Sainte-Justine Hospital Research Articles

3. Asymmetric hypsarrythmia: An insight into the pathophysiology of infantile spasms. A retrospective cohort

Infantile spasms (IS) is a catastrophic epilepsy where treatment precocity improves outcome. Previous studies demonstrated an association between asymmetric hypsarrhythmia on EEG and ipsilateral hemispheric lesions on MRI, suggesting a possible role of cortical lesions in the initiation of IS. Epileptiform abnormalities appearing during early infancy have also been linked to IS emergence. We hypothesized that focal lateralized EEG abnormalities during the prehyspasrrhythmic period will be associated with asymmetric hypsarrhythmia at IS onset. We recruited a retrospective cohort of 80 infants, 7.4 ± 3.6 months old at the onset of hypsarrhythmia and IS, admitted to Sainte-Justine Hospital between 2007 and 2016. Seven infants showed an asymmetric hypsarrhythmia pattern and, as expected, all of them had lateralized lesions on MRI. Of the remaining 73, 42 had abnormal MRI, but only 3 infants had lateralized lesions (100% vs 7.1%, p Six had focal lateralized, while 23 multifocal abnormalities. The proportion of patients with focal abnormalities was higher in those who developed asymmetric compared to symmetric hypsarrhythmia (50% vs 13.3%, p Our data confirm the link between asymmetric hypsarrhythmia and lateralized MRI lesions. Moreover, we show that focal lateralized EEG abnormalities precede asymmetric hypsarrythmia, which supports the involvement of cerebral cortex in the IS genesis. More sensitive EEG biomarkers of high IS risk may help developing preventative treatments that will improve outcomes in IS.

Norepinephrine infusion improves haemodynamics in the preterm infants during septic shock.

This study evaluated the clinical and haemodynamic effects of norepinephrine infusion in preterm infants. The effects of norepinephrine therapy for refractory hypotension were evaluated in preterm infants between April 2009 and April 2011 at the neonatal intensive care unit of Sainte-Justine Hospital, Montreal, Quebec. Changes in haemodynamics and clinical parameters were analysed eight hours before and eight hours after the start of norepinephrine infusion, and eight hours after its cessation. During the study, 30 preterm infants at a mean gestational age of 26.5±2.6weeks (median: 25.7, 23.4-34) and birthweight of 903±437g (median 827, 450-2550) received norepinephrine infusion for neonatal septic shock. After eight hours of treatment, mean blood pressure, urine output and FiO2 significantly improved. Eight hours after cessation of norepinephrine infusion, the number of patients treated with other inotropes decreased significantly, 24 patients (80%) had normal mean blood pressure and 27 patients (90%) had normal urine output. Norepinephrine therapy could be considered to improve blood pressure and urine output during neonatal septic shock in preterm infants. Further studies are needed to prove the efficacy and safety of norepinephrine infusion in neonates.

Revisiting the psychometric properties of the Scoliosis Research Society-22 (SRS-22) French version

BackgroundAdolescent idiopathic scoliosis (AIS) is among the most common spinal deformities affecting adolescents. The Scoliosis Research Society-22 questionnaire is commonly used to assess health-related quality of life in AIS patients, including pain.The objective of this study is to verify the psychometric properties of the Scoliosis Research Society-22 French version (SRS-22fv) questionnaire.MethodsA prospective methodological design was used to verify the psychometric properties of the French version of the SRS-22fv. Participants were initially recruited from the orthopaedic scoliosis department at Sainte-Justine Hospital (Montreal, Canada) and completed the SRS-22fv and the SF-12 questionnaire. The SRS-22fv’s structure was evaluated through principal component analysis (PCA). Linear regression was used to assess convergent validity between the SRS-22fv and the SF-12.ResultsData was available from 352 participants with AIS. Most participants were female (87%, n = 307), and the average age was 14.3 (SD = 1.8) years. The mean thoracic and lumbar Cobb angles were 27.9° (SD = 3.3) and 23.6° (SD = 9.4), respectively. Overall, 71.4% (n = 252) of the participants presented with spinal pain. About one-third (29%) reported thoracic pain, and almost half (44%) experienced lumbar pain. The PCA identified four redundant items, which resulted in a modified 18-item questionnaire. In comparison to the original questionnaire, the modified version showed higher levels of internal consistency for four of the five factors, explained a greater proportion of the total variance (63.3%), and generated higher inter-item total correlations.ConclusionWe propose a shorter version of the SRS-22fv, thus the Canadian SRS-18fv, which showed an improved internal consistency and scale structure compared to the original SRS-22fv. We believe that this modified version would be better suited to assess the quality of life of adolescents with idiopathic scoliosis.

