifacts used by a group of 58 faculty. Results indicate the primacy of fixed affordances, such as workload constraints, course content, and class size, and that these constraints contribute to the routine maintenance of preexisting lecture notes and PowerPoint slides. I recommend that educational leaders consider these cultural practices when designing instructional reforms and enact policies that require faculty to engage in brief, postclass reflection that results in minor updates to these artifacts

Developing skills in higher education for post-pandemic work

Many white-collar workplaces have changed substantially because of the pandemic. This paper examines how the rise of technologybased distributed work arrangements impacts on the knowledge, skills and competencies that higher education graduates must possess to be successful in post-pandemic white-collar work environments. In addition, this paper examines prior literature to identify digital forms of Work Integrated Learning as a mechanism through which higher education providers can assist graduates in their postpandemic transition from university to the workplace

Performance of the Infrared Array Camera (IRAC) for SIRTF during Instrument Integration and Test

The Infrared Array Camera (IRAC) is one of three focal plane instruments in the Space Infrared Telescope Facility (SIRTF). IRAC is a four-channel camera that obtains simultaneous images at 3.6, 4.5, 5.8, and 8 microns. Two adjacent 5.125.12 arcmin fields of view in the SIRTF focal plane are viewed by the four channels in pairs (3.6 and 5.8 microns; 4.5 and 8 microns). All four detector arrays in the camera are 256256 pixels in size, with the two shorter wavelength channels using InSb and the two longer wavelength channels using Si:As IBC detectors. We describe here the results of the instrument functional and calibration tests completed at Ball Aerospace during the integration with the cryogenic telescope assembly, and provide updated estimates of the in-flight sensitivity and performance of IRAC in SIRTF