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Objective: To describe the development and initial experience of a clinical research program in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) in Canada: The Rossy PSP Centre, to share the data acquisition tools adopted, and to report preliminary results. Methods: Extensive demographic and longitudinal clinical information is collected every 6 months using standardized forms. Biofluids are collected for biobanking and genetic analysis, and many patients are enrolled in neuroimaging research protocols. Brain donation is an important component of the program, and standardized processing protocols have been established, including very short death to autopsy times in patients undergoing medical assistance in dying. Results: Between Oct 2019 and Dec 2021, 132 patients were screened, 91 fulfilling criteria for PSP and 19 for CBS; age 71 years; 41% female; duration 5 years, age-of-onset 66 years. The most common symptoms at onset were postural instability and falls (45%), cognitive-behavioral changes (22%), and Parkinsonism (9%). The predominant clinical phenotype was Richardson syndrome (82%). Levodopa and amantadine resulted in partial and short-lasting benefit. Conclusions: The Rossy PSP Centre has been established to advance clinical and basic research in PSP and related tauopathies. The extent of the clinical data collected permits deep phenotyping of patients and allows for future clinical and basic research. Preliminary results showed expected distribution of phenotypes, demographics, and response to symptomatic treatments in our cohort. Longitudinal data will provide insight into the early diagnosis and management of PSP. Future steps include enrollment of patients in earlier stages, development of biomarkers, and fast-tracking well-characterized patients into clinical trials.

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Background As compared to term neonates, those < 35 weeks gestation (wks GA) are at greater risk for both acute and chronic bilirubin encephalopathy (ABE, CBE). Among these with postnatal total bilirubin rate of rise, (TB ROR) at age 0 to 72 hours has been observed because of either loss placental elimination system or increased postnatal production due to hemolysis. The ranges are known to vary > 8.5 umol/L/h in neonates with Rh disease to >3.4 umol/L/h at the 95th percentile track of the hour-specific nomogram. TB ROR in healthy term neonates is <3.4 umol/L/h. Objectives To determine the GA ranges on TB ROR to explore predictive TB ROR in preterm neonates who are more vulnerable with each <35 wks GA. Design/Methods A multi-site observational study to quantify TB ROR in preterm infants between 28 to 35 weeks. 1804 infants born between January 2013- March 2018 at 28–35 wks GA from three canadian perinatal centres were included and those with Rh disease were excluded. Analysis included infants >27 weeks with at least one TB prior to the initiation of treatment for severe hyperbilirubinemia treatment. Feeding patterns, birth history and maternal health conditions were documented. Results The TB ROR were compared by two prematurity GA groups (28–31 weeks and 32–35 weeks) then per individual gestational age in four time periods in hours, 0–24, 24–48, 48–96, and 96 -120 in 1049 preterm infants using 3065 TB samples. Infants <28 weeks GA were excluded since they represented a more diverse population. TB ROR by prematurity groups over all was higher in 32–35 weeks group at 0–12 hours (3.85 umol/L/h) and 25–36 hours (2.81umol/L/h) and decreased at 49–72 hours (0.24umol/L/h) time period as compared to the less mature group. Conclusion Though TB ROR were of similar patterns between prematurity groups (28–31 weeks and 32–35 weeks) it was at higher rate of rise between 13–36 hours and decreasing from 36–72 hours, with a plateau after 72 hours of age. There was a significant difference in the magnitude of TB ROR between prematurity groups at 0–24 hours. Additional research into the clinical care impact on the TB ROR should be conducted to study impact of production and elimination.