Using internal and external reviewers can help to optimise neonatal mortality and morbidity conferences

This study determined whether there was a difference in the conclusions reached by neonatologists in morbidity and mortality conferences based on their level of involvement in a case. All neonatal deaths occurring between August 2014 and September 2015 at the neonatal intensive care unit of Sainte-Justine Hospital, Montreal, Quebec, Canada, were reviewed by internal physicians involved in the case and external physicians who were not. The reviewers were asked to identify positive and negative clinical practice items and provide written recommendations. These were classified into eight categories and compared for each case. During the study, 55 patients died leading to 110 reviews and a total of 590 positive and negative items. Most items were in the communication (25.2%), ethical decision-making (16.7%) and clinical management (14.8%) categories. Both the internal and external reviewers were in agreement 48.5% of the time for positive items and 44.8% for negative items. There were 242 written recommendations, which differed significantly among the internal and external reviewers. Reviews of neonatal deaths by two independent reviewers, internal physicians and external physicians, led to different positive and negative practice items and recommendations. This could allow for a richer discussion and improve recommendations for patient care.

Head Ultrasound or MRI? The Role of Neuroimaging in the Assessment of Symptomatic and Asymptomatic Infants with Congenital CMV Infection

BackgroundDespite interest in universal screening for congenital CMV infection (cCMV), there is little consensus on the management of asymptomatic newborns, and on the role or type of neuroimaging to be performed in infected infants. The objective of this study was to assess the concordance between head ultrasound (US) and magnetic resonance imaging (MRI) in identifying neurological abnormalities in infants with cCMV infection.MethodsRetrospective review of infants with cCMV infection, referred to the Centre Maternel Infantile d’Infectiologie Congenitale at Sainte-Justine Hospital Center in Montreal, between 2008 and 2016. This was a secondary analysis of a previous study and included only patients who underwent baseline CMV qPCR and had neuroimaging records available.ResultsOf 46 cases of cCMV infection, 10 were categorized as clinically asymptomatic, and were identified following maternal seroconversion during pregnancy (8) or during targeted screening of HIV exposed newborns (2). Twenty-eight patients had US followed by MRI, 4 underwent US followed by CT (3) or CT and MRI(1), and 11 had only 1 imaging modality (US, CT, or MRI). Among cases with sequential US and MRI, US was performed at a mean of 13 days (SD ±19) and MRI at a mean of 70 days of age (SD ±164). In 20/28 cases, US and MRI were concordant (9 abnormal, 11 normal). In 4 cases, US was normal and MRI later found to be abnormal; however in these 4 cases patients were clinically symptomatic and the initial imaging findings did not influence the decision to treat. In 4 cases, US was abnormal and subsequent MRI found to be normal; in 2 of these cases, patients were clinically symptomatic and the imaging findings did not influence the decision to treat. However, in 2 cases, the patients were clinically asymptomatic and categorized as symptomatic for treatment based only on the abnormal US findings.ConclusionIn this study, there was a discordance between MRI and US findings in 29% of infants with cCMV infection. While the addition of MRI to baseline head ultrasound did not influence the decision to treat in clinically symptomatic infants, the addition of MRI for infants with abnormal head US who are clinically asymptomatic could help refine treatment decisions in these cases.Disclosures All authors: No reported disclosures.

Platelet Transfusions in Pediatric Intensive Care.

