bluuebunny/crossref_metadata_2025 huggingface dataset:
Cardiomyopathy mutation (F88L) in troponin T abolishes length dependency of myofilament Ca2+ sensitivity
Authors: Reda Sherif M. , Chandra Murali
Published: May 2018

Recent clinical studies have revealed a new hypertrophic cardiomyopathy–associated mutation (F87L) in the central region of human cardiac troponin T (TnT). However, despite its implication in several incidences of sudden cardiac death in young and old adults, whether F87L is associated with cardiac contractile dysfunction is unknown. Because the central region of TnT is important for modulating the muscle length–mediated recruitment of new force-bearing cross-bridges (XBs), we hypothesize that the F87L mutation causes molecular changes that are linked to the length-dependent activation of cardiac myofilaments. Length-dependent activation is important because it contributes significantly to the Frank–Starling mechanism, which enables the heart to vary stroke volume as a function of changes in venous return. We measured steady-state and dynamic contractile parameters in detergent-skinned guinea pig cardiac muscle fibers reconstituted with recombinant guinea pig wild-type TnT (TnTWT) or the guinea pig analogue (TnTF88L) of the human mutation at two different sarcomere lengths (SLs): short (1.9 µm) and long (2.3 µm). TnTF88L increases pCa50 (−log [Ca2+]free required for half-maximal activation) to a greater extent at short SL than at long SL; for example, pCa50 increases by 0.25 pCa units at short SL and 0.17 pCa units at long SL. The greater increase in pCa50 at short SL leads to the abolishment of the SL-dependent increase in myofilament Ca2+ sensitivity (ΔpCa50) in TnTF88L fibers, ΔpCa50 being 0.10 units in TnTWT fibers but only 0.02 units in TnTF88L fibers. Furthermore, at short SL, TnTF88L attenuates the negative impact of strained XBs on force-bearing XBs and augments the magnitude of muscle length–mediated recruitment of new force-bearing XBs. Our findings suggest that the TnTF88L-mediated effects on cardiac thin filaments may lead to a negative impact on the Frank–Starling mechanism.
Influences: Cold Spring Harbor summer courses and Drosophila melanogaster neurogenetics
Authors: Jan Lily Yeh , Jan Yuh Nung
Published: May 2018

The Jans recollect features of the scientific environments that influenced their careers.
Final report on APMP.EM.RF-S21.F
Authors: Ishii Masanori , Kim Jeong Hwan , Ji Yu , Cho Chi Hyun , Zhang Tim
Published: May 2018

The supplementary comparison report APMP.EM.RF-S21.F describes the comparison of loop antennas, which was conducted between April 2013 and January 2014. The two comparison artefacts were well-characterised active loop antennas of diameter 30 cm and 60 cm respectively, which typically operate in a frequency range from 9 kHz to 30 MHz. These antennas represent the main groups of antennas which are used around the world for EMC measurements in the frequency range below 30 MHz. There are several well-known methods for calibrating the antenna factor of these devices.

The calibration systems used in this comparison for the loop antennas employed the standard magnetic field method or the three-antenna method. Despite the limitations of the algorithm, which we used to derive the reference value for each case (particularly for small samples), the actual calculated reference values seem to be reasonable. As a result, the agreement between each participant was very good in all cases.

Main text

To reach the main text of this paper, click on Final Report. Note that this text is that which appears in Appendix B of the BIPM key comparison database kcdb.bipm.org/.

The final report has been peer-reviewed and approved for publication by the CCEM, according to the provisions of the CIPM Mutual Recognition Arrangement (CIPM MRA).
Measuring Environmental and Socio-economic Impact of Deforestation at Kalimantan Island
Authors: Nahib Irmadi , Trenggana Soma , Turmudi  , Suryanta Jaka , Munajati Sri Lestari , Windiastuti Rizka
Published: May 2018

Indonesia’s forests in the period of 2000-2009 has been deforested by about 15.158 million ha out of 103.309 milion ha. Deforestation caused carbon emissions. One method for measuring emissions from deforestation and forest degradation is GeOSIRIS model. A modeled GeOSIRIS policy used a carbon payment system to incentivize emission reductions. Data used in this study were maps of forest cover in 2005 and 2010, map of deforestation 2005-2010, carbon and agricultural price and driver variables for deforestation such as slope, elevation, logarithmic distance to the nearest road or provincial capital, or the amount of area per pixel included in a national park, or a timber plantation. The result of this study showed rate of deforestation was 1.417 million ha/5 years (observed). The REDD policy could decrease deforestation in Kalimantan Island by 0.170 million ha (16.70%), with assumption that international carbon price of US$ 10/tCO2e. The change of emissions due to REDD was 22.29%, or reduced emissions by 245.03 million tCO2e/5 years. Finally, Gross National Revenue from carbon payments (NPV 5 years) was US$ 2,450.34 billion, where incentivize emission reductions to sub-national entities (NPV, 5 years) was US$ 2,150.07 million and net central government surplus from carbon payments was US$ 300.26 million (NPV, 5 years).
Identifying the driving forces of urban expansion and its environmental impact in Jakarta-Bandung mega urban region
Authors: Pravitasari A E , Rustiadi E , Mulya S P , Setiawan Y , Fuadina L N , Murtadho A
Published: May 2018

