Poster Day - Session #8 by BC Children's Hospital Research

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Session #8: Clinical Dilpreet Bharaj Tereza Blahova Tara Gholamian Jasleen Grewal Kaitlyn Kwok

Nikita Menon Ashleigh Nazareth Sumara Stroshein Stephanie U Adrian Haasler

Judge: Kaie Rosborough

Dilpreet Bharaj

#72

Undergraduate Student, University of British Columbia Supervisor: Theodore Steiner, Childhood Diseases A study to detect and measure food sensitivities in children and adolescents with Eosinophilic Esophagitis (EoE) or Food Protein Induced Enterocolitis (FPIES)

Abstract & Poster - https://bcchr.ca/posterday

A study to detect and measure food sensitivities in children and adolescents with Eosinophilic Esophagitis (EoE) and Food Protein Induced Enterocolitis (FPIES) Dilpreet Bharaj1,2, Laura Oliveira1, Chad Poloni2, Stacy Wang2, Theodore Steiner1,2 (1) Division of Infectious Diseases, UBC Faculty of Medicine (2) BC Children’s Hospital Research Institute

Background

Methods

Eosinophilic esophagitis (EoE) and food protein induced enterocolitis syndrome (FPIES) are diseases resulting from repeated allergic reactions to food.1,2 Activated food antigenspecific T-lymphocytes drive these chronic disease states, as they provide immunologic memory and recognize various food peptides.1,2 The allergic reactions result in painful chronic inflammation of the esophagus and/or intestines, resulting in tissue pathology.1,2

This is a single-center pilot observational study. 20 children and adolescents with EoE (aged 7-18 years) or FPIES (from birth to 18 years) will be recruited using referrals from the Allergy Clinic at the BC Children’s Hospital. 10 control patients without food allergies or confirmed EoE/FPIES will be recruited in parallel.

Therefore, diagnosing what foods trigger EoE/FPIES is important. However, there are currently no clinical tests that accurately identify which specific food-allergen triggers the reaction. Common tests that are used for other allergic diseases, including positive serum food-specific antibody levels, food skin prick test responses, and food patch tests alone, are not sufficient to diagnose food triggers for EoE/FPIES.1 Additionally, testing used to assess the inflammation and confirm an EoE/FPIES diagnosis includes invasive endoscopy.

Objective To assess the sensitivity and specificity of a novel assay, the activation induced marker (AIM) assay, in measuring T cell responses, of patients with EoE/FPIES, to specific food-antigens.

One 4mL blood sample will be collected from participants and used to perform the AIM assay. The AIM assay measures low-frequency allergen-specific CD4+ and CD8+ T-lymphocytes by quantifying activation-induced upregulation of co-expressed CD25 and OX40 or CD69 and CD137 markers, respectively, using flow cytometry.3 The following food antigens will be tested: milk, wheat, egg, and soy, depending on what food-allergens are suspected triggers for each participant.

Clinical data including demographics, medical and allergy history, and a food consumption diary for 7 days prior to blood sample collection will be collected.

Expected Results Patients with EoE/FPIES are expected to have measurable populations of CD4+ T cells using the AIM assay when stimulated with suspected or known food-allergens, compared to controls.

Patients who have out grown their allergic reactions to specific food-allergens may or may not have significant T cell responses, compared to controls. If a significant T cell response is not seen in these patients, this may indicate that foods that were previously triggers can be reintroduced in the diet. If a significant T cell response is seen, however, this may indicate that other immune mechanisms are in play to suppress the allergic reaction.

Implications Since this is a pilot study, results from this study will be able to inform research protocols and techniques for larger future studies. The ultimate aim of the study is the clinical implementation of the AIM assay as a diagnostic tool for EoE/FPIES.

References 1. Groetch, M., C. Venter, I. Skypala, et al.Dietary therapy and nutrition management of eosinophilic esophagitis: a work group report of the American Academy of Allergy, Asthma, and Immunology J Allergy Clin Immunol Pract, 5 (2) (2017), pp. 312-324. 2. Nowak-Wegrzyn, A. et al. International consensus guidelines for the diagnosis and management of food protein-induced enterocolitis syndrome: Executive summary-Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology. J. Allergy Clin. Immunol. 139, 1111-1126.e4 (2017). 3. Zaunders JJ, Munier ML, Seddiki N, Pett S, Ip S, Bailey M, et al. High levels of human antigen-specific CD4+ T cells in peripheral blood revealed by stimulated coexpression of CD25 and CD134 (OX40). J Immunol. 2009;183(4):282736.

Acknowledgements •

• •

Co-investigators: o Edmond S. Chan, MD, FRCPC o Vishal Avinashi, MD, MPH, BSc o Raymond Mak, MD, FRCPC Steiner Research Team BC Children’s Hospital Research Institute Summer Studentship

Tereza Blahova

#73

Undergraduate Student, University of Toronto Supervisors: Gabriella Horvath & Osman Ipsiroglu, Brain, Behaviour & Development The Interconnection of Sleep Disturbances in Rett Syndrome: A Scoping Review

Abstract & Poster - https://bcchr.ca/posterday

The Interconnection of Rett Syndrome and Sleep Disturbances: a Scoping Review Tereza Blahova¹, Scout McWilliams¹, Prubjot Gill , Anita Datta , Gabriella Horvath , Osman Ipsiroglu 2

1,3

¹ H-Behaviours Research Lab, BC Children’s Hospital Research Institute, UBC, Vancouver, Canada 2 Pediatric Library, UBC Vancouver, Canada 3 Department of Pediatrics, BC Children’s Hospital, UBC, Vancouver, Canada

