Alberta Children Research Articles

P2B.18: Feeding difficulties in children with Intestinal Failure

Introduction: Children with intestinal failure (IF) may be at risk for developing feeding difficulties given the negative stimuli associated with feeding, chronic illness and prolonged hospitalization. There is no literature on the prevalence of feeding difficulties in the IF population, its associated factors or intervention strategies. As a first step, we sought to screen for the frequency of the feeding difficulties and eating behaviours in children and herein provide the results of a preliminary sample. Methods:Children who were greater than 1 year of age with IF and on home PN for > 3 months were included from two centres: Alberta Children’s Hospital (ACH), Canada and Hôpital Necker-Enfants Malades, France. The validated MCH-Feeding Scale was used to screen for mild, moderate and severe feeding difficulty. The validated parent-rated Child Eating Behaviour Questionnaire (CEBQ) was used to screen for eating styles and food avoidant behaviours. Results: Preliminary data was gathered for n=34 subjects, median age 6 years with diagnoses of short bowel syndrome (n=22), chronic intestinal pseudo-obstruction (n=4), or other (n=8). Analysis of the MCH-Feeding Scale indicated that 24% of the subjects had no feeding difficulty, while 29%, 18%, and 29% reported mild, moderate and severe difficulty respectively. The subset of n=21 completing the CEBQ rated very high on food fussiness, very high on food enjoyment and very low for emotional over-eating relative to the UK reference sample (Fig 1). In particular, the ACH children scored very high on satiety responsiveness. Conclusions: Preliminary data indicate that approximately half of children with IF have moderate to severe eating difficulties. Interestingly, both food fussiness and enjoyment of food are more commonly featured eating behaviours in this sample. It is unclear whether the feeding difficulties of children with IF are related to the impact of the disease etiology and severity, early feeding history, degree of gastrointestinal symptomatology and/or attempts to assert some control. Future work will examine how IF specific factors including the use of tube feeds are associated with feeding difficulties.

P3B.19: The sensory profile of children with Intestinal Failure

Introduction: Sensory responses are shaped by a person’s experiences. A child’s sensory processing patterns may support and/or interfere with skill development and functional performance. Children with intestinal failure (IF) endure physical and psychological hardship from a young age that may impact sensory processing. We studied the sensory profile of children with IF. Methods:The Sensory Profile 2, a validated questionnaire-based tool, was used to evaluate processing patterns in the context of everyday life in children with IF (on home PN>3 months). An occupational therapist met with parents to apply the tool to a sample of children from two centres (Alberta Children’s Hospital, Canada, and Hopital Necker-Enfants Malades, France). The assessed sensory domains included: seeking, avoiding, sensitivity, registration, auditory, visual, touch, movement, body position, oral, conduct, emotional expression, and attentional responses. Individual scores were compared with normative data to determine z-scores. Results: Preliminary data for n=30 subjects, median age 5.0 (range 1.3, 14.6) years, included IF diagnoses of short bowel syndrome (n=18), chronic intestinal pseudo-obstruction (n=3) and other (n=9). A developmental diagnosis was noted in 7% of subjects. The sensory domains for which >20% of subjects had z-scores > +2.0 are listed in table 1. Compared to the normal reference data, 20% of the group avoided sensory input at higher rates, while 20% were “sensors” reacting more quickly and intensely to sensory input. Sensitivity to oral touch and taste were higher than average in almost half the children. Among 15–35 month olds, 27% rated with z-scores > +2.0 for general sensory. Among >3 year olds, 26% expressed strong emotional responses with sensory processing. Conclusions: Preliminary data suggests that a proportion of children with IF have sensory processing issues including oral sensory processing problems. Determining protective factors among those who do not develop oral sensitivity merits further study. Children with IF should undergo assessment of their sensory responses, and prevention strategies for sensory dysregulation in this population need to be developed.

The R941L mutation in MYH14 disrupts mitochondrial fission and associates with peripheral neuropathy.