The socio-economic development in Jakarta-Bandung Mega Urban Region (JBMUR) caused the increasing of urban expansion and led to a variety of environmental damage such as uncontrolled land use conversion and raising anthropogenic disaster. The objectives of this study are: (1) to identify the driving forces of urban expansion that occurs on JBMUR and (2) to analyze the environmental quality decline on JBMUR by producing time series spatial distribution map and spatial autocorrelation of floods and landslide as the proxy of anthropogenic disaster. The driving forces of urban expansion in this study were identified by employing Geographically Weighted Regression (GWR) model using 6 (six) independent variables, namely: population density, percentage of agricultural land, distance to the center of capital city/municipality, percentage of household who works in agricultural sector, distance to the provincial road, and distance to the local road. The GWR results showed that local demographic, social and economic factors including distance to the road spatially affect urban expansion in JBMUR. The time series spatial distribution map of floods and landslide event showed the spatial cluster of anthropogenic disaster in some areas. Through Local Moran Index, we found that environmental damage in one location has a significant impact on the condition of its surrounding area.
Small group discussion to promote reflection and social change: A case study of a Half the Sky intervention in India
Authors: Kogen Lauren
Published: May 2018

An important sub-discipline within the field of Communication and Social Change addresses how meaningful participation can be practically implemented. This article presents the case of an intervention developed by the NGO ‘Half the Sky Movement’ and reflects upon how participation took shape within a primarily top-down program model. The design of the project bridges traditional, outsider-led and participatory, bottom-up design. The project accomplishes this by focusing on small group discussion and short videos as catalysts for reflection. In addition, the data suggest that storytelling may be particularly helpful for promoting engaged discussion and critical reflection.
The Geometric Structure of Symplectic Contraction
Authors: Lane Jeremy
Published: May 2018

We show that the symplectic contraction map of Hilgert–Manon–Martens [9], a symplectic version of Popov’s horospherical contraction, is simply the quotient of a Hamiltonian manifold $M$ by a “stratified null foliation” that is determined by the group action and moment map. We also show that the quotient differential structure on the symplectic contraction of $M$ supports a Poisson bracket. We end by proving a very general description of the topology of fibers of Gelfand–Zeitlin (also spelled Gelfand–Tsetlin or Gelfand–Cetlin) systems on multiplicity-free Hamiltonian $U(n)$ and $SO(n)$ manifolds.
Primary care provider adherence to an alert for intensification of diabetes blood pressure medications before and after the addition of a “chart closure” hard stop
Authors: Ramirez Magaly , Maranon Richard , Fu Jeffery , Chon Janet S , Chen Kimberly , Mangione Carol M , Moreno Gerardo , Bell Douglas S
Published: May 2018

Objective

To evaluate provider responses to a narrowly targeted “Best Practice Advisory” (BPA) alert for the intensification of blood pressure medications for persons with diabetes before and after implementation of a “chart closure” hard stop, which is non-interruptive but demands an action or dismissal before the chart can be closed.

Materials and Methods

We designed a BPA that fired alerts within an electronic health record (EHR) system during outpatient encounters for patients with diabetes when they had elevated blood pressures and were not on angiotensin receptor blocking medications. The BPA alerts were implemented in eight primary care practices within UCLA Health. We compared data on provider responses to the alerts before and after implementing a “chart closure” hard stop, and we conducted chart reviews to adjudicate each alert’s appropriateness.

Results

Providers responded to alerts more often after the “chart closure” hard stop was implemented (P < .001). Among 284 alert firings over 16 months, we judged 107 (37.7%) to be clinically unnecessary or inappropriate based on chart review. Among the remainder, which represent clear opportunities for treatment, providers ordered the indicated medication more often (41% vs 75%) after the “chart closure” hard stop was implemented (P = .001).

Discussion

The BPA alerts for diabetes and blood pressure control achieved relatively high specificity. The “chart closure” hard stop improved provider attention to the alerts and was effective at getting patients treated when they needed it.

Conclusion

Targeting specific omitted medication classes can produce relatively specific alerts that may reduce alert fatigue, and using a “chart closure” hard stop may prompt providers to take action without excessively disrupting their workflow.
LINKS BETWEEN MATERNAL AND ADOLESCENTS’ CANNABIS USE AMONG THE INUIT OF NUNAVIK
Authors: Simard Alexandra , Forget-Dubois Nadine , Muckle Gina , Belanger Richard
Published: May 2018

BACKGROUND

Inuit from Nunavik, Northern Quebec, show some of the highest prevalence of cannabis use in the world, particularly for youths. Based on the ecological system theory, family (the more proximal unit to adolescents) is susceptible to influence their behaviors the most.

OBJECTIVES

The aim of this study was to examine, among the Inuit population from Nunavik, the association between maternal use of cannabis and the subsequent use of this substance by their children while adolescents.

DESIGN/METHODS

This study was based on longitudinal data from the Nunavik Child Development Study (endorsed by community and public health stakeholders; approved by ethic research boards from 2 North American Universities). We use data from two follow-ups, between 2005 and 2010 (T1: time 1 – children aged between 8–15 years-old), and between 2012 and 2016 (T2: time 2 – adolescents aged 16–21 years-old). Structured interviews were used to assess cannabis use and other concomitant variables among mothers at T1, and for adolescents at T2. Our final sample included 184 mother-adolescent dyads. Hypotheses that exposure to an increased frequency of maternal cannabis use at the end of childhood will be associated with 1) earlier initiation of cannabis during adolescence, and 2) will increase the frequency of cannabis use by youths, were tested with Cox regression analyses and ordinal regressions, taking into account socioeconomic status (T1), gender, and student status of adolescents(T2).