Results

Outcome 2

Sleep

Total

Pharm

Non-Pharm

Both

PSG n=3 EEG n=2 Parent Report n=2 Questionnaire n=2

50 40 30

Secondary Outcome

PSG n=2 EEG n=4 Parent Report n=1 Questionnaire n=1

changes in EEG

Breathing Irregularities

Age as a Determinant of Sleep Disturbances (n=11) Age Group 0-7 years (%) >18 years (%) Night Seizure Daytime Naps

Study Selection

Awakenings

526 studies screened

Insomnias & Parasomnias Bruxism

2 reviewers

Both

Adverse Events

Primary Outcome

Protocol

Scoping review methodology, search strategy and data collection sheet developed in collaboration with librarian. 3

by epileptiform

Rett Syndrome

Sleep

Sleep Outcomes

Common Sleep Disturbances

Insomnias & Parasomnias

Population

Treatment n=16

Sleep Disturbances

*Characterized

Search Strategy

Rett

(PSG, EEG, video)

Movement Disorders

Sleep Assessments = 23

Review n= 8

Paroxysm.

Methods

Clinical Assessments=44

Seizure*

Rett syndrome (RS) is a rare X-linked neurodevelopmental disorder, affecting almost exclusively female patients, characterized by a decline in cognitive and motor skills, slowed growth, breathing irregularities, and sleep disturbances (SD). RS is characterized by epileptiform changes captured in EEGs, therefore epilepsy is the priority for treatment. SDs are prevalent in 80% of patients and result in significant burden to the health of the patient and their family. We are investigating the role of SD in RS patients and conducting a scoping review to address the following research questions: (1) what tools were used to assess sleep as an outcome (2) the effects on sleep of interventions used to treat symptoms or SDs.

Treatment Modality 91 Studies

Number of Participants (Total n=101)

Introduction

100

Less frequent

More frequent

100

Intervention Psychotropics n=5

Adverse Drug Effect Sleep Rett

Antiepileptics n=3 Melatonin n=3 Immuno. n=1 Oligopeptide n=1 Opiate anta. n=1 n=# studies reporting TEAE

Conclusion Sleep disturbances are extremely common in patients with Rett syndrome and are more prevalent in younger populations. The clinical severity of Rett syndrome can be diagnosed with the help of an EEG instrument as each clinical stage is characterized by changes in epileptiform activity. Many common medications used to treat symptoms of Rett syndrome, such as antiepileptics and antidepressants, have the potential to negatively affect sleep architecture, and therefore must be integrated as an outcome measure in all treatment studies.

#74 Tara Gholamian

Medical Student, University of Ottawa Supervisor: Anthony Cooper, Evidence to Innovation Morquio B Disease: A Case Report

Abstract & Poster - https://bcchr.ca/posterday

Morquio B Disease: A Case Report Gholamian, T. (1), Chhina, H.(3), Stockler, S.(4,5,6), Cooper, A.(2,3,4). (1)University of Ottawa Medical School, Ottawa, ON, Canada; (2)Department of Orthopaedics, University of British Columbia, Vancouver, BC; (3) Department of Orthopaedic Surgery, BC Children’s Hospital, Vancouver, BC; (4) Investigator, BC Children’s Hospital Research Institute, Vancouver, BC; (5) Department of Biochemical Diseases, University of British Columbia, Vancouver, BC; (6) Department of Biochemical Diseases, BC Children’s Hospital, Vancouver, BC

Background

Diagnosis

• Mucopolysaccharidosis IV type B, or Morquio B Disease (MBD), is a rare autosomal recessive lysosomal storage disease, with a worldwide prevelance of less than 70 patients • MBD caused by a mutation in the gene that codes for β-galactosidase, GLB1, on chromosome 3p22.33 • β-galactosidases are a family of glycosidase hydrolase enzymes that break down various βgalactosides such as and keratan sulfate • Deficiency in the enzymatic activity of β-galactosidases causes abnormal accumulation of glycosaminoglycans such as keratan in the cornea, urine, and cartilage where it causes abnormal endochondral ossification and bone growth • The pathology of MBD includes severe skeletal manifestations (dysostosis multiplex) without storage in neuronal tissues and central nervous system involvement • The dysostosis multiplex of MBD patients appears with a disproportionate short stature, atlanto-axial instability, odontoid hypoplasia, platyspondyly, kyphoscoliosis, pectus carinatum, coxa valga, and genu valgum • Other characteristics of disease include lax joints, trigger fingers, corneal clouding, sensorineural hearing loss, dental abnormalities, and cardiopulmonary complications • While life expectancy is not reduced, quality of life is severely impacted through joint pain, limitations in mobility, and excessive keratan sulfate storage • Despite the devastating health consequences and lowered quality of life in such patients, there are very few studies reporting on the manifestations, natural history, and treatments that patients with MBD receive

• The diagnosis of MBD was made at 5 years of age after the patient was seen by physiotherapy in 2009 due to weak lower extremities and gait concerns • Physical features suggestive of MBD were seen that included: short stature, normal skull shape, a barrel-shaped chest, prominent sternum (pectus carinatum), short neck and trunk, longer limbs, hyperextensible joints, thoracic kyphosis, and a mild scoliosis with a right rib hump • Radiographic features included flat vertebrae, a hypoplastic odontoid with a stable relationship to C1-C2, thoracic kyphosis, lumbar hyperlordosis, proximal tapering and shortened metacarpal bones, small and irregular carpal and tarsal bones, platyspondyly, hip dysplasia, and a flared lower coastal margin • Lab tests at diagnosis included: • Elevated mucopolysaccharides in the patient’s urine 18 mg/mmol creatinine (normal <15) • Abnormal band pattern of urine oligosaccharides on gel electrophoresis • Reduced activity of β-galactosidase in his white blood cells at 12. 3nmol/hr/mg protein (normal 78-363) in an enzyme assay • Molecular testing confirmed the patient to be compound heterozygous for 2 pathological mutations in GBL1 gene (W273L/N484K)