BackgroundPeripheral neuropathies are often caused by disruption of genes responsible for myelination or axonal transport. In particular, impairment in mitochondrial fission and fusion are known causes of peripheral neuropathies. However, the causal mechanisms for peripheral neuropathy gene mutations are not always known. While loss of function mutations in MYH14 typically cause non-syndromic hearing loss, the recently described R941L mutation in MYH14, encoding the non-muscle myosin protein isoform NMIIC, leads to a complex clinical presentation with an unexplained peripheral neuropathy phenotype.MethodsConfocal microscopy was used to examine mitochondrial dynamics in MYH14 patient fibroblast cells, as well as U2OS and M17 cells overexpressing NMIIC. The consequence of the R941L mutation on myosin activity was modeled in C. elegans.FindingsWe describe the third family carrying the R941L mutation in MYH14, and demonstrate that the R941L mutation impairs non-muscle myosin protein function. To better understand the molecular basis of the peripheral neuropathy phenotype associated with the R941L mutation, which has been hindered by the fact that NMIIC is largely uncharacterized, we have established a previously unrecognized biological role for NMIIC in mediating mitochondrial fission in human cells. Notably, the R941L mutation acts in a dominant-negative fashion to inhibit mitochondrial fission, especially in the cell periphery. In addition, we observed alterations to the organization of the mitochondrial genome.InterpretationAs impairments in mitochondrial fission cause peripheral neuropathy, this insight into the function of NMIIC likely explains the peripheral neuropathy phenotype associated with the R941L mutation.FundThis study was supported by the Alberta Children's Hospital Research Institute, the Canadian Institutes of Health Research and the Care4Rare Canada Consortium.

Trends in psychiatric disorders prevalence and prescription patterns of children in Alberta, Canada.

To describe the trends in the prevalence and incidence of children with psychiatric disorders, the types of medication prescribed, and the type of physician providing the prescriptions. This retrospective study linked six population-based administrative databases (2008-2015) in Alberta, Canada. The prevalence of paediatric psychiatric disorders increased from 12.6 per 100 population in 2008 to 15.0 per 100 population in 2015, while the incidence rate increased from 2.0 per 100 population to 2.2 per 100 population in the same period. The proportion of patients dispensed any psychiatric medication increased from 21.4% in 2008 to 28.2% in 2015. Over the same period, dispensations for antidepressants increased from 7.0% to 11.2% and stimulants to treat ADHD, from 11.9% to 15.9%. For antidepressants, general practitioners (GPs) wrote the highest proportion of prescriptions (44.3% in 2011-48.1% in 2015), while paediatricians wrote the lowest proportion (8.7% in 2011-11.0% in 2015) and the proportion by psychiatrists decreased from 33.4% in 2011 to 27.2% in 2015. For stimulants to treat ADHD, paediatricians were the most frequent prescribers (36.9% in 2011-39.3% in 2015) followed by GPs as the second most frequent (33.1% in 2011-33.5% in 2015), while psychiatrists were the least likely to prescribe stimulants for ADHD. The increasing trend of psychiatric diagnoses and medication prescriptions in the paediatric population is evident using population-based administrative databases. The lack of safety and adverse consequences of medication use in this cohort warrants additional monitoring data.

Hybrid gel electrophoresis using skin fibroblasts to aid in diagnosing mitochondrial disease.

ObjectiveWe developed a novel, hybrid method combining both blue-native (BN-PAGE) and clear-native (CN-PAGE) polyacrylamide gel electrophoresis, termed BCN-PAGE, to perform in-gel activity stains on the mitochondrial electron transport chain (ETC) complexes in skin fibroblasts.MethodsFour patients aged 46–65 years were seen in the Metabolic Clinic at Alberta Children's Hospital and investigated for mitochondrial disease and had BN-PAGE or CN-PAGE on skeletal muscle that showed incomplete assembly of complex V (CV) in each patient. Long-range PCR performed on muscle-extracted DNA identified 4 unique mitochondrial DNA (mtDNA) deletions spanning the ATP6 gene of CV. We developed a BCN-PAGE method in skin fibroblasts taken from the patients at the same time and compared the findings with those in skeletal muscle.ResultsIn all 4 cases, BCN-PAGE in skin fibroblasts confirmed the abnormal CV activity found from muscle biopsy, suggesting that the mtDNA deletions involving ATP6 were most likely germline mutations that are associated with a clinical phenotype of mitochondrial disease.ConclusionsThe BCN-PAGE method in skin fibroblasts has a potential to be a less-invasive tool compared with muscle biopsy to screen patients for abnormalities in CV and other mitochondrial ETC complexes.