To characterize the determinants of platelet transfusion in a PICU and determine whether there exists an association between platelet transfusion and adverse outcomes. Prospective observational single center study, combined with a self-administered survey. PICU of Sainte-Justine Hospital, a university-affiliated tertiary care institution. All children admitted to the PICU from April 2009 to April 2010. None. Among 842 consecutive PICU admissions, 60 patients (7.1%) received at least one platelet transfusion while in PICU. In the univariate analysis, significant determinants for platelet transfusion were admission Pediatric Risk of Mortality Score greater than 10 (odds ratio, 6.80; 95% CI, 2.5-18.3; p < 0.01) and Pediatric Logistic Organ Dysfunction scores greater than 20 (odds ratio, 26.9; 95% CI, 8.88-81.5; p < 0.01), history of malignancy (odds ratio, 5.08; 95% CI, 2.43-10.68; p < 0.01), thrombocytopenia (platelet count, < 50 × 10/L or < 50,000/mm) (odds ratio, 141; 95% CI, 50.4-394.5; p < 0.01), use of heparin (odds ratio, 3.03; 95% CI, 1.40-6.37; p < 0.01), shock (odds ratio, 5.73; 95% CI, 2.85-11.5; p < 0.01), and multiple organ dysfunction syndrome (odds ratio, 10.41; 95% CI, 5.89-10.40; p < 0.01). In the multivariate analysis, platelet count less than 50 × 10/L (odds ratio, 138; 95% CI, 42.6-449; p < 0.01) and age less than 12 months (odds ratio, 3.06; 95% CI, 1.03-9.10; p = 0.02) remained statistically significant determinants. The attending physicians were asked why they gave a platelet transfusion; the most frequent justification was prophylactic platelet transfusion in presence of thrombocytopenia with an average pretransfusion platelet count of 32 ± 27 × 10/L (median, 21), followed by active bleeding with an average pretransfusion platelet count of 76 ± 39 × 10/L (median, 72). Platelet transfusions were associated with the subsequent development of multiple organ dysfunction syndrome (odds ratio, 2.53; 95% CI, 1.18-5.43; p = 0.03) and mortality (odds ratio, 10.1; 95% CI, 4.48-22.7; p < 0.01). Among children, 7.1% received at least one platelet transfusion while in PICU. Thrombocytopenia and active bleeding were significant determinants of platelet transfusion. Platelet transfusions were associated with the development of multiple organ dysfunction syndrome and increased mortality.

Learning Neonatal Intubation Using the Videolaryngoscope: A Randomized Trial on Mannequins.

The use of the videolaryngoscope (VL) facilitates intubation in adults and children, but experience in neonates is scarce. The objective of this study was to compare the VL with the classic laryngoscope (CL) in acquiring the skill of neonatal endotracheal intubation (ETI) and evaluate transferability of skill from VL to CL. We hypothesize that, on a neonatal mannequin, the VL will be superior to the CL with regard to success rate and that the skill will be transferred from VL to CL. A randomized controlled trial was held at Sainte-Justine Hospital's simulation center. Third- and fourth-year medical students were randomized into group A, which used VL for the first phase and CL for the second phase, and group B, which used CL for both phases. Each subject performed 9 ETI on 3 simulated neonatal airways in each phase. Thirty-four students performed 612 intubations. Success in group A was higher than in group B in the first phase of the study (96.5% vs. 84.6%, P < 0.001). During phase 2, group A's success did not change significantly (91.7% vs. 96.5%, P = 0.07). Time to successful intubation was longer using the VL (27.6 vs. 15.6 seconds, P < 0.001), but there was no difference in phase 2 (12.5 vs. 10.2 seconds, P = 0.24). There were no esophageal intubations using the VL. Success rate of ETI on mannequins was improved, and esophageal intubations decreased while learning ETI using the VL compared with the CL. Once ETI is learned on mannequins using the VL, this skill is transferrable to the CL.

Multiple organ dysfunction syndrome in critically ill children: clinical value of two lists of diagnostic criteria.