Aims

Spine Abnormalities • Followed up with radiographs (yearly) and MRI (every 2-3 years) • Stable spine with no pain or discomfort • Kyphosis at thoracolumbar junction with anterior subluxation of T12 to L1 • Narrowing of the spinal canal diameter at all levels, with diffuse lumbar spinal stenosis due to prominent disc annuli with narrow exit foramina of all lumbar nerve roots • Hypoplastic dens present, but patient has full neck range of motion in neutral, flexion, and extension • No instability demonstrated radiographically or clinically but monitored for C1-C2 subluxation

Orthopedic Concerns Bilateral Ankle Valgus • Jan 2015 – Bilateral ankle valgus deformities measured 32 degrees bilaterally • April 2015 – The patient underwent bilateral screw epiphysiodesis of the distal tibia to correct the deformity, prevent further bone deformation and cartilage damage in the talar dome

LAT Cervical Extension (Aug-2020)

LAT Cervical Flexion (Aug-2020)

LAT Spine (Aug-2020)

1. Increase awareness about rare biochemical diseases such as MBD

Conclusion • There are a wide range of skeletal and non-skeletal manifestations of MBD • This case report illustrates the complex orthopedic challenges and treatments that a patient with MBD underwent from their diagnosis to late adolescence • An increasing number of case reports are needed to better understand the natural history of MBD and the various orthopedic treatment options available to improve the care received by these patients • Once such data is available, comparative studies of the care received by patients with this rare condition can be undertaken to determine the best treatment options for MBD patients to reduce pain, decrease burden of disease, and improve quality of life

2. Outline all aspects of care that a patient with MBD received to offer clinical guidance for the treatment and follow-up of MBD patients

3. Improve the care received by MBD patients by providing the first case report outlining the in-depth orthopedic treatment a patient with MBD has received in their childhood and adolescence

Birth & Infancy • Caucasian male who was born to non-consanguineous parents at 361/2 weeks via caesarean section • Born following a normal and healthy pregnancy accompanied with normal ultrasounds at prenatal care • He was crawling by 9 months and could walk unsupported by 15 months • Experienced difficulty feeding but was otherwise well with no hospital admissions or significant illnesses

AP Bilateral Lower Extremities (Jan 2015)

AP Bilateral Lower Extremities (June 2015)

AP Bilateral Lower Extremities (March 2021)

Bilateral Genu Valgum • Nov 2015 – Bilateral genu valgum deformity measured 16 degrees right and 19 degrees left • Dec 2015 – The patient underwent bilateral distal femoral and proximal tibia medial 8-plate application, and 1 month post surgery was walking independently with no pain • Feb 2021 – All hardware removed • Denied pain, had full ROM to knees, but minimal walking tolerance (100-150m)

Other Medical Concerns • Ophthalmology – Mild corneal clouding that is clinically insignificant in this patient and has been stable • Respirology – Regular pulmonary function tests (PFTs) have been normal • Audiology Clinic – Hearing Stable • No cognitive deficits with normal nerve conduction studies • Cardiology – A soft systolic ejection murmur is present at the mid left sternal border along with a mitral regurgitation murmur at the apex. There was increased mitral and aortic valve thickness, but no obstruction to flow in either valve • Patient reported not being physically restricted by his cardiac function, but performing at a lower level than his peers physically due to MSK limitations and fatigue

References 1. Sohn Y, Park H, Park S, Kim S, Cho S, Ko A, Ki C, Yeau S, Jin D. A Korean Patient with Morquio B Disease with a Novel c.13_14insA Mutation in the GLB1 gene. Ann Clin Lab Sci [Internet]. Winter 2012 [cited 2021 June 07]; 42 (1):89-93. Available from: http://www.annclinlabsci.org/content/42/1/89.long 2. Ramphul K, Mejias S, Ramphul-Sicharam Y. Morquio Syndrome: A Case Report. Cureus [internet]. March 2018 [cited 2021 June 07]; 10 (3):e2270. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5935432/ doi: 10.7759/cureus.2270 3. Gupta S, Sengar K, Subramanian A, Satyarthee G. Morquio Syndrome Presenting with Dural Band Pathology: A Case Report. J Lab Phys [internet]. 2020 [cited 2021 June 07]; 12 (04):285-288. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7773441/ doi: 10.1055/s-0040-1722548 4. Sheth JJ, Sheth FJ, Raktima B. Morquio-B Syndrome (MPS-IV B) associated with Bgalactosidase Deficiency in Two Siblings. Indian J Pediatr [internet]. 2002 [cited 2021 June 07]; 69 (1):109-11.Available from: https://pubmed.ncbi.nlm.nih.gov/11876111/ doi: 10.1007/BF02723790

Acknowledgements • Walk Tall Research Team • Stockler Research Team • BCCH Department of Orthopedics BCCH Department of Biochemical Diseases • BC Children's Hospital Research Institute Summer • BC Children’s Summer Research Education Program AP Bilateral Lower Extremities (Nov 2015)

AP Bilateral Lower Extremities (April 2017)

AP Bilateral Lower Extremities (March 2021)

Jasleen Grewal

#75

Undergraduate Student, Queen's University Supervisor: Ian Pike, Evidence to Innovation Indigenous Driver Training; Aiming to Bridge the Injury Gap between Indigenous and non-Indigenous Populations in BC

Abstract & Poster - https://bcchr.ca/posterday

Indigenous Driver Training: Aiming to Bridge the Injury Gap between Indigenous and non-Indigenous Populations in BC Jasleen Grewal1, Kate Turcotte2, Ian Pike2,3 (1) Faculty of Health Sciences, Queen’s University, Kingston, Canada; (2) BC Injury Research and Prevention Unit, BC Children’s Hospital, Vancouver, Canada; (3) Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada

Background • Worldwide, Aboriginal populations are disproportionately represented in transport-related injury rates.4 • In BC, Indigenous communities living in rural areas face over twice the risk of injury related to transport than the general population.4,5 • Geographical and systemic barriers to driver training and licensing services exist for Indigenous people living in rural and remote communities. • Limited research has been conducted regarding driver licenses as a social determinant of health in BC.

