Congratulations to Madeleine on receiving a BC Children’s Hospital Research Institute Summer Studentship

SESSION #2

Poster #14

Tyrone Ly

Watch In-Person:

Thursday, July 27 | 10:00 - 11:30 am

Chieng Family Atrium, BCCHR

Master’s Student, University of British Columbia | Supervisor: Todd S. Woodward

Task-based Working Memory Functional Brain Networks Detectable by fMRI

Tyrone Ly, Linda Chen, Todd S. Woodward

Research Objectives: In cognitive neuroscience, fMRI investigation of the working memory through analysis of the Sternberg delayed recognition task has been a prominent research area for decades, but the field has yet to arrive at a consensus for a set of macroscale functional brain networks. In the present study, we utilized a version of the task called Sternberg Item Recognition Paradigm and hypothesized the emergence of the Working Memory Big Five brain networks that were established in previous research. They consist of Response, Focus on Visual Features, Initiation, Internal Attention, and Default Mode Networks.

Methods: fMRI data was obtained from the Function Biomedical Informatics Research Network phase II multi-site study in which 55 healthy controls and 54 patients with schizophrenia or schizoaffective disorder performed the Sternberg task while undergoing fMRI scanning sessions. Constrained Principal Component Analysis for fMRI (fMRI-CPCA) was employed to identify and characterize functional brain networks that emerged during task performance and their associated hemodynamic responses.

Preliminary Results: Auditory Attention for Response Network was extracted together with three of the Big Five, which included Response, Focus on Visual Features, and Traditional Default Mode Networks. Traditional Default Mode Network had the most significant effect of group, while Response demonstrated load-dependency. Interestingly, Focus on Visual Features Network showed patterns of activation, instead of suppression as expected from previous findings. Internal Attention Network was potentially merged into the extracted Response Network, and Initiation was potentially merged into Focus on Visual Features.

Future Directions: These findings suggest that Traditional Default Mode Network have reduced activity in schizophrenia patients, implicating their dysfunction may underlie cognitive impairments in schizophrenia. The effects of site difference, sex differences in brain networks, and neuropathological differences between schizophrenia and schizoaffective disorder will be further investigated. Correlations with symptoms will also be established, which will contribute to advancements in diagnosis and the development of novel treatment options.

Congratulations to Tyrone on receiving a UBC Faculty of Medicine Summer Studentship

SESSION #2 Poster #15

Kudrat Mundi

Watch In-Person:

Thursday, July 27 | 10:00 - 11:30 am

Chieng Family Atrium, BCCHR

Medical Student, University of British Columbia | Supervisor: Marie Wright

Use of long term ventilation for preterm infants with severe bronchopulmonary dysplasia in British Columbia

Kudrat

Mundi, Marie Wright, Emily Kieran, Jessie Van Dyk

Background: Advancements in care have resulted in improved survival of infants born at increasingly low gestational age, however, these interventions have not lowered the prevalence of chronic lung disease of prematurity, also known as bronchopulmonary dysplasia (BPD).

An increasing number of infants are therefore being discharged from the neonatal intensive care unit (NICU) who require ongoing management of BPD. Home respiratory management options include: supplementary oxygen, non-invasive positive pressure ventilation (NIPPV), and invasive ventilation via tracheostomy.

There is a lack of information about the trajectory of this patient population including the duration for which respiratory support is needed, long term respiratory/non-respiratory outcomes, and differences in outcomes depending on the type of support received.

Study Objectives:

1. Characterize the population of infants with BPD managed with home respiratory support in terms of their gestational age and antenatal background, early neonatal management, and medical comorbidities.

2. Describe their clinical outcomes including duration of respiratory support, treatment-related complications, growth trajectory, neurodevelopment, and hospital admissions in the two years after NICU discharge.

3. Investigate for predictors of the above outcomes in terms of patient characteristics and the type of respiratory support used.