RESULTS

About seven out of ten Inuit adolescent participants reported having used cannabis during the previous year of T2, and about 45% of users consumed daily. Among the adolescents who answered the initiation age question, 60% had used it before reaching 14 years. Associations between mother’s frequency of cannabis use at T1 and 1) adolescents’ initiation age to cannabis and 2) adolescents’ frequency of cannabis use were not statistically significant. Higher socioeconomic status and student status were associated with lower risk of cannabis use by youths.

CONCLUSION

Among a group of adolescents where cannabis use is highly prevalent, we were unable to highlight the impact from maternal substance use on subsequent behavior of their children while adolescents. Yet, key determinants already known to influence cannabis use among southern populations seem to be also relevant to the Inuit population from Nunavik. Common Aboriginal wisdom that it takes a village to raise a child seems to apply to cannabis use as well.
CLINICIAN KNOWLEDGE, COMFORT, AND PERCEIVED BARRIERS IN TREATING TRANS PATIENTS WITHIN A TERTIARY CARE PEDIATRIC CENTRE
Authors: Butler Kat , Johnson Natasha
Published: May 2018

BACKGROUND

Historically, there have been numerous barriers to trans people - including youth - in terms of their access to health care in a variety of settings. Trans-specific care for youth is sparse across the country with long wait lists to access care. Further, there is a known period of vulnerability for trans people who have decided to access transition support and have not yet received it.

OBJECTIVES

This needs assessment survey was a first step to assess what training clinicians have received regarding working with trans patients, how comfortable they are working with trans patients, and what might be barriers to providing good care to trans patients. Addressing clinician perceived barriers may improve access to care.

DESIGN/METHODS

A 36-question survey was distributed electronically to faculty within the departments of paediatrics and psychiatry and to paediatric residents. The anonymous survey was designed to take approximately 10 minutes. The questions covered the topics of training, comfort, and practice. Data was analysed descriptively, and will be used to inform future educational sessions. Results are reported in the following areas: Training, Comfort, Practice, Barriers and Facilitators.

RESULTS

38 clinicians completed the survey (29 faculty, 7 residents). 16% reported having received training in trans-specific care. 86% indicated interest in training in trans-related care, with the majority favouring grand rounds (74%) and online modules (71%) as learning formats.

22 respondents (58%) felt ‘comfortable’ or ‘very comfortable’ in assessing overall health of trans patients. More than 40% of respondents responded ‘not applicable’ to questions about prescription of puberty blockers/exogenous sex hormones, indicating that they consider this outside of their scope of practice.

82% of respondents agreed that they were aware of colleagues to whom they could refer trans patients. Two respondents (5%) indicated that they regularly provide trans-specific care. One respondent (3%) indicated having a religious and/or cultural objection to providing trans-specific health care. Most respondents reported using patients’ preferred names and gender in verbal communication (89%) and when documenting (78%). 68% of respondents indicated that they would mirror language used by patients or try to use gender-neutral language when discussing anatomy.

Ten participants with experience in caring for trans patients identified a variety of facilitating factors. It was noted that this is “rewarding work” and “very much needed”. 76% cited a ‘lack of training in trans-specific care’ as the primary barrier.

CONCLUSION

Information collected from the survey will assist us in creating appropriate educational sessions for the relevant departments. It is hoped that addressing clinician perceived barriers will facilitate better access to care amongst a group of patients that has historically been marginalized within health care.
PREPAREDNESS OF CANADIAN PHYSICIAN OFFICES FOR PAEDIATRIC EMERGENCIES
Authors: Weeraratne Anushka , Jeong Dayae , Verma Suhrata , Atalla Marina , Hassan-Ali Mohammed , Kam April J
Published: May 2018

BACKGROUND

Studies in the US have demonstrated that many primary care staff and offices are inadequately prepared for paediatric emergencies. Although the Canadian Paediatric Society (CPS) recently reaffirmed their “Guidelines for Paediatric Emergency Equipment and Supplies for a Physician’s Office”, no evaluation has been made regarding the impact of publishing these recommendations, or on the state of preparedness for paediatric emergencies in family physician offices.

OBJECTIVES

The aim of this study was to evaluate awareness of and adherence of family physicians in Ontario to the CPS guidelines on preparedness for paediatric emergencies.

DESIGN/METHODS

We conducted a province-wide, cross-sectional survey of 749 randomly selected family physicians. Participants were asked to complete a 14-question survey regarding clinic characteristics, incidence of paediatric emergencies, and preparedness of the clinic in the case of a paediatric emergency. Ethics approval was obtained from the regional Ethics Review Board.

RESULTS

94 physicians responded to our survey (response rate of 13.1%). 68.1% of respondents reported seeing more than 10 children per week, and 59.6% and had experienced at least one paediatric emergency in the past year. The proportion of physicians reporting paediatric emergencies within the last year increased with the number of children seen - 37.9% of physicians who saw fewer than 10 children per week reported an emergency, compared to 100.0% of those who saw more than 40 children per week.

Only 4.3% respondents reported that they were unaware of the CPS guidelines on paediatric emergency preparedness. Although 85.1% of respondents were aware of the guidelines, only 10.6% of respondents had read them. Of the physicians who were aware of but had not read the guidelines, 2.5% engaged in mock code sessions, 27.8% were up-to-date on Paediatric Advanced Life Support (PALS), 1.3% had written protocols outlining safe transport of children to hospitals, and 41.8% stocked half or more of the recommended supplies. In comparison, of the physicians who had read the guidelines, 20.0% engaged in mock code sessions, 50.0% were up-to-date on PALS, 10.0% had written protocols, and 70.0% stocked half or more of the recommended supplies.