LO35: Characterizing pain in children with acute gastroenteritis presenting to the emergency department

Introduction: Although acute gastroenteritis is an extremely common childhood illness, there is a paucity of literature characterizing the associated pain and its management. Our primary objective was to quantify the pain experienced by children with acute gastroenteritis in the 24-hours prior to emergency department (ED) presentation. Secondary objectives included describing maximum pain, analgesic use, discharge recommendations, and factors that influenced analgesic use in the ED. Methods: Study participants were recruited into this prospective cohort study by the Alberta Provincial Pediatric EnTeric Infection TEam between January 2014 and September 2017. This study was conducted at two Canadian pediatric EDs; the Alberta Children's Hospital (Calgary) and the Stollery Children's Hospital (Edmonton). Eligibility criteria included < 18 years of age, acute gastroenteritis (□ 3 episodes of diarrhea or vomiting in the previous 24 hours), and symptom duration □ 7 days. The primary study outcome, caregiver-reported maximum pain in the 24-hours prior to presentation, was assessed using the 11-point Verbal Numerical Rating Scale. Results: We recruited 2136 patients, median age 20.8 months (IQR 10.4, 47.4); 45.8% (979/2136) female. In the 24-hours prior to enrolment, 28.6% (610/2136) of caregivers reported that their child experienced moderate (4-6) and 46.2% (986/2136) severe (7-10) pain in the preceding 24-hours. During the emergency visit, 31.1% (664/2136) described pain as moderate and 26.7% (571/2136) as severe. In the ED, analgesia was provided to 21.2% (452/2131) of children. The most commonly administered analgesics in the ED were ibuprofen (68.1%, 308/452) and acetaminophen (43.4%, 196/452); at home, acetaminophen was most commonly administered (77.7%, 700/901), followed by ibuprofen (37.5%, 338/901). Factors associated with analgesia use in the ED were greater pain scores during the visit, having a primary-care physician, shorter illness duration, fewer diarrheal episodes, presence of fever and hospitalization. Conclusion: Although children presenting to the ED with acute gastroenteritis experience moderate to severe pain, both prior to and during their emergency visit, analgesic use is limited. Future research should focus on appropriate pain management through the development of effective and safe pain treatment plans.

Human Craniofacial Spliceosomopathies

Germline mutation of core components of the spliceosomes have been associated with a range of clinical disorders from isolated retinitis pigmentosa (RP) to developmental syndromes, while somatic mutations of spliceosome components are linked to cell cycle dysregulation and cancer. Recently a group of craniofacial and skeletal disorders caused by dominant mutations in spliceosomal genes has emerged. The first of these to be identified is mandibulofacial dysostosis type Guion‐Almeida (MFDGA), caused by haploinsufficiency of the U5 snRNP component EFTUD2. We recently identified haploinsufficiency of SF3B4 as the cause of Nager syndrome, an acrofacial dysostosis (AFD), although only in 60% of cases suggesting genetic heterogeneity. Mutation of SF3B4 was subsequently reported in AFD Rodriquez type. SF3B4 is part of the U2 snRNP of the major spliceosome. We also identified heterozygous mutations in a highly conserved regulatory exon of SNRPBas the cause of cerebro‐costo‐mandibular syndrome (CCMS). Our experimental data show that these mutations disturb highly conserved exonic splicing silencer sequences crucial to the regulation of the gene's expression. SNRPB encodes a component of the Sm complex of the spliceosome. Additional craniofacial spliceosomopathies result from mutations in splicing factors TXNL4A, RBM8A and CWC27. The frequent involvement of craniofacial structures invite questions on the sensitivity of craniofacial and in particular pharyngeal arch development to spliceosomal defects, and on the role of the spliceosome in the regulation of development. The report of a young patient with Nager syndrome with bilateral synchronous breast cancer raises the possibility of a link between germline mutation of splicing factors and cancer predisposition. We also have a patient with a pathogenic SNRPB mutation who is non‐penetrant for CCMS syndrome but was diagnosed with early onset breast cancer, further linking heritable splicing mutations with cancer predisposition.Many of the craniofacial spliceosomopathies share overlapping features with Treacher‐Collins syndrome (TCS), a ribosomopathy. We are currently validating mutations in a ribosomal gene in two patients with typical Nager syndrome. This finding may provide unique insight into the overlapping roles of transcription and translation in craniofacial development. A combination of human genetics and model systems research will be required to further unravel the role of the spliceosome and the ribosome in craniofacial and skeletal development.Support or Funding InformationGenome Canada, Alberta Children's Hospital Research Institute, Genome AlbertaThis abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Use of a magnetic double J stent in pediatric patients: A case–control study at two Canadian pediatric centers