Program: All Nations Driving Academy (ANDA) In 2018, Lucy Sager founded ANDA, a for-profit organization, in Terrace BC as an initiative to help rural Indigenous communities travel to work.

Driver Licenses as a Social Determinant of Health

Delivery • Communities hire ANDA to visit their communities and teach driver training for students to receive their class 7L, 7N, class 5, and class 4 unrestricted licenses. • The program was funded by the Ministry of Advanced Education from 2019-2020. Evaluation Primary evaluation metrics are based on licenses produced from classes and impact to students’ quality of life. Over 200 licensed drivers have been produced.

Further research and distribution of the program are expected to be key steps in reducing injury and mortality rates for rural Indigenous communities. Figure 2) Benefits of Licensing-related mobility in Australian Aboriginal Populations 6

To develop a case study evaluating Indigenous Driver Training Schools as injury prevention initiatives in BC.

Driver’s licenses also provide empowerment, ID, and promote community engagement. 6

Photo Credit: Lucy Sager

Objective

Methods Key informant interviews were conducted with experts in Indigenous driver licensing and training in BC and Australia. A review of publications relevant to BC road safety data and Indigenous driver licensing in BC and Australia was conducted.

Figure Interpretation Motor vehicle crashes (MVCs) remain the leading cause of injuries and deaths in Indigenous children and youth.5

Based on expert opinion and pilot studies in Australia, culturally responsive driver training initiatives show promise for decreasing injury rates for Indigenous road users. Individuals and communities have reported increased access to healthcare, education, and employment opportunities outside of their rural areas.

Without access to transportation for groceries, employment, and medical appointments, Indigenous people see a higher rate of: • diabetes • lack of treatment • poor or non-existent vision, dental, and prenatal care

Figure 1) Primary causes of death and fatality rates among children age 1-4 years between 1992-2006 5

Discussion

Primary Barriers to Licensing 6 • Accessibility challenges in rural testing centers • Vision care • Historical trauma • Challenges to accessing primary ID and secondary ID • Outstanding fines

Photo Credit: Lucy Sager

References (4) George MA, Jin A, Brussoni M, Lalonde CE. Is the injury gap closing between the Aboriginal and general populations of British Columbia? Health Rep. 2015;26(1):3-14. (5) Reducing Motor Vehicle Crashes BC. Accessed July 27, 2021. https://www2.gov.bc.ca/assets/gov/health/about-bc-s-health-care-system/office-of-theprovincial-health-officer/reports-publications/annual-reports/reducing-motor-vehiclecrashes-bc.pdf (6) Cullen P, Clapham K, Hunter K, Porykali B, Ivers R. Driver licensing and health: A social ecological exploration of the impact of licence participation in Australian Aboriginal communities. Journal of Transport & Health. 2017;6:228-236. doi:10.1016/j.jth.2017.07.001 (7) Road to Reconciliation UBCIC. Accessed July 27, 2021. https://d3n8a8pro7vhmx.cloudfront.net/ubcic/pages/1440/attachments/original/1616615 564/3.3.1_2021Feb_RoadtoReconciliation_UBCICFinalCopy.pdf?1616615564

Acknowledgement This project was funded by a BCIRPU SRP Studentship. Thank you to the BCIRPU team and the key informants for their contributions to the case study.

Kaitlyn Kwok

#76

Undergraduate Student, McMaster University Supervisor: Srinivas Murthy, Healthy Starts A Scoping Analysis of Trial Sponsorship and Data Flows of Global Clinical Trials

Abstract & Poster - https://bcchr.ca/posterday

Department of Pediatrics, Faculty of Medicine, University of British Columbia

A Scoping Analysis of Trial Sponsorship and Data Flows of Global Clinical Trials K. KWOK1, N. SATI2, L. DRON2, S. MURTHY3 1McMaster

University, Hamilton, ON 2Cytel: Clinical Trial Software & Data Analysis 3Department of Pediatrics, University of British Columbia, Vancouver, BC

INTRODUCTION Clinical trials frequently recruit across different income-groupings to improve generalizability. However, data is often disproportionately shared between countries wherein it is collected in the Global South yet analyzed and managed in the Global North. Recent movements for more equity in global health research have questioned the status quo.

RESULTS Included Studies & Funding Source

Income Classifications by Country Using World Bank Definitions

AIMS This analysis seeks to document the extent of clinical trial data flow from Global South to Global North in studies published in major journals.

MATERIALS & METHODS PRISMA Flow Diagram Both unidirectional and non-unidirectional data flow analysis demonstrated movement towards high-income countries (HICs) in 198 (72.3%) of the studies for which data analysis site was reported.

Direction of Clinical Trial Data Flow

The location of data management was not reported in 230 (75.4%) of the studies. Of those that did report: • 27 (36.0%) were managed in HICs • 11 (14.6%) were managed in upper-middle-income countries (UMICs) • 16 (21.3%) were managed in lower-middle-income countries (LMICs) • 18 (24.0%) were managed in a mix of income-groupings

CONCLUSIONS Randomized clinical trials from The BMJ, BMJ Global Health, the Journal of the American Medical Association (JAMA), The Lancet, Lancet Global Health, and the New England Journal of Medicine (NEJM) were included if they involved recruitment and author affiliation across different country income-groupings. The direction of data flow was extracted with a data collection tool using sites of trial recruitment as the starting point and the location of authors conducting statistical analysis as the ending point.