Methods: A retrospective review of all infants with severe BPD requiring home respiratory support, discharged from a Level 3 NICU in British Columbia between January 2013 and December 2022.

Cases were identified from clinical databases of the BC Children’s and Women’s Hospital (BCWH) Neonatal Follow-up (NFU) and Home Tracheostomy & Ventilation (HTV) programs, and from BCWH NICU discharge coding. Data were also collected about infants with severe BPD who died prior to hospital discharge while still on respiratory support.

Significance: Our center is uniquely placed to answer these important questions because of our large BPD population who are closely followed by specialist outpatient services (HTV and NFU), and our extensive experience of using NIPPV in this patient group.

Findings will address existing knowledge gaps around optimal management and long-term outcomes of infants with severe BPD. It will also characterise the size, healthcare utilization, and long-term medical issues of this patient group within British Columbia which will help to plan future medical services.

Congratulations to Kudrat on receiving a Florence E Heighway Summer Research Award, UBC Faculty of Medicine Summer Student Research Program

SESSION #2 Poster #16

Iqbal Sarai

Medical Student, University of Galway | Supervisor: Mor Cohen-Eilig

Watch In-Person:

Thursday, July 27 | 10:00 - 11:30 am

Chieng Family Atrium, BCCHR

The role of Baclofen in the treatment of Pediatric Gastroesophageal Reflux Disease: A Scoping Review

Iqbal Sarai, Ram Mishaal, Mor Cohen-Eilig

Background: Gastroesophageal reflux disease (GERD) is a pathological condition that results in persistent reflux of stomach contents into the distal esophagus and is quite common in pediatric patients. A major contributing factor to GERD is a compromised lower esophageal sphincter (LES). Transient lower esophageal sphincter relaxations (TLESRs) are one of the emerging theories, another is that the lower esophageal sphincter (LES) tension is weakened. Current treatments of GERD in pediatric patients focus on using Proton pump inhibitor (PPI’s). These classes of drugs are effective but do not work in all cohort of patients to the same degree. Baclofen is a drug that has been shown to help relieve symptoms of GERD in adults and may be a promising therapeutic option that could be explored in this condition.

Methods: Literature searching using PubMed and Ovid Medline databases. Finding individual studies to construct a literature review on the use of baclofen in pediatric patients who have GERD.

Results: Literature searching showed that baclofen has a major impact on reducing TLESRs. In pediatric patients’ baclofen shows significant reduction of symptoms such as vomiting, regurgitation, abdominal pain and heartburn. Studies also show similar results in animals, and in adult patients and healthy volunteers. Baclofen increases the threshold volume needed to trigger secondary peristalsis and decreased the frequency of secondary peristalsis. Other papers also showed that the LES tension was increased with administration of baclofen resulting in reduction of acid reflux episodes. Adverse effects associated with baclofen were dizziness and drowsiness.

Conclusion and Future Directions: Baclofen shows promise to be an option in treating GERD in pediatric patients. Reduction in TLESRs and increase in LES tension both help with the two major theories of why GERD occurs. The data is however lacking for normalized values in children and more experiments with larger cohorts should be established.

SESSION #2 Poster #17

Rene Tandun

Watch In-Person:

Thursday, July 27 | 10:00 - 11:30 am

Chieng Family Atrium, BCCHR

Medical Student, University of British Columbia | Supervisor: Catherine Biggs

Beyond infections: Immune dysregulation in patients with Inborn Errors of Immunity

Rene Tandun, Abigail Netanya Tan Ngan, Catherine Biggs

Background: Inborn Errors of Immunity (IEI) are disorders caused by monogenic germline mutations that affect how the immune system works or develops. More emphasis has been given to recurrent infections as a clinical presentation, and less to immune dysregulation, a process of uncontrolled immune responses leading to inflammation, allergy and malignancy. Understanding non-infectious manifestations are key to reducing diagnostic delays and reducing morbidity and mortality seen in IEIs.