BackgroundUreteral stents with magnetic tips (Blackstar©) were recently approved for use in Canada. To our knowledge this is the first published evidence of their use in pediatric patients. Traditionally, pediatric stent insertion and removal are performed under general anesthetic. Magnetic stents have three main benefits in pediatric patients; cost savings, decreased OR time and reduced general anesthetic exposure. MethodsThis study was a proof of concept pilot, ran from May 2017–May 2018 to demonstrate the safety and efficacy of magnetic stents in pediatric patients. Patients undergoing ureteroscopy, ureteric re-implantation, and pyeloplasty with simultaneous magnetic stent insertion. Forty (40) patients had regular double J stents removed under anesthesia and served as control cases, and 40 patients had a magnetic double J at initial surgery at two different sites, CHU de Quebec and Alberta Children's Hospital. ResultsOverall, 39 magnetic stents were successfully retrieved without general anesthetic, representing a retrieval failure rate of only 2.5%. ConclusionAs demonstrated in our research, magnetic stents represent a safe and equally effective alternative to traditional stents, especially in a pediatric patient. This is because, at worst, if retrieval of the magnetic stent fails, traditional cystoscopic removal can be performed, so nothing is lost. Study TypeCase–control study. Level of evidenceLevel III.

Postpartum Anxiety, Depression Raise Risk of Developmental Delays

Back to table of contents Previous article Next article Clinical and Research NewsFull AccessPostpartum Anxiety, Depression Raise Risk of Developmental DelaysTerri D’ArrigoTerri D’ArrigoSearch for more papers by this authorPublished Online:14 Mar 2019https://doi.org/10.1176/appi.pn.2019.3a11AbstractData from the All Our Families study show increased risk of personal-social delays and communication delays at age 3 years.Children born to mothers who have persistent anxiety from late pregnancy to three years postpartum have an increased risk of delays in communication and personal-social development, according to a study in the Journal of Affective Disorders. The findings highlight the importance of early detection and support for mothers experiencing distress during pregnancy and the postpartum period and their offspring.Muhammad Kashif Mughal, M.B.B.S., Ph.D., says the population of at-risk women needs to be redefined because women with subclinical symptoms, which are associated with increased risk, are often “off the radar” of health professionals.Muhammad Kashif Mughal, M.B.B.S., Ph.D., a postdoctoral fellow at the University of Calgary, and colleagues evaluated data from the three-year follow-up to the All Our Families study, an ongoing study of mothers and children in Calgary, Canada. The study, begun in 2008 as the All Our Babies study, includes self-reported assessments of mothers’ distress (including depression, anxiety, and stress) at multiple time points ranging from before the 25th week of gestation up to three years postpartum. Also included are parent-reporting tools to measure child development at age 3 years across five domains: communication, gross motor, fine motor, problem solving, and personal-social. For the current study, the researchers analyzed data from 1,983 mother-child pairs.Overall, 4.7 percent to 6.4 percent of the mothers reported depressive symptoms at four time points: less than 25 weeks gestation, 34 to 36 weeks gestation, four months postpartum, and one year postpartum. Also, 13.8 percent to 18.5 percent of mothers reported anxiety symptoms at six time points: less than 25 weeks gestation, 34 to 36 weeks gestation, four months postpartum, one year postpartum, two years postpartum, and three years postpartum.Statistical analysis revealed that mothers with persistent high anxiety symptoms and early postpartum depression were more likely to report personal-social delays in their children at age 3 years compared with those mothers without such symptoms. The researchers also found that persistent subclinical and high anxiety symptoms were associated with an increased risk of communication delay at age 3 years. Furthermore, the relationship between maternal anxiety and risk of child communication delay was dose-dependent.“The proportion of children with risk of communication delays were highest in children of mothers experiencing clinical anxiety symptoms, followed by the subclinical, and the lowest for minimal symptoms,” Mughal told Psychiatric News.Mughal said that the results carry important implications for both practice and research, in which physicians and researchers traditionally rely upon clinical cutoffs to identify patients for intervention and follow-up.“The mothers experiencing subclinical symptoms are usually off the radar of health care professionals and are not identified by traditional cutoff approaches,” Mughal said. “The study highlights the importance of redefining ‘at-risk’ populations and moving from established cutoff approaches to ones that capture the chronicity and severity of symptoms during pregnancy and the postpartum period.”No statistically significant associations were found between maternal depression, anxiety, and stress symptoms over time and children’s gross motor, fine motor, and problem-solving development at 3 years. However, the researchers identified other risk factors for these domains, such as maternal age greater than 35 years, male sex, and preterm birth for gross-motor delays; low maternal education and male sex for fine motor delays; and low family income and preterm birth for problem-solving delays. Having a family with two or more children was a protective factor for fine motor development.“We need further studies to investigate the impact of these risk factors on child delays,” said Mughal.Mughal added that the findings call for a shift in perinatal health care to a model that emphasizes multiple perinatal screenings for maternal distress using validated tools and chronicity of symptoms.“Ultimately, improving processes for routine, timely, and appropriate maternal mental health assessment, treatment, and referral at multiple time points during the perinatal period will increase the quality of life in pregnant women and will help reduce the likelihood of adverse birth outcomes, postpartum mental health concerns, and adverse effects on offspring,” Mughal said.This study was supported by funding from Alberta Innovates, the Alberta Children’s Hospital Foundation, and the Maxbell Foundation. ■“Trajectories of Maternal Distress and Risk of Child Developmental Delays: Findings From the All Our Families (AOF) Pregnancy Cohort” can be accessed here. ISSUES NewArchived

Calgary Shunt Protocol, an adaptation of the Hydrocephalus Clinical Research Network shunt protocol, reduces shunt infections in children.