Clinical trial data flow continues to demonstrate a trajectory from lower- and middle-income countries towards HICs. Geographically, this coincides with movement from the Global South to the Global North . Current policies and the global health research framework should be re-examined to assess how data can begin to be shared across country income-groupings in a more balanced and equitable model.

REFERENCES 1. 2. 3. 4.

Abimbola, S. and M. Pai (2020). "Will global health survive its decolonisation?" Lancet 396(10263): 1627-1628. Aguilera, B., et al. (2020). "Regulating international clinical research: an ethical framework for policy-makers." BMJ Glob Health 5(5). Lang, T. and S. Siribaddana (2012). "Clinical trials have gone global: is this a good thing?" PLoS Med 9(6): e1001228. Park, J. J. H., et al. (2021). "How COVID-19 has fundamentally changed clinical research in global health." Lancet Glob Health 9(5): e711-e720.

ACKNOWLEDGEMENTS Thank you to Dr. Murthy, Neha, and Louis for all their guidance throughout this process. Summer student funding was provided by the BC Children’s Hospital Research Institute Summer Studentship.

Nikita Menon

#77

Medical Student, University of British Columbia Supervisor: Christine Voss, Evidence to Innovation Exploring the journey of patients with congenital heart disease in the context of physical activity education: A narrative review

Abstract & Poster - https://bcchr.ca/posterday

Exploring the journey of patients with congenital heart disease in the context of physical activity education: A narrative review Nikita

1 Menon ,

Christine

2 Voss

1Vancouver-Fraser

Medical Program, Faculty of Medicine, UBC 2Dept of Pediatrics; Centre for Chronic Disease Prevention and Management, Faculty of Medicine, UBC

BACKGROUND

RESULTS & DISCUSSION

Congenital heart disease (CHD) affects approximately 1 in 100 live births1-4.

Need for PA education

Children with CHD do not engage in enough physical activity (PA) to meet current guidelines, an important factor in optimizing long-term cardiovascular health5. Studies have shown that exercise interventions are safe and beneficial for the CHD population, but evidence regarding the optimal timing and format of strategies to increase PA behaviours is scarce and conflicting4,6,8.

Clear counselling around PA could avoid unnecessary activity restriction and alleviate concerns about safety. There may be a lack of clarity around whose responsibility it is to provide this education. Major Milestones in the Patient’s Journey

Opportunity to begin PA interventions?

OBJECTIVE To explore the patient’s journey with CHD in order to identify the optimal time to begin interventions related to PA.

METHODS A search was conducted on MEDLINE and CINAHL using terms for CHD in combination with patient experience, healthcare interventions and continuity of care.

• Parents and children may be receiving overwhelming amounts of information, have pertinent decisions to consider, and other urgent priorities • PA levels are heavily influenced by the child’s social and academic environment

• Patients with CHD aged 12 and older are encouraged to learn more about their health and take ownership of decisions • PA habits built early can influence adult lifestyles

The results were filtered for being published in English between January 2006 and April 2021. Studies were screened for research being conducted in North America and relating to experiences of patients, their families, and their healthcare team, as well as recommendations for the care of CHD patients. The search resulted in 1861 articles, of which 24 were included in the review1-24.

nikmenon@student.ubc.ca christine.voss@ubc.ca

• Patients are transferred from pediatric to adult care around age 18, but many patients may be lost to follow-up

Results and discussion are based on the 24 articles that were included in the review1-24

CONCLUSION

REFERENCES

There is a need for PA education and the initiation of transition from pediatric to adult care, at approximately age 12, may be an appropriate opportunity to begin PA interventions.

1-24For

Further research is required to determine the optimal type of intervention to increase PA and ultimately improve long-term cardiovascular health in the CHD population.

a full list of references, please see: tinyurl.com/fullreferencelist

Ashleigh Nazareth

#78

Medical Student, University of British Columbia Supervisor: Robert Baird, Evidence to Innovation External Validation of the PRESTO Pediatric Trauma Risk Adjustment Tool

Abstract & Poster - https://bcchr.ca/posterday

External Validation of the PRESTO Pediatric Trauma Risk Adjustment Tool Ashleigh

1 Nazareth ,

Recep

2 Gezer ,

Jaimini

2 Thakore ,

Etienne

3 St-Louis ,

Genevieve

1,4 Ernst ,

Robert

1,4 Baird

1 – UBC Faculty of Medicine, 2 – Trauma Services BC, 3 – Department of Surgery, McGill University, 4 – BC Children’s Hospital Research Institute

Pediatric Trauma

Risk Adjustment

Traumatic injury is a key cause of child mortality.

Risk adjustment tools predict outcomes based on risk factors.

Mortality is largely related to injury management, thus quality improvement (QI) of trauma systems is important.

Predicted and observed outcomes can be compared (benchmarked) to set QI goals.

1. Validate the Pediatric Resuscitation and Trauma Outcome (PRESTO) risk adjustment tool in a high-income setting, such as BC 2. Compare the effectiveness of PRESTO and the current standard, the Injury Severity Score (ISS), for predicting in-hospital mortality 3. Develop an alternate PRESTO, measuring neurological status on the Glasgow Coma Scale (GCS) instead of the Alert-Verbal-PainUnresponsive (AVPU) scale for easy use in high-income settings

PRESTO is a simple pediatric tool developed for resourcelimited settings, validated in lowand-middle income countries.

Pediatric Resuscitation and Trauma Outcome (PRESTO) Tool

Is designed for resource-limited settings,

Objectives 1 and 2

considering only easily-assessed factors

Was recently developed (2018 vs 1974) Equation 1. The PRESTO benchmarking tool.

However, PRESTO requires further validation, specifically in high-income countries.