BackgroundTwo sets of diagnostic criteria of paediatric multiple organ dysfunction syndrome (MODS) were published by Proulx in 1996 and by Goldstein in 2005. We hypothesized that this changes the epidemiology of MODS. Thus, we determined the epidemiology of MODS, according to these two sets of diagnostic criteria, we studied the intra- and inter-observer reproducibility of each set of diagnostic criteria, and we compared the association between cases of MODS at paediatric intensive care unit (PICU) entry, as diagnosed by each set of diagnostic criteria, and 90-day all-cause mortality.MethodsAll consecutive patients admitted to the tertiary care PICU of Sainte-Justine Hospital, from April 21, 2009 to April 20, 2010, were considered eligible for enrolment into this prospective observational cohort study. The exclusion criteria were gestational age < 40 weeks, age < 3 days or > 18 years at PICU entry, pregnancy, admission immediately after delivery. No patients were censored. Daily monitoring using medical chart ended when the patient died or was discharged from PICU. Mortality was monitored up to death, hospital discharge, or 90 days post PICU entry, whatever happened first. Concordance rate and kappa score were calculated to assess reproducibility. The number of MODS identified with Proulx and Goldstein definitions was compared using 2-by-2 contingency tables. Student’s t test or Wilcoxon signed-ranked test was used to compare continuous variables with normal or abnormal distribution, respectively. We performed a Kaplan–Meier survival analysis to assess the association between MODS at PICU entry and 90-day mortality.ResultsThe occurrence of MODS was monitored daily and prospectively in 842 consecutive patients admitted to the PICU of Sainte-Justine Hospital over 1 year. According to Proulx and Goldstein diagnostic criteria, 180 (21.4 %) and 314 patients (37.3 %) had MODS over PICU stay, respectively. Concordance of MODS diagnosis over PICU stay was 81.3 % (95 % CI 78.6–83.9 %), and kappa score was 0.56 (95 % CI 0.50–0.61). Discordance was mainly attributable to cardiovascular or neurological dysfunction criteria. The proportion of patients with MODS at PICU entry who died within 90 days was higher with MODS diagnosed with Proulx criteria (17.8 vs. 11.5 %, p = 0.038), as well as the likelihood ratio of death (4.84 vs. 2.37). On the other hand, 90-day survival rate of patients without MODS at PICU entry was similar (98.6 vs. 98.9 % (p = 0.73).ConclusionsProulx and Goldstein diagnostic criteria of paediatric MODS are not equivalent. The epidemiology of paediatric MODS varies depending on which set of diagnostic criteria is applied.Electronic supplementary materialThe online version of this article (doi:10.1186/s13613-016-0144-6) contains supplementary material, which is available to authorized users.

Exploring a New Simulation Approach to Improve Clinical Reasoning Teaching and Assessment: Randomized Trial Protocol.

BackgroundHelping trainees develop appropriate clinical reasoning abilities is a challenging goal in an environment where clinical situations are marked by high levels of complexity and unpredictability. The benefit of simulation-based education to assess clinical reasoning skills has rarely been reported. More specifically, it is unclear if clinical reasoning is better acquired if the instructor's input occurs entirely after or is integrated during the scenario. Based on educational principles of the dual-process theory of clinical reasoning, a new simulation approach called simulation with iterative discussions (SID) is introduced. The instructor interrupts the flow of the scenario at three key moments of the reasoning process (data gathering, integration, and confirmation). After each stop, the scenario is continued where it was interrupted. Finally, a brief general debriefing ends the session. System-1 process of clinical reasoning is assessed by verbalization during management of the case, and System-2 during the iterative discussions without providing feedback.ObjectiveThe aim of this study is to evaluate the effectiveness of Simulation with Iterative Discussions versus the classical approach of simulation in developing reasoning skills of General Pediatrics and Neonatal-Perinatal Medicine residents.MethodsThis will be a prospective exploratory, randomized study conducted at Sainte-Justine hospital in Montreal, Qc, between January and March 2016. All post-graduate year (PGY) 1 to 6 residents will be invited to complete one SID or classical simulation 30 minutes audio video-recorded complex high-fidelity simulations covering a similar neonatology topic. Pre- and post-simulation questionnaires will be completed and a semistructured interview will be conducted after each simulation. Data analyses will use SPSS and NVivo softwares.ResultsThis study is in its preliminary stages and the results are expected to be made available by April, 2016.ConclusionsThis will be the first study to explore a new simulation approach designed to enhance clinical reasoning. By assessing more closely reasoning processes throughout a simulation session, we believe that Simulation with Iterative Discussions will be an interesting and more effective approach for students. The findings of the study will benefit medical educators, education programs, and medical students.