The shunt protocol developed by the Hydrocephalus Clinical Research Network (HCRN) was shown to significantly reduce shunt infections in children. However, its effectiveness had not been validated in a non-HCRN, small- to medium-volume pediatric neurosurgery center. The present study evaluated whether the 9-step Calgary Shunt Protocol, closely adapted from the HCRN shunt protocol, reduced shunt infections in children. The Calgary Shunt Protocol was prospectively applied at Alberta Children's Hospital from May 23, 2013, to all children undergoing any shunt procedure. The control cohort consisted of children undergoing shunt surgery between January 1, 2009, and the implementation of the Calgary Shunt Protocol. The primary outcome was the strict HCRN definition of shunt infection. Univariate analyses of the protocol, individual elements within, and known confounders were performed using Student t-test for measured variables and chi-square tests for categorical variables. Multivariable logistic regression was performed using stepwise analysis. Two-hundred sixty-eight shunt procedures were performed. The median age of patients was 14 months (IQR 3-61), and 148 (55.2%) were male. There was a significant absolute risk reduction of 10.0% (95% CI 3.9%-15.9%) in shunt infections (12.7% vs 2.7%, p = 0.004) after implementation of the Calgary Shunt Protocol. In univariate analyses, chlorhexidine was associated with fewer shunt infections than iodine-based skin preparation solution (4.1% vs 12.3%, p = 0.02). Waiting ≥ 20 minutes between receiving preoperative antibiotics and skin incision was also associated with a reduction in shunt infection (4.5% vs 14.2%, p = 0.007). In the multivariable analysis, only the overall protocol independently reduced shunt infections (OR 0.19 [95% CI 0.06-0.67], p = 0.009), while age, etiology, procedure type, ventricular catheter type, skin preparation solution, and time from preoperative antibiotics to skin incision were not significant. This study externally validates the published HCRN protocol for reducing shunt infection in an independent, non-HCRN, and small- to medium-volume pediatric neurosurgery setting. Implementation of the Calgary Shunt Protocol independently reduced shunt infection risk. Chlorhexidine skin preparation and waiting ≥ 20 minutes between administration of preoperative antibiotic and skin incision may have contributed to the protocol's quality improvement success.

Microstructural neuroimaging of white matter tracts in persistent post-concussion syndrome: A prospective controlled cohort study.

IntroductionChildren with mild traumatic brain injury (mTBI) typically recover quickly, however approximately 15% experience persistent post-concussive symptoms (PPCS) past 3 months. The microstructural pathology associated with underlying persistent symptoms is poorly understood but is suggested to involve axonal injury to white matter tracts. Diffusion tensor imaging (DTI) can be used to visualize and characterize damage to white matter microstructure of the brain.ObjectiveWe aimed to investigate white matter microstructure in children with persistent concussive symptoms as compared to typically developing controls, alongside evaluating differences in white matter changes over time and how this relates to symptom recovery.MethodsThe current study is a prospective, longitudinal, controlled cohort study of children with mTBI. 104 children aged 8 to 18 years with a mTBI (72 symptomatic; 32 asymptomatic) were recruited from the Alberta Children's Hospital and compared to 20 healthy controls. Microstructural evidence of white matter injury was evaluated using DTI one month post injury and repeated 4 to 6 weeks later. Primary outcomes included fractional anisotropy and mean diffusivity of the corticospinal tracts, uncinate fasciculi, and motor fibers of the corpus callosum. Post-concussive symptoms were also measured using the Post-Concussion Symptom Inventory (PCSI) taken at both time points.ResultsFractional anisotropy of the left uncinate fasciculi was lower in symptomatic children compared to controls (F(2,119) = 3.582, p = 0.031). No other significant differences were observed.ConclusionsOur findings provide evidence of microstructural injury following mTBI in children with ongoing post-concussive symptoms one month post injury. The changes were persistent 4–6 weeks later. Further longitudinal studies of white matter microstructure in PPCS will be helpful to clarify whether these white matter alterations resolve over time.