CONCLUSION

A large proportion of respondents had experienced at least one paediatric emergency in the past year, but were overall underprepared. There was a discrepancy between physicians who were aware of the CPS guidelines on emergency preparedness (85.1%), and those who have actually read them (10.6%), though offices with the latter were more adherent to the guidelines’ recommendations. It will be important for CPS to consider how to further advocate for paediatric emergency preparedness in clinics that see children regularly.
DEVELOPMENT OF A STANDARDIZED APPROACH FOR THE ASSESSMENT OF BOWEL AND BLADDER DYSFUNCTION IN COMMUNITY PAEDIATRICS
Authors: Wang Rebecca , Kanani Ronik , Mistry Niraj , Bardisi Yara El , Santos Joana Dos
Published: May 2018

BACKGROUND

Bowel and bladder dysfunction (BBD) is a common yet underdiagnosed paediatric condition that describes a constellation of lower urinary tract symptoms (LUTS) associated with constipation and/or encopresis. Many children with BBD have co-morbid neuropsychiatric symptoms (e.g., inattention, anxiety) and psychosocial stressors. Thus, clinical assessment of behavioural symptoms and social risk factors in this population is essential. However, there is no standardized approach for a comprehensive assessment of BBD.

OBJECTIVES

We aim to develop and evaluate physician and parent perceptions with 1) a standardized BBD history and physical intake form for physicians, and 2) a parent-reported intake form.

DESIGN/METHODS

From June to Oct 2017, a quality improvement study was conducted in the BBD network, an existing paediatric collaborative initiative consisting of 7 community sites with support of the paediatric urology division in a tertiary hospital. Based on literature review and expert opinions, a standardized intake form was developed for BBD assessment with targeted questions for LUTS, constipation, behavioural, dietary, and psychosocial history, along with a physical exam checklist for neurological red flags. Further, a shorter parent-reported intake questionnaire was developed to clarify patterns of dysfunctional voiding symptoms, dietary recall and stool history. Both forms underwent usability testing and iterative refinement. Prior to clinic, families of children referred for BBD were mailed an intake package for completion. During the clinic, physicians were asked to use the standardized intake form for new referrals. Afterwards, both physicians and parents were given anonymous surveys to evaluate their perceptions of the intake process.

RESULTS

A total of 8 physicians and 20 parents responded, with 60% of patients being between ages 4–10 and 55% male. Physicians found the standardized intake form to be a useful guide that reminded them to ask about specific urinary symptoms (88% of the time), constipation (75%), and psychosocial history (76%). The majority of physicians (75%) agreed they would use the intake form again and recommended its implementation. Further, parents responded positively by agreeing that the intake package was easy to complete (65%), felt included in care decisions (95%), and had questions answered appropriately (100%).

CONCLUSION

In assessment of BBD, a standardized intake form can help guide physicians to efficiently gather a comprehensive history, rule out red flags, and screen for psychosocial risk factors. With refinements, it can potentially help create a common clinical experience and empower more community paediatricians to manage BBD in the future.
UNDERSTANDING DISCHARGE COMMUNICATION BEHAVIOURS IN PEDIATRIC EMERGENCY CARE
Authors: Curran Janet , Bishop Andrea , Plint Amy
Published: May 2018

BACKGROUND

One of the most important transitions in the continuum of care for children is discharge to home. However, optimal discharge communication between healthcare providers and parents who present to the emergency department (ED) with their children is not well understood. Current research regarding discharge communication is equivocal and predominantly focused on evaluating different delivery formats or strategies with little attention given to communication behaviours or the context in which the communication occurs.

OBJECTIVES

The aim of this mixed methods study was to characterize the process and structure of discharge communication in a paediatric ED context.

DESIGN/METHODS

Real-time video observation methods were used in two academic paediatric EDs in Canada. Parents who presented with their child to the ED with one of six illness presentations, a Canadian Triage Acuity Score of 3 to 5, and English speaking, were eligible to participate. All ED physicians, learners, and clinical staff members were also eligible to participate. Provider-parent communication was analyzed using the Roter Interaction Analysis System (RIAS) to code each utterance. Parent comprehensive was evaluated using a follow-survey 72 hours after discharge.

RESULTS

A total of 106 unique ED visits involving six illness presentations: abdominal pain (n=23), asthma (n=6), bronchiolitis (n=4), diarrhea/vomiting (n=20), fever (n=27), and minor head injury (n=26) were video recorded. The average length of stay in the ED was 3 hours, with an average of three provider-parent interactions per visit. Interactions ranged in time from less than one minute to 29 minutes, with an average of six minutes per interaction. A total of 34,544 unique utterances were coded across all interactions. The majority of patient visits were first-time visits for the illness presentation (63.2%). Physicians most commonly gave medical information (22.9%) or asked close-ended medical questions (9.4%), whereas nurses most commonly gave orientation instructions (20.9%). Medical trainees were most likely to employ active listening techniques (e.g. back channels, 14.2%). Communication that included post-discharge instructions for parents comprised 8.5% of all utterances. Overall, providers infrequently assessed parental understanding of information (2.0%). Parent satisfaction with the amount of information communicated was generally high (89.6% agreed or strongly agreed).