Steroid Administration and Growth Impairment in Children with Crohn's Disease.

Growth impairment remains a major concern in children with Crohn's disease, but evidence remains unclear, in particular, whether steroid use is implicated. We aimed to (1) determine the frequency of temporary (TGI) and permanent (PGI) growth impairment in children administered steroids and (2) examine whether cumulative steroid administration was associated with TGI and/or PGI. A retrospective cohort study was performed in patients with Crohn's disease (<18 yr) administered steroids at the gastroenterology clinics of Sainte-Justine Hospital, Montreal. Steroid dosage, height during follow-up, adult height (after age 20), and parental heights were ascertained. Patients with height z score <-1.64 on more than 1 occasion before age 18 were considered as patients with TGI. Patients with adult heights <8.5 cm below the expected target heights were considered as patients with PGI. Association between steroid dosage and TGI/PGI was studied using logistic regression analyses. Data from the Swiss IBD Cohort Study were analyzed for comparison. A total of 221 children were studied. Approximately 19% (42/221) children were deemed as TGI, and 8/137 patients (5.8%) had PGI. TGI was associated with diagnosis at younger age (P value 0.002) and steroid administration at younger age (P value 0.001), but not with steroid dosage. Final adult height was associated with target height, but not with cumulative steroid dosage. Rates of PGI in the Swiss cohort were ∼ 9.1% in steroid users and 2.7% in nonusers. Most children with TGI attain normal adult heights. Cumulative steroid use does not seem to be associated with either TGI or PGI in children with Crohn's disease.

An Order Protocol for Respiratory Distress/Acute Pain Crisis in Pediatric Palliative Care Patients: Medical and Nursing Staff Perceptions.

An order protocol for distress (OPD), including respiratory distress and acute pain crisis, has been established for pediatric palliative care patients at Sainte-Justine Hospital (SJH). After discussion with the patient/his or her family, the OPD is prescribed by the attending physician whenever judged appropriate. The OPD can then be initiated by the bedside nurse when necessary; the physician is notified after the first dose is administered. The study objectives were to evaluate the perceptions and experience of the medical/nursing staff towards the use of the OPD. A survey was distributed to all physicians/nurses working on wards with pediatric palliative care patients. Answers to the survey were anonymous, done on a voluntary basis, and after consent of the participant. Surveys (258/548) were answered corresponding to a response rate of 47%. According to the respondents, the most important motivations in using the OPD were the desire to relieve patient's distress and the speed of relief of distress by the OPD; the most important obstacles were going against the patient's/his or her family's wishes and fear of hastening death. The respondents reported that the OPD was frequently (56%) or always (36%) effective in relieving the patient's distress. The respondents felt sometimes (16%), frequently (34%), or always (41%) comfortable in giving the OPD. They thought the OPD could never (12%), rarely (32%), sometimes (46%), frequently (8%), or always (1%) hasten death. Physicians were less favorable than nurses with the autonomy of bedside nurses to initiate the OPD before notifying the physician (p = 0.04). Overall, 95% of respondents considered that they would use the OPD in the future. Data from this survey shows that respondents are in favor of using the OPD at SJH and find it effective. Further training as well as support for health care professionals are mandatory in such palliative care settings.

Blood transfusion in acute and chronic pediatric settings: beliefs and practices.

Blood has been imbued with powerful connotations through history and across cultures. Currently bestowed with scientific meaning, blood nevertheless carries symbolic resonance. This study examines these representations among practitioners and sheds light on the clinical and nonclinical factors that guide blood transfusion (BT) decision-making in Quebec, Canada. With a qualitative exploratory study design, data were collected in the pediatric intensive care unit and the hematology-oncology unit of Sainte-Justine Hospital in 2009. A total of fifteen 1-hour-long semistructured interviews were conducted with physicians. Physicians affirm that the symbolic connotations of blood found in the lay population do not influence their transfusion decisions. However, there are other "social" and "cultural' aspects that influence these practices. Also, BT strategies remain diverse across units. Practitioners perceive these situations as resulting