Persistent Postconcussive Symptoms in Children and Adolescents.

Investigators from the Children's Hospital of Eastern Ontario, Boston Children's Hospital, Alberta Children's Hospital, University of Montreal, McGill University Health Center, Hospital for Sick Children, University of Calgary, and the University of Ottawa researched the association between early physical activity and persistent postconcussive symptoms (PPCS).

Single-Use Detergent Sacs: A Retrospective Multicenter Canadian Review of Emergency Department Cases.

Single-use detergent sacs (SUDS) are widely used in North America and Europe with emerging literature on their toxicity. This is the first Canadian multicenter study aimed to quantify and compare SUDS exposures to traditional detergent exposures. A retrospective review of the Canadian Hospitals Injury Reporting and Prevention Program databases was conducted at the Hospital for Sick Children in Toronto, Alberta Children's Hospital in Calgary and the Stollery Children's Hospital in Edmonton. All exposures presenting to these 3 centers between 2009 and 2014 were identified, a case form was completed, and data were analyzed. Forty cases of SUDS exposure were identified alongside 35 cases of traditional detergent exposure during the study period resulting in an incidence of 3.16 SUDS exposures per million children per year presenting to tertiary pediatric emergency departments (EDs). In contrast, traditional detergent exposures had an incidence of 2.78 exposures per million children per year presenting to tertiary pediatric EDs over the study period. Although there was no change in incidence of exposure to traditional detergent over the study period, there was an increase in the incidence of SUDS exposures from 2010 to 2013, with a decrease seen in 2014. There was no significant difference seen in age, sex, location of exposure, transportation to hospital, morbidity, or mortality associated with SUDS exposures compared with traditional detergent exposure. Although not statistically more likely to cause long-term complications, SUDS-exposed children required more follow-up visits to health care providers than traditional detergents. This multicenter study is the first to establish the incidence of SUDS and traditional detergent exposure in 3 Canadian cities. Overall, the frequency of exposure to detergents-both traditional and SUDS-is very low. Given the increase in SUDS exposure seen from 2011 to 2013, alongside larger sales of SUDS, continued efforts are required to monitor exposures, and reduce potential exposures to SUDS and traditional detergents in the future.

Using Administrative Health Data to Define a Cohort of Youth Affected by Chronic Health Conditions: Preparing for Cross-Sectoral Data Linkage

IntroductionIn Alberta, 2,400 youth with chronic needs transition to adulthood every year, and many are not prepared for this change. Transferring youth from pediatric to adult-oriented care is poorly managed. To improve this process, we need to know how youth patients use health services during this period.
 Objectives and ApproachWe used the Alberta Health Services Corporate Data Repository (CDR-9), which collects records of ambulatory visits, to define a cohort of patients with chronic disease using pediatric tertiary care; data is available from 2008 to 2016. Personal health numbers allowed for deterministic data linkage to CDR-9, registry data (e.g., death dates, moves out of province), and area deprivation indices. Eligible patients were: (a) between ages 12-15 years in 2008 (for ≥2 years observation in adulthood, after age 18), (b) involved with a Chronic Care Clinic (CCC) at Alberta Children’s Hospital, and (c) had repeated CCC visits with ≥3 months between visits.
 ResultsWe identified 26 Chronic Care Clinics (CCC) at Alberta Children’s Hospital (Calgary, Alberta), with stakeholder input. Using CDR-9, a total of 10,111 patients at the hospital were identified who were 12 to 15 years old at the start of the study window (in 2008), and who visited a CCC before age 18. Less than 1% (n=418) were excluded due to moving out of province or having an invalid personal heath number. Final sample sizes were captured across 3 algorithms (A1, A2, A3), based on frequency of CCC visits within a 2-year period: (i) A1: ≥2 CCC visits (N=4123); (ii) A2: ≥3 CCC visits (N=2242); (iii) A3: ≥4 CCC visits (N=1344).
 Conclusion/ImplicationsOur identified cohort of youth affected by chronic conditions is the first of its kind in Alberta, and can answer important questions about patterns of service utilization in other sectors of care. Our next step is to link the cohort to population-level datasets (e.g., physician claims, NACRS, CIHI-DAD).

Agricultural injuries among farm and non-farm children and adolescents in Alberta, Canada.