Purpose: The purpose of the study is to determine the frequency of immune dysregulatory complications in children with IEIs. We hypothesize that immune dysregulation is a common initial manifestation, and that the presence of immune dysregulation is associated with worse clinical outcomes in this patient population.

Methods: In this retrospective chart review, data was extracted from medical records of pediatric patients with confirmed IEIs seen at BC Children’s Hospital (BCCH). Deidentified demographics, diagnosis, treatment and clinical outcomes were collected in REDCap. Immune dysregulatory features included lymphoproliferation, granuloma, autoimmunity, allergy or autoinflammation. Outcome variables included age at initial presentation, age at diagnosis, and chronic disease or end-organ involvement.

Preliminary Results: Among the forty-two pediatric patients with IEI (76% males) identified, infection was the most common initial symptom (n=18, 42.9%) followed by immune dysregulation (n=11, 26.2%). Thirty-two patients (76.2%) reported a history of immune dysregulation, with allergic disease being the most common. The mean age at diagnosis was 35.7 months.

Significance: Given the prevalence of immune dysregulatory symptoms in patients with IEI, it is important that health care providers consider an IEI diagnosis when investigating non-infectious presentations even in the absence of recurrent infections. The results of this study can inform clinical guidelines, with the goal of improving outcomes for patients with IEI.

Congratulations to Rene on receiving a BC

SESSION #2 Poster #18

Zixin Grace Yang

Watch In-Person:

Thursday, July 27 | 10:00 - 11:30 am

Chieng Family Atrium, BCCHR

Medical Student, University of Edinburgh | Supervisor: Linlea Armstrong

Understanding the Care Provided to Patients With a Pediatric Cancer Predisposition Syndrome Across British Columbia

Zixin G. Yang, Linlea Armstrong, Caron Strahlendorf, Pediatrics CPS Team members

Background: A pediatric cancer predisposition syndrome (CPS) increases the chances of cancer due to a sporadic or inherited gene variant. Common cancer predisposition syndromes include: Li-Fraumeni, familial adenomatous polyposis, and neurofibromatosis type 1. Literature shows that cancer predisposition syndromes account for 5-10% of all cancers, and that the quality-of-life following diagnosis and treatment is decreased. There are also physical, neurocognitive, and psychosocial late effects after recovery from cancer. Currently there are no provincial guidelines on care provided to children and at risk family members with a pediatric CPS in British Columbia (BC).

Objective: The project goal is to develop a care pathway for patients with a, or suspected, pediatric CPS. The initial step is to identify all the children in BC then determine if they are accessing suitable care with regards to referrals, triaging, genetic testing and treatment.

Methods: An Excel database was created. The data variables were identified through an environmental scan of clinics across North America. To ensure consistency, Excel data entry forms were coded using Visual Basic Application. Most fields have a dropdown list to minimize free text entry. Patient information will be entered if under 19 yearsold and suspected to have a CPS, or diagnosed with a CPS and symptomatic or asymptomatic. The exclusion criteria are individuals diagnosed with a CPS after turning 19. Electronic and paper charts will be used to retrieve patient information. Historical and prospective patients will be assessed through referrals to the Medical Genetics Department. It is estimated that there are around 200 historical patients and 20-40 prospective patients annually.

A patient and healthcare provider symposium is planned for 2024 to explore how the information collected in the database can be utilized to improve patient care. This engagement event is crucial to understand what is important to patients, families and healthcare providers.

Conclusion: The development of a patient database will support qualitative improvement projects and research regarding pediatric CPS. The database is a stepping stone to a defined and resourced care pathway for CPS patients, allowing equitable and consistent care to be provided across British Columbia.

SESSION #2 Poster #18A

Layan Bashi

Watch In-Person:

Thursday, July 27 | 10:00 - 11:30 am

Chieng Family Atrium, BCCHR

Master’s Student, University of British Columbia | Supervisor: Sharon Dell