After successful BC validation, Canada-wide validation will be sought

The effectiveness of PRESTO

Severity Score (ISS), PRESTO: Is pediatric-specific

Elimination of the GCS to AVPU conversion step will improve PRESTO ease of use in high-income settings

Expected Results

Methods

Unlike the current standard, the Injury

PRESTO can benchmark pediatric trauma outcomes in BC, identifying under and overachievers, thereby driving cycles of institutional QI

*Alert-Verbal-Pain-Unresponsive Scale of neurological status

Assess data missingness

Obtain Objective 3 pediatric records (2013-2021) from the BCTR** Adjust PRESTO tool to use GCS instead of AVPU ** BC Trauma Registry

and ISS is assessed by the area

Model the data using PRESTO and ISS tools

Compare PRESTO and ISS tools

underneath the receiver-

Sensitivity

Trauma registries record injury information that can be used for research and QI purposes.

Significance and Future Directions

Objectives

operator curve (Figure 1). PRESTO ISS

PRESTO has a greater area than ISS and is therefore more

Model the data using the new PRESTO (PRESTON) tool

Compare PRESTO and PRESTON tools

effective at predicting in-hospital

1- Specificity Figure 1. Receiver-operator curve comparing PRESTO and ISS. PRESTO has a greater area (p < 0.05) and is more effective.

mortality (Figure 1).

Acknowledgements

Neurological Status in PRESTO

𝜷𝟖 . 𝑮𝑪𝑺

PRESTO measures neurological status according to the Alert-Verbal-PainUnresponsive (AVPU) Scale (Equation 1). AVPU is a simple, 4-level scale commonly utilized in low-and-middle-income countries. In high-income countries, the Glasgow Coma

AVPU

GCS

Alert

Verbal

11-14

(responsive to verbal stimuli)

Painful (responsive to painful stimuli)

Unresponsive

Equation 2. The PRESTON benchmarking tool, utilizing a new coefficient, 𝛽! , and GCS scores instead of AVPU scores. PRESTO PRESTON

After altering the PRESTO equation, the effectiveness of

Kiragu AW, Dunlop SJ, Mwarumba N, Gidado S, Adesina A, Mwachiro M, et al. Pediatric Trauma Care in Low Resource Settings: Challenges, Opportunities, and Solutions. Front Pediatr. 2018 Jun 4;6:155.

1- Specificity

St-Louis E, Bracco D, Hanley J, Razek T, Baird R. Development and validation of a new pediatric resuscitation and trauma outcome (PRESTO) model using the U.S. National Trauma Data Bank. J Pediatr Surg. 2018 Jan;53(1):136–40.

the original and new PRESTO

St-Louis E, Séguin J, Roizblatt D, Deckelbaum DL, Baird R, Razek T. Systematic review and need assessment of pediatric trauma outcome benchmarking tools for low-resource settings. Pediatr Surg Int. 2017 Mar;33(3):299–309.

eliminate a conversion step in high-income countries, such as Canada.

Funding generously provided by the UBC Faculty of Medicine Summer Student Research Program

References 2.

An alternate PRESTO utilizing GCS would than AVPU and scores range from 3-15.

BC Children’s Hospital Research Institute Summer Student Research Program

4-10

Scale (GCS) is more commonly used. GCS involves a more detailed assessment

Dr. Mathias Schede Wendy Song Irena Zivkovic

Sensitivity

Table 1. Conversion between AVPU and GCS scores.

St-Louis E, Hassamal R, Razek T, Baird R, Poenaru D, Hardcastle TC. Validation of the PRESTO score in injured children in a South-African quaternary trauma center. J Pediatr Surg. 2020 Jul;55(7):1245–8.

Partners

Figure 2. The receiver-operator curve of the original and new PRESTO (PRESTON) are not significantly different (p > 0.05)

(PRESTON) is not significantly different (Equation 2, Figure 2).

#79 Sumara Stroshein

Medical Student, University of British Columbia Supervisor: Rachel Murphy Developing a Community Food Security Indicator Framework

Abstract & Poster - https://bcchr.ca/posterday

School of Population and Public Health, Faculty of Medicine, University of British Columbia

Developing a Community Food Security Indicator Framework Sumara Stroshein1, Seri Niimi-Burch2, Sara Kozicky3, Casey Hamilton4, Carmen Kim2, Tamasha Hussein5, Lauren Ebert2, Rachel Murphy6 1. Northern Medical Program, Faculty of Medicine, University of British Columbia, Prince George; 2. Faculty of Land and Food Systems, University of British Columbia, Vancouver; 3. UBC Wellbeing, Vancouver; 4. Campus Health, University of British Columbia Okanagan, Kelowna; 5. School of Nursing, University of British Columbia, Vancouver; 6. School of Population and Public Health, University of British Columbia, Vancouver

Background

Definitions

Next Steps

§ Food insecurity is defined inadequate or insecure access to food due to financial constraints or other resources. § Recent Undergraduate Experience Survey (UES) data showed 37% of students at UBC Vancouver report low to very low food security. § The prevalence of food insecurity is higher among those that experience systemic marginalization including international students, Black, Indigenous, students of colour, and transgender/non-binary students. § The health impacts of food insecurity are significant, including mental health issues and increased risk of chronic conditions. § Community Food Hubs (CFH) which integrate support services, programming, and opportunities for community connection and advocacy, have been proposed as a long-term, sustainable approach to address food insecurity. § Therefore, a student-driven team is undertaking a series of coordinated research projects to create a UBC Community Food Security Hub.

Community-Based Participatory Action Research (CBPAR): CBPAR involves collective, reflective and systematic inquiry where researchers and community members are equal partners with the goals of bringing about social change.

§ Key informant interviews to ensure that the framework is effective for the UBC context.