Understanding of the specific risk of agricultural injury sustained by different populations of children and adolescents is needed for effective safety intervention. To compare the rates and patterns of agricultural injury incidence (fatal and non-fatal injury) between farm and non-farm children less than 18 years of age in Alberta, Canada. A total of 115 378 children (five subgroups: two groups of farm children and three groups of non-farm children) in Alberta were followed from 1999 to 2010 to examine injury incidence using the linkage of three administrative health databases. A recurrent event survival analysis using Cox proportional hazards regression was carried out. A total of 1 849 agricultural injury episodes (1 616 emergency department visits, 225 hospitalizations, and 8 deaths) were identified from 1999 to 2010. The age- and gender-adjusted rate (per 100 000 person years) of agricultural injury was 672.3 for rural-living farm children, 369.4 for urban-living farm children, 180.2 for rural non-First Nations (FN) children, 64.4 for rural FN children, and 23.7 for urban children in descending order. Specific strategies for different children's populations to prevent agricultural injuries and to extend agricultural injury controls to non-farming populations are needed.

Educating children and youth in care in Alberta: a scoping review

Alberta Education and Alberta Children Services have reported consistent low achievement in school by children and youth in care, especially those in residential group care. This article provides the current picture of research and practice (policy) regarding the learning experience of children and youth in care. Utilizing a scoping review of local and international research studies, the paper argues that the education of children in care in Alberta is not considered an important issue. The dominance of the social work paradigm in children and family services is exposed as inadequate, and hence the call for social pedagogy to be adopted. Determinants of educational achievement for children and youth in care are examined and using these attainment factors, the article identifies and recommends areas that Alberta Education and Alberta Children Services need to consider with urgency if children and youth in care are to benefit from schooling like all other children.

C.07 Calgary shunt protocol, an adaptation of the hydrocephalus clinical research network shunt protocol reduces risk of shunt infection in children

Background: The effectiveness of the Hydrocephalus Research Network (HCRN) shunt protocol has not been validated in a non-HCRN, small-to-medium volume pediatric neurosurgery center. This study evaluates whether the 9-step Calgary Shunt Protocol (CSP) adapted from the HCRN shunt protocol reduced shunt infections. Methods: The CSP was prospectively applied at Alberta Children’s Hospital from May 23rd, 2013 to all children undergoing any shunt procedure. Children undergoing shunt surgery before CSP implementation acted as a control-cohort. The strict HCRN definition of shunt infection was applied. Results: A total of 268 shunt procedures were performed. There was a significant absolute risk reduction of 10.0% ([95%CI 3.9%-15.9%], p=0.004) in shunt infections after implementation of the CSP. In univariate analyses, chlorhexidine compared to povidone skin prep reduced shunt infection by 8.2% ([95%CI 1.84-14.6%], p=0.02) and waiting ≥ 20 min between receiving preoperative antibiotics and skin incision reduced shunt infections by 9.6% ([95%CI 2.4%-16.9%], p=0.02). In multivariate analysis, only protocol implementation independently reduced shunt infections (OR 0.19 [95%CI 0.06-0.67], p=0.004). Conclusions: This study externally validates the published HCRN protocol for reducing shunt infection in an independent, non-HCRN, and small-to-medium volume neurosurgery setting. Chlorhexidine skin prep and waiting ≥ 20 min between preoperative antibiotic and skin incision may have contributed to the protocol’s quality improvement success.

DIAGNOSTIC AND COLLABORATION NEEDS REGARDING ASD BY COMMUNITY PAEDIATRICIANS IN SOUTHERN ALBERTA

Abstract BACKGROUND Paediatricians play a central role in the screening and diagnosis for autism spectrum disorder (ASD). Current diagnostic procedures rely on a history, screening and diagnostic tools, and collaboration with developmental specialists. The assessment process may vary among paediatricians due to the variety of available screening and diagnostic tools and opportunities to collaborate. As part of a quality improvement initiative through Child Development Services at the Alberta Children’s Hospital, paediatricians were surveyed about their present ASD screening and diagnostic practice. OBJECTIVES To examine ASD screening and diagnostic practices among paediatricians for 4 to 6 year-old children in southern Alberta. DESIGN/METHODS Paediatricians were recruited from southern Alberta to complete an anonymous online survey. Data were analyzed using descriptive statistics. RESULTS The response rate was 40% (36/90). The majority of participants (86%) reported using an ASD screening tool, and (56%) reported experiencing one or more barriers related to screening tool use. All participants reported experiencing one or more barriers to ASD diagnosis. Despite these barriers, 69% of participants reported making an ASD diagnosis within the last 12 months, and 61% of paediatricians indicated feeling confident in their ability to diagnose ASD. Most participants (57%) indicated that they would prefer to make an ASD diagnosis themselves, rather than have this be undertaken by another clinician. CONCLUSION Paediatricians in southern Alberta report important barriers in screening and diagnostic practices related to ASD. Further discussion with community paediatricians is required related to addressing these barriers to develop care pathways for this population.