CONCLUSION

This is the first discharge communication study to be conducted in a paediatric ED context using video observation methods. Provider-parent communication was predominantly characterized by the exchange of medical information, with little time devoted to adequately preparing parents to care for their child at home. Greater assessment of parental comprehension is needed to ensure that parents understand important instructions and know when to seek further care.
THE EFFECTIVENESS OF VIDEO DISCHARGE INSTRUCTIONS FOR ACUTE OTITIS MEDIA IN CHILDREN: A RANDOMIZED CONTROLLED TRIAL
Authors: Belisle Sheena , Dobrin Andrei , Elsie Sharlene , Ali Samina , Brahmbhatt Shaily , Kumar Kriti , Jasani Hardika , Ferlisi Frank , Bertram Kaitlyn , Poonai Naveen
Published: May 2018

BACKGROUND

Pain, the most identifiable feature of acute otitis media (AOM), is undertreated with 30% of children ≤ 2 years experiencing pain, fever, or both for up to 7 days, highlighting the importance of effective caregiver education.

OBJECTIVES

We sought to determine if video discharge instructions were associated with improved symptomatology, functional outcomes, and knowledge compared to a paper handout.

DESIGN/METHODS

We conducted a randomized controlled superiority trial comparing video discharge instructions (Easy Sketch Pro3TM) on management of pain and fever to a paper handout detailing the same. We included primary caregivers of children 6 months to 5 years presenting to the emergency department (ED) with a clinical diagnosis of AOM. The primary outcome was symptomatology using the Acute Otitis Media Severity of Symptom (AOM SOS) score between 48 and 72 hours. The 7-item self-report AOM-SOS is scored from 0 to 13 with a higher score indicating more symptomatology. Secondary outcomes included knowledge gain using a 10-item survey, days of daycare/school/work missed, and recidivism.

RESULTS

A total of 219 caregivers were randomized and 149 completed the 72-hour follow-up (72 paper and 77 video). Participants were primarily mothers (175/219, 79.9%); 136/219 (62.1%) completed post-secondary education and 147/219 (67.1%) had previously cared for a child with AOM. Children included 107/219 (48.6%) females with an overall mean (SD) age of 2.9 (2.8) years. Caregivers did not offer analgesia to 41/219 (18.7%) of children. The median (IQR) AOM-SOS score in the video group was significantly lower than the paper group, even after adjusting for pre-intervention AOM-SOS and medication (analgesics and antibiotics) given by caregivers [8 (7,11) versus 10 (7,13), respectively, p = 0.004]. There were no significant differences between video and paper in the mean (SD) number of correct answers given on the post-intervention survey [9.2 (1.3) versus 8.8 (1.8), respectively, p = 0.07], mean (SD) number of children that returned to a health provider [8/77 versus 10/72, respectively, p = 0.49), mean (SD) number of daycare/school missed by child [1.2 (1.5) versus 1.1 (2.1), respectively, p = 0.62, mean (SD) number of work missed by caregiver [0.5 (1) versus 0.8 (2), respectively, p = 0.05].

CONCLUSION

Children of caregivers with AOM who received a five-minute video detailing the identification and management of pain and fever experienced less symptomatology compared to a paper handout. Our findings suggest that video discharge instructions in the ED are effective for caregiver education and should be used routinely.
KINDERGARTEN-AGE QUALITY OF LIFE OUTCOMES AFTER LIVER TRANSPLANTATION AT <6 YEARS
Authors: Wong Kerry , Joffe Ari R , Yap Jason , Acton Bryan , Dinu Irina , Moez Elham Khodayari , Robertson Charlene MT
Published: May 2018

BACKGROUND

Given the improved mortality after paediatric liver transplantation, health related quality of life (HRQL) is an important outcome measure and provides valuable information for families.

OBJECTIVES

To determine the HRQL of kindergarten-age children who have undergone liver transplantation at age <6 years old.

DESIGN/METHODS

Between 1999–2014, all paediatric liver transplant recipients at our center were enrolled in this ethics board approved, longitudinal inception-cohort study. Following informed consent, HRQL was measured using the parent completed Pediatric Quality of Life Inventory, version 4.0 (PedsQL). The association between pre-transplant, transplant, and post-transplant variables and HRQL was examined using multiple regression analyses. Scores were compared to normative scores for HRQL in 8700 children [total PedsQL 82.2 (15.5), psychosocial summary 81.2 (15.3), and physical summary 84.0 (19.7)] and to scores for 130 children with surgical congenital heart disease from early infancy [81.1 (13.9), 77.5 (16.4), and 86.4 (15.3) respectively].

RESULTS

78 liver transplants for children <6 years of age were performed; 69 patients (88.5%) survived, and all (100%) were assessed at 55.4 (7.2) months of age: 38 with biliary atresia, 11 with acute liver failure, 11 with cholestasis, and 9 with metabolic disease. The mean total PedsQL was 75.6 (SD=15.6), psychosocial summary 72 (15.9) [a composite of emotional functioning 73.8 (16.4), social functioning 74.6 (19.2), and school functioning 70.6 (19.1)], and physical summary 78.2 (20.9). These composite scores were all statistically significantly different from population norms (p<0.001, <0.001, 0.026 respectively) and surgical congenital heart disease patients (p=0.015, 0.029, 0.005). The proportion having a score >1 (expected 15.9%) and >2 (expected 2.27%) SD below population norms were: total PedsQL 20.6% and 11.8%, psychosocial summary 32.3% and 11.3%, and physical summary 19.1% and 7.4%. Having a wait-list status 1 on the Canadian System of Classification was associated with higher total PedsQL (Effect size 9, 95%CI 2, 16; p=0.018) and physical summary (Effect size 15, 95%CI 16, 79; p=.004). None of the perioperative variables were predictive for psychosocial summary. Variables not associated with PedsQL included metabolic disease, PELD score, growth failure, any post-operative severe complication, age at transplant, weight at transplant, socioeconomic status, and year of transplant.