Community Food Security: ”When all community residents have access to enough safe and nutritious food through a sustainable food system that maximizes community self-reliance and social justice while meeting cultural requirements." (Hamm & Bellows, 2003)

Methods

Progress

The framework development process includes: 1) Drawing on a conceptual model for community food security assessment created by the BC Centre for Disease Control (BCCDC). 2) Utilizing CBPAR community engagement methods to create an effective framework for the UBC context.

§ Completion of a literature review of food security assessment tools. § Development of a three-stage engagement plan. § Creation of a draft framework. § Consultation with BCCDC and UBC staff. § Survey design for initial community consultation.

§ Revisions of draft framework. § Data collection of existing and novel data. § Creation of knowledge translation products to share with UBC leadership, the UBC community and external partners/stakeholders.

Acknowledgements Funding for this project was provided by the UBC Faculty of Medicine’s Summer Student Research Program. The Community Food Hub project is a Campus as a Living Lab (CLL) project funded by a grant from UBC Sustainability.

References BC Centre for Disease Control. Conceptual Framework for Food Security Indicators: Summary Report [Internet]. BC Centre for Disease Control, Population and Public Health; 2019 p. 29. Available from: http://www.bccdc.ca/pop-publichealth/Documents/conceptual-framework-food-security-indicators.pdf Bottorff JL, Hamilton C, Huisken A, Taylor D. Correlates of Food Insecurity Among Undergraduate Students. cjhe [Internet]. 2020 [cited 2021 Jun 9];50(2):15–23.

Rationale

§ Currently, food security at UBC is captured at an individual level with versions of the Household Food Security Survey Module (HFSSM). Developing a food secure campus-community necessitates community-level food security indicators. § Developing a framework and identifying indicators of food security in the UBC community will 1) inform the scope, scale, and needs of the CFH and 2) provide a ‘baseline’ measure of communitylevel food security to enable assessment of the impact of the CFH.

§ Student engagement to centre the perspectives of those affected by food insecurity.

Clement S. Envisioning a Physical Food Hub at UBCO [Internet]. 2020. Available from: https://sustain.ubc.ca/sites/default/files/202008_Envisioning%20a%20Physical%20Food%20Hub_Clement.pdf Cohen B, Andrews M, Kantor L. Community Food Security Assessment Toolkit. Food Assistance & Nutrition Research Program; 2002 Jul p. 166. Hamm MW, Bellows AC. Community Food Security and Nutrition Educators. Journal of Nutrition Education and Behavior [Internet]. 2003 Jan 1 [cited 2021 Jul 23];35(1):37– 43. Kaiser ML. Redefining Food Security in a Community Context: An Exploration of Community Food Security Indicators and Social Worker Roles in Community Food Strategies. Journal of Community Practice [Internet]. 2017 Apr 3 [cited 2021 Jun 14];25(2):213–34.

Draft Framework Structure The four major themes in the framework are: Household Food Insecurity, Food Environment, Food Systems, and Capacity. 17 subthemes and 71 specific indicators have been identified.

Partners

Kozicky S. Measuring Food Insecurity at the University of British Columbia [Internet]. UBC Wellbeing; 2019. Available from: https://sustain.ubc.ca/sites/default/files/Sustainability%20Scholars/2018_Sustainabili ty_Scholars/Reports/201872%20Measuring%20Food%20Insecurity%20at%20UBC%20%20Literature%20Review%20%26%20Best%20Practices_Kozicky.pdf

#80 Stephanie U

Undergraduate Student, Simon Fraser University Supervisor: Osman Ipsiroglu, Brain, Behaviour & Development Sleep and Traumatic Brain Injury: A Scoping Review of Polysomnography Findings

Abstract & Poster - https://bcchr.ca/posterday

Sleep and Traumatic Brain Injury: A Scoping Review of Polysomnography Findings Stephanie U¹, Scout McWilliams¹, Prubjot Gill , Jacqueline Purtzki , Calvin Kuo , Osman Ipsiroglu 2

1,4

1,3

¹ H-Behaviours Research Lab, BC Children’s Hospital Research Institute, UBC, Vancouver, Canada 2 Woodward Library, UBC Vancouver, Canada 3 Department of Pediatrics, BC Children’s Hospital, UBC, Vancouver, Canada 4 School of Biomedical Engineering, Faculty of Science and Faculty of Medicine, UBC, Vancouver, Canada

Methods

Introduction

Traumatic brain injury (TBI) affects sleep architecture, the basic organization of sleep, resulting in cognitive, behavioural, and/or quality of life impairments. Polysomnography (PSG), simultaneously measures neurophysiological sleep parameters including sleep staging, awakenings, and limb movements. We are conducting a scoping review of PSG findings to better understand sleep architecture changes in individuals with TBI. The research questions are (1) what are the main characteristics of PSG recorded sleep in individuals with TBI (2) what are the experienced sleep disturbances and, (3) Which medications were prescribed?

Search Strategy

Population Outcome

TBI Sleep

Databases: Medline, Embase, PsychInfo, CINAHL

Protocol

Study Selection

Scoping review methodology, search strategy and data collection sheet developed in collaboration with librarian

1055 Studies Screened

Results

* One pediatric study, 19 participants aged 10-16.5 years

Conclusion Depending on the experienced sequela, intervention and methodology/design of studies varied. Future quality improvement projects require harmonized intake and outcome measures. Sleep studies and investigations of sleep architecture seems to be an objective therapeutic evaluation tool.

OSA

300

PLMD

No PSG Differences (n=437 participants)

200 100 0 100 200 Number of Participants

SWD

Treatment n=7

300

Hypersomn.

Review n=11

Insomnia

Association n=59

(n=76 participants in 7 studies)

Studies selected for inclusion (n=77)

Movement disorders

233 Excluded

Insomnia/parasomn.