Artificially Sweetened Vitamin Drink Consumption Reduces Insulin Sensitivity and Alters One‐Carbon, B‐Vitamin Dependent Metabolism in Adolescents

ImportanceDiscretionary fortified beverages contain several ingredients such as vitamins and minerals in excess amounts, yet their consumption and sales have been burgeoning. Marketed as providing a unique health benefits to consumers, concerns have been raised about the overconsumption of some nutrients, especially in children and adolescents.ObjectiveTo evaluate the impact of discretionary fortified beverage consumption on energy metabolism, gut hormones and metabolic profiles of adolescents.DesignA double blind, randomized, placebo‐controlled crossover trial examined the metabolic effects of a concentrated, small format B‐vitamin fortified beverage. Healthy adolescents (10 males, 10 females, 13–19y) completed two trials separated by at least 1 week, consuming either water as placebo (PL) or an artificially sweetened discretionary fortified beverage (FB, 1.5ml/kg), which were given 40min prior to an oral glucose tolerance test (OGTT).MethodBlood samples were collected at baseline and at different time‐points throughout the OGTT for insulin, gut hormones and 1H‐NMR spectroscopy‐based metabolomics analyses. To evaluate gene‐metabolic interactions, a group of the most common single nucleotide polymorphisms (SNPs) linked to B‐vitamin metabolism was also assessed.ResultsPlasma glucose was not different between the treatment groups (p=0.111). However, plasma insulin was significantly higher in the FB group (p=0.003) and accompanied by a 28% decrease in insulin sensitivity in FB compared to PL as indicated by the composite Insulin Sensitivity Index (ISI). Gut hormone secretion was also differentially modulated by the consumption of FB. We found a significant increase in AUC of GLP‐1 (p<0.001), glucagon (p=0.015), PYY (p=0.001) and C‐peptide (p=0.017) in FB compared to the PL group. Employing 1H‐NMR metabolomics, we also show perturbations to one carbon, B‐vitamin linked sulfur amino acid metabolism with FB consumption. This metabolic response was dependent on genotypic variance at rs651852 (Betaine Homocysteine methyltransferase, BHMT, p<0.05).ConclusionCurrent data provide evidence that FB are not inert, but may promote insulin resistance, perturb one carbon, B‐vitamin‐linked metabolism and differentially modulate gut hormone secretion in adolescent consumers. Results may warrant reconsideration of regular, artificially sweetened FB consumption for adolescents at risk for obesity and metabolic disease.Support or Funding InformationThe research was funded by the Alberta Children's Hospital Research Institute and the National Science and Engineering Council of Canada (JS). SM is funded through an Eyes High Postdoctoral Fellowship and an Alberta Innovates Health Solutions Postdoctoral Fellowship.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Addition of Digoxin Improves Cardiac Function in Children With the Dilated Cardiomyopathy With Ataxia Syndrome: A Mitochondrial Cardiomyopathy

BackgroundThe dilated cardiomyopathy with ataxia syndrome (DCMA) is a rare mitochondrial disorder characterized by progressive cardiomyopathy, prolonged QT interval and early death in childhood related to intractable heart failure. We present a case series of 9 children with DCMA who demonstrated functional improvement and favourable left ventricular remodeling only after digoxin was added to their medical therapy. MethodsA retrospective review of 46 patients with DCMA followed at the Alberta Children’s Hospital from 2005 to 2017 identified 9 patients who were treated with digoxin and had serial echocardiography data. For each subject, we calculated the difference between baseline and follow-up for left ventricular ejection fraction (LVEF), end-diastolic dimension (LVEDD), and end-systolic dimension (LVESD) as determined by echocardiography. ResultsPatients were on average 45.6 ± 59 months of age when digoxin was started with a mean LVEF of 40% ± 11% when digoxin was started. Seven patients were on angiotensin-converting enzyme inhibitors (ACEIs) at the time of initiation of digoxin, and all were on β-receptor antagonists (BB). After being on digoxin for a mean of 11.7 ± 10.9 months, average LVEF improved to 55% ± 10% (P = 0.0005), and there were significant decreases in the Z-scores for LVEDD (+2.1 ± 1.9 to +0.65 ± 1.4, P = 0.02) and LVESD (+3.83 ± 2.07 to +1.79 ± 1.76, P = 0.01). ConclusionsIn children with DCMA, we report that digoxin seems to have additive beneficial properties when combined with ACEI and BB therapy. This novel observation may have implications for the medical treatment of mitochondrial cardiomyopathies.