CONCLUSION

HRQL after liver transplantation is lower than population norms and congenital heart disease patients. More research is needed to determine why HRQL is low in order to improve the lives of these children.
IMPACT OF HIGH LEVELS OF CONTINUOUS POSITIVE AIRWAY PRESSURE ON CARDIAC OUTPUT IN PRETERM NEONATES: A PROSPECTIVE PHYSIOLOGICAL STUDY
Authors: Mukerji Amit , Muzafar Wahab Abdul Gani , Mitra Souvik , Mondal Tapas , Paterson Debie , Beck Jennifer , Fusch Christoph
Published: May 2018

BACKGROUND

Many NICUs employ high (>8 cmH2O) positive end-expiratory pressures (PEEP) on nasal continuous positive airway pressure (NCPAP) to prevent intubation and associated ventilator-induced lung injury, despite limited safety/efficacy data.

OBJECTIVES

This study sought to evaluate the physiological impact of high NCPAP PEEP.

DESIGN/METHODS

Fifteen preterm neonates at postmenstrual age ≥32 weeks (without congenital anomalies or acute intercurrent illness) on NCPAP PEEP of 5 cmH2O were enrolled. PEEP was increased by 2 cmH2O increments until 13 cmH2O. At each increment, following 5 minutes washout, cardiac output (aortic velocity-time integral x heart rate) and cardiorespiratory parameters including blood pressure, heart rate, respiratory rate were measured over 10 minutes. Predefined cut-off values for changes in cardiorespiratory parameters were used as termination criteria. Data are presented as mean (SD), and were compared using one-way ANOVA.

RESULTS

The mean GA, age at study, and weight of subjects were 27.4 (2.6) weeks, 58.5 (35.5) days, and 2.3 (0.6) kg, respectively. Cardiac output (mL/kg/min) at PEEPs of 5, 7, 9, 11, and 13 cmH2O were not different at 295 (75), 290 (66), 281 (69), 286 (73), and 292 (58), respectively (P=0.986), as shown in Figure 1a. Importantly there were also no differences in either aortic velocity-time integral or heart rate over these PEEP ranges (Figures 1b and 1c). There were no significant differences in cardiorespiratory parameters; no subjects met cut-off criteria. Data collection was terminated in 2 subjects after PEEP 9 cmH2O due to lung over-distension subjectively noted on echocardiogram.

CONCLUSION

High levels of NCPAP PEEP were well tolerated for short durations. Further physiological and clinical research is required on safety/efficacy in neonates with more severe lung disease, as well as its impact over longer durations.
CEREBRAL OXYGENATION AND PERIPHERAL OXIMETRY IN EXTREMELY PRETERM INFANTS WITH PULMONARY AND/OR CEREBRAL INTRAVENTRICULAR HEMORRHAGE IN THE FIRST 72 HOURS OF LIFE
Authors: Beausoleil Thierry , Janaillac Marie , Barrington Keith , Raboisson Marie-Josée , Karam Oliver , Lapointe Anie , Dehaes Mathieu
Published: May 2018

BACKGROUND

Extremely premature infants born <28 weeks of gestation are at higher risk of pulmonary (PH) and cerebral intraventricular (IVH) hemorrhage due to immature cardiovascular and transitioning physiology. Non-invasive monitoring has the potential to detect early abnormal circulation.

OBJECTIVES

To explore time-frequency relationships between cerebral oxygenation and peripheral oximetry.

DESIGN/METHODS

Near infrared spectroscopy cerebral regional haemoglobin oxygen saturation (CrSO2), preductal peripheral perfusion index (PI), heart rate (HR), capillary oxygen saturation (SpO2), and blood pressure (BP) were monitored in the first 72h of life. Patients were grouped in infants with PH and/or IVH (n=8) and controls (n=10). Signals were decomposed in wavelets allowing the analysis of localized variations of power. This approach allowed to quantify the common power and determine the duration of significant cross-correlation, phase and coherence between each pair of signals. Groups were compared with Wilcoxon tests.

RESULTS

Figure 1 shows an example of CrSO2 and PI, and their cross-correlation, phase (semblance) and coherence in a control (left column) and a PH-IVH patient (right column). Durations of significant cross-correlation between CrSO2 and HR (p<0.01), and CrSO2 and SpO2 (p=0.02) were significantly lower in PH-IVH infants compared to controls. The duration of significant anti-phase between CrSO2 and SpO2 (p=0.01) and the duration of significant coherence between PI and BP (p=0.03) were also significantly lower in PH-IVH infants compared to controls. These differences may indicate a disruption in auto-regulation, which is currently incompletely understood in this population.