Full Text Screening (n=310)

SWD

465 Excluded

Stage 1 Stage 2 Slow Wave Sleep REM Time in Bed Total Sleep Time Sleep Onset Latency REM Latency Wake After Sleep Onset Sleep Efficiency Arousals Awakenings*

Used For

1000 900 800 700 600 500 400 300 200 100 SDB

Title and Abstract Screening (n=791)

Outcome Decrease Increase

Hypersomnia

264 Duplicates

Common Sleep Disturbances

Number of Participants (n=1822)

Imported n=1055

PSG Changes in Association Studies

Treatment Modality for Sleep/Wake Behaviours

Melatonin

Zopiclone

CBT

Modafinil

Methylpredisone*

Methylphenidate

Phototherapy

0 1

Pramipexole

CPAP

* Early Intervention

#81 Adrian Haasler

Undergraduate Student, McGill University Supervisors: Rajavel Elango & Catherine Brunel-Guitton In-vivo assessment of energy metabolism in metabolic myopathies: Repeatability of the 13C-glucose breath test

Abstract & Poster - https://bcchr.ca/posterday

In-vivo assessment of energy metabolism in metabolic myopathies: Repeatability of the 13C-glucose breath test Adrian Haasler1,2, Betina Rasmussen1,2, Abrar Turki1,2, Kerri Scherbinsky1,2, Shaymaa Shurrab1,3, Rajavel Elango1,2,4, Catherine Brunel-Guitton1,2,3 (1) Dept. of Pediatrics, BC Children’s Hospital, Vancouver, BC, Canada, (2) BC Children’s Hospital Research Institute, Vancouver, Canada, (3) Division of Biochemical Diseases, BC Children’s Hospital, Vancouver, BC, Canada, (4) School of Population and Public Health, UBC, Vancouver, Canada

Background

Metabolic myopathies (MM) are a group of genetic conditions that can affect the production of energy (ATP) in muscles. It is difficult to assess the effectiveness of treatments for patients with MM as ATP production cannot be measured in-vivo and requires invasive methods. Traditionally, substrate oxidation or ATP production are measured by invasive in-vitro methods using tissue specimens taken from biopsies1. One way to indirectly measure ATP production in-vivo is through the measurement of glucose oxidation as this contributes to ATP synthesis. A newer minimally invasive approach, the 13 C-glucose breath test (13C-GBT), utilizes isotopically Dlabelled glucose to measure glucose oxidation2, 3. However, the repeatability of the current method is unknown.

Objectives

To measure glucose oxidation using U-13C-glucose and demonstrate the repeatability (low intra-individual variability, <10% CV) of the 13C-GBT as a proof of principle in healthy adults and children.

Methods

Experimental Design: Healthy adults underwent 13C-GBT protocols on three alternating days. Each study involved an oral dose of 75g glucose along with 75mg U-13Cglucose. Sample Collection: Breath samples were collected in triplicates at baseline, 5 and 0 minutes prior to glucose administration, and every 30 minutes thereafter for 240 minutes. The rate of carbon dioxide production (VCO2) was measured using indirect calorimetry.

Statistical Analyses 13CO

2 enrichment was measured through isotope ratio mass spectrometry (IRMS). The atom percent excess (APE) was calculated by subtracting the baseline 13CO2 enrichment from the breath samples.

The mean rate of 13CO2 oxidation4 (F13CO2) was plotted over time in a line graph for each participant. Standard deviations were added to demonstrate the precision of the method. Subsequently, the area under the curve (AUC) for F13CO2 of each study day was calculated. CV values were determined for each participant to find intra-individual variability (GraphPad PRISM 6).

Results Participant Characteristics (n=3) GR01

GR02

GR03

Age (years)

Sex

Weight (kg)

58.3

59.1

77.0

Height (cm)

160

166

181

BMI (kg/m2)

22.77

21.45

23.49

Fat Free Mass (kg)

40.1

43.1

56.1

Fat mass (%)

31.5

27.8

27.1

Glucose Oxidation

Implications & Future Directions

The experiment will determine whether the 13C-GBT is repeatable in healthy individuals. If proven repeatable, this method can be helpful for monitoring the effectiveness of therapies and drugs for metabolic myopathies such as mitochondrial disorders, fatty acid oxidation defects, glycogen storage disorders, and myoadenylate deaminase (MADA) deficiencies. As a next step, the study will be conducted in ten healthy children (10-14 y). Furthermore, results will provide guidance and can be utilized in subsequent breath tests for patients with metabolic myopathies.

References

[1] Wibom, R., Hagenfeldt, L., & von Döbeln, U. (2002). Measurement of ATP production and respiratory chain enzyme activities in mitochondria isolated from small muscle biopsy samples. Analytical biochemistry, 311(2), 139–151. https://doi.org/10.1016/s00032697(02)00424-4 [2] Turki, A., Stockler, S., Sirrs, S., Salvarinova, R., & Elango, R. (2020). Development of Minimally Invasive 13C-Glucose Breath Test to Examine Different Dietary Therapies in Patients with Glycogen Storage Disorders. Current Developments in Nutrition, 4(Suppl 2), 1149. https://doi.org/10.1093/cdn/nzaa055_034 [3] Dillon, E. L., Janghorbani, M., Angel, J. A., Casperson, S. L., Grady, J. J., Urban, R. J., Volpi, E., & Sheffield-Moore, M. (2009). Novel non-invasive breath test method for screening individuals at risk for diabetes. Diabetes care, 32(3), 430–435. https://doi.org/10.2337/dc081578 [4] Turki, A., Ueda, K., Cheng, B., Giezen, A., Salvarinova, R., Stockler-Ipsiroglu, S., Elango, R. (2017). The Indicator Amino Acid Oxidation Method with the Use of L-[1-13C] Leucine Suggests a Higher than Currently Recommended Protein Requirement in Children with Phenylketonuria, The Journal of Nutrition, 147(2), 211-217. https://doi.org/10.3945/jn.116.240218

Acknowledgements