CONCLUSION

This study is the first to apply time-frequency analysis to simultaneous NIRS and preductal peripheral oximetry in extremely preterm infants early in life. Significantly lower durations of cross-correlation (CrSO2 with HR and SpO2), anti-phase (CrSO2, SpO2) and coherence (PI, BP) in PH-IVH patients may reflect early abnormal circulation. Our results show the potential of non-invasive monitoring to identify premature infants at-risk of early PH-IVH.
DOES THE TIMING OF MEDICAL TREATMENT OF PATENT DUCTUS ARTERIOSUS (PDA) HAVE AN IMPACT ON THE SUCCESS OF TREATMENT IN PRETERM INFANTS?
Authors: Kaur Sharandeep , Stritzke Amelie , Soraisham Amuchou
Published: May 2018

BACKGROUND

Hemodynamically significant patent ductus arteriosus (hsPDA) is the most common cardiovascular problem of prematurity often treated with nonsteroidal anti-inflammatory drugs (NSAIDs-indomethacin and ibuprofen). There is limited data on the effect of postmenstrual age (PMA) at the time of start of treatment on response rates to NSAIDs in preterm infants with hsPDA.

OBJECTIVES

The objective of our study was to examine the effect of PMA at the start of treatment on response to NSAIDs in hsPDA.

DESIGN/METHODS

In this retrospective cohort study, we included infants with gestational age (GA) ≤ 32 weeks admitted to a tertiary NICU between January 2014 and December 2016, who had received one or more doses of NSAIDs for the treatment of hsPDA. Positive response (responders) to NSAIDs was considered if post treatment echocardiogram showed that PDA was either closed or small, and did not require further medical or surgical treatment. We compared baseline characteristics between responders and non-responders using univariate and multivariable logistic regression.

RESULTS

A total of 183 infants received 257 courses (one, two and three courses in 183, 62 and 12 neonates respectively) of NSAIDs. Positive response rate was overall 60%, and 66%, 48%, and 50% for the first, second, and third course respectively. The baseline characteristics were comparable between responders and non-responders except for higher GA, birth weight (BW), and earlier (<14 days of life) start of NSAIDs in responders. After adjusting for BW, Apgar score and gender, GA at birth but not the PMA, was associated with positive response to NSAIDs (aOR 1.24, 95% CI 1.07, 1.42 for GA; aOR 0.92, 95% CI 0.79, 1.07 for PMA). Early NSAIDs treatment was associated with increased odds of PDA closure (aOR 1.95; 95% CI 1.00–3.8).

CONCLUSION

Gestational age is the strongest predictor for response to NSAIDs in hs-PDA and PMA at treatment does not affect efficacy of NSAIDs.
CHROMOSOMAL MICROARRAY TESTING IN A DEVELOPMENTAL PAEDIATRICS SETTING
Authors: Jenkin Brock , Mitchell Clare
Published: May 2018

BACKGROUND

Current guidelines recommend chromosomal microarray (CMA) testing as a first line etiologic investigation for developmental disorders such as intellectual disability or autism spectrum disorder (ASD). How often a copy number variation (CNV) is found, a definitive etiologic diagnosis is made and a change in clinical management occurs has not been well studied in a community setting.

OBJECTIVES

The study objective was to examine the real world use of CMA testing in a developmental paediatric setting: the prevalence of positive results and management decisions.

DESIGN/METHODS

This was a retrospective, descriptive study. The charts of 170 children seen by a single developmental paediatrician in a small city over a 7 year period (2010 - 2017) were reviewed. Referrals were received from both urban and rural communities. Information regarding reason for referral, clinical diagnosis, requests for CMA testing, test results and subsequent management decisions were extracted. The patient age ranged from 1 to 18 years (average 5.1 years). Children were referred for a wide variety of developmental and behavioural problems. Developmental delay, disruptive behavior, possible autism spectrum disorder or speech delay were the most common reasons for referral. Children were considered for CMA testing according to published guidelines. The most common clinical diagnoses in referred children were attention deficit hyperactivity disorder (ADHD), ASD and global developmental delay (GDD). Clinical management decisons were obtained from the medical chart and included follow-up visits.

RESULTS

CMA testing was recommended for 78 children, of which 65 had CMA testing completed (83%). Of these, 15 (23%) had an abnormal result and 6 (9%) were deemed pathogenic. The most common finding was a CNV at 2p16.3 in 2 children (3%). Of the children with pathogenic CNVs, 3 (50%) had more than one CNV. One child had a previously diagnosed trisomy X. One child with normal CMA had further testing, and a genetic diagnosis of atypical Rett Syndrome was made. The primary management decisions based on the CMA test results included parent education, genetic counselling and prognosis clarification.

CONCLUSION

In a developmental paediatrics setting, the use of CMA testing for first-line etiologic assessment in children with developmental disorders obtains positive results in close to 10% of tested children. This is similar to previously published results. Approximately 1/6 tested children had results of uncertain significance which require further study over time.
THE USE OF PFA-100 IN PEDIATRICS IN THE INVESTIGATION OF HEMOSTATIC DISORDERS, A RETROSPECTIVE COHORT STUDY
Authors: Caron Andréane , Gélinas Marie-Christine
Published: May 2018

BACKGROUND

The screening of hemostatic disorders in paediatrics is challenging because often children have never undergone hemostatic challenges. The PFA-100 was introduced as an alternative to the in vivo bleeding time test and is now routinely used in the screening of these disorders. Numerous studies have assessed its limits and there remains uncertainty regarding its utility.

OBJECTIVES

The goal of our study was to describe the utilization of the PFA-100 in a tertiary paediatric center, to evaluate its accuracy and to determine the average cost generated by the investigation following an abnormal result.

DESIGN/METHODS

We did a retrospective descriptive study by reviewing the charts of 250 paediatric patients who had a PFA-100 done in our center over a one year period. Accuracy and reliability of the PFA-100 were calculated with a ROC curve (95% CI).

RESULTS

Of 225 patients included in our study, 99 (44%) PFA-100’s were done in a preoperative context, and in average, each patient had 1.52 PFA-100’s (range 1–6). Personal hemorrhagic diathesis was not described in 59 patients (26.2%). 91 patients (40.4%) had an abnormal PFA-100 result. Further investigation revealed no disorder in 55 patients (60.4%), von Willebrand disease in 20 patients (8.9%) and non-specific platelet function defects in 15 patients (6.7%). No severe platelet function defects were found in our study cohort. There was a high variability of the PFA-100 result in a same patient (coefficient of variation 15.8%). A higher cut-off value of 140 seconds for PFA-100 col_EPI (AUC 0,87) and 111 seconds for PFA-100 col_ADP (AUC 0,68) produced more accurate sensitivity (89% and 74%), specificity (78% and 63%) and positive (44% and 49%) and negative (97% and 84%) predictive values, compared to our center’s threshold of 130 seconds and 100 seconds respectively. The average cost per patient for the investigation following an abnormal PFA-100 was 434,92$ ± 232,66 (CI 95%).

CONCLUSION

PFA-100 was mainly used in a preoperative context in our study and patient history was often incomplete. The PFA-100 shows high sensitivity and negative predictive value making it a potentially good screening test however it is to be used with caution considering the high variability of results in a single patient.
VARICELLA-RELATED HOSPITALIZATIONS IN IMPACT CENTERS AFTER INTRODUCTION OF 1- AND 2-DOSE VARICELLA VACCINATION PROGRAMS BETWEEN 2000 AND 2015
Authors: Tan Ben , Bettinger Julie , McConnell Athena , Thibault Roseline , Jadavji Taj , Li Anita , Vaudry Wendy , Halperin Scott , Samson Heather
Published: May 2018

BACKGROUND

The Canadian Immunization Monitoring Program ACTive (IMPACT) conducts active surveillance for varicella-related hospitalizations in 12 paediatric tertiary care centres, starting Apr 1999.

OBJECTIVES

This study was to determine the hospitalization trends in relation to vaccine program introduction in the provinces, between 2000 and 2015.

DESIGN/METHODS

Demographic data of varicella cases from the 12 IMPACT centers were analysed. The cases were subdivided into three groups (A, B and C), combining provinces with approximately the same time periods at introduction of 1-dose and 2-dose vaccination programs (see results).

RESULTS

The mean number of hospitalizations per year declined during the 1-dose and 2-dose eras in each group of provinces, as follows:

CONCLUSION

Varicella hospitalizations declined by 63–77% but reached a plateau during the 1-dose vaccine era. There was a further decline to 81–89% of baseline in the two groups which instituted 2-dose vaccine programs in 2011–12, but too early to document any further decline in Group C, the provinces which initiated 2-dose programs in 2014–16. Introduction of 2-dose programs appear to have provided further benefit, after 1-dose programs in Canada.
TEN EPIQ STEPS TO IMPROVING CARE AND OUTCOMES IN RURAL TANZANIA
Authors: Ali Ismat , Mwaikasu Lusako , McMillan Doug , Aziz Khalid , Brenner Jennifer , Matovelo Dismas , Singhal Nalini
Published: May 2018

BACKGROUND

Maternal and child health (MCH) remains a priority globally and in Mwanza Region, Tanzania, where under-five mortality exceeds 100/1000 live births. Facility-based Quality Improvement (QI) is critical to enhancing MCH service delivery. The ‘Evidence-based Practice for Improving Quality (EPIQ)’ approach aims to equip clinical providers with basic QI understanding and skills. In Canada, EPIQ introduction has led to significant newborn intensive care improvements.

OBJECTIVES

To adapt, pilot and evaluate an EPIQ workshop for rural Tanzania.

DESIGN/METHODS

The 10-step EPIQ process is introduced through a practical, clinically-focused, hands-on workshop. A team of Canadian EPIQ experts with African partnership experience initially adjusted the EPIQ package for a Tanzanian pilot. They then facilitated a one day EPIQ training-of-trainers to QI-interested Tanzanian university faculty and health managers. The new facilitators then delivered the training to selected local health providers. The EPIQ training was evaluated through facilitator and provider (1) pre/post QI-focused knowledge tests, (2) pre/post QI confidence surveys and (3) post-workshop satisfaction surveys. Post training semi-structured group interviews also collected feedback on workshop content and format from facilitators. Written surveys and tests were analyzed using descriptive statistics and aggregate scores; semi-structured interview notes were reviewed for key themes, recommendations and representative quotations.

RESULTS

Workshop content including flipcharts, workbooks and locally-relevant cases were prepared. In September 2017, workshops were delivered to 16 facilitators and 29 providers in Mwanza. Post-workshop, overall knowledge scores increased by +7.6% (facilitators) and +2.1% (providers). Aggregate confidence survey scores (5-point Likert) increased for both groups by a median increase of 1 (20%). Participant workshop satisfaction scores averaged 4.7/5. Interviews suggested indicated content was perceived as very relevant however too heavy for allocated time; certain wording was too complex for the language setting. Participants reported that the small group and hands-on format encouraged skill development and EPIQ tools equipped for critical thinking and problem solving. It was strongly recommended to increase workshop length to two days.

CONCLUSION

A clinically-focused, participatory EPIQ training was feasible and well-received in this setting. Despite self-reported confidence increase, a lacking associated knowledge increase may reflect content too complex, limited workshop duration or poor knowledge test face validity. Future considerations should include revised content and/or knowledge testing tool, extended length, and post-workshop implementation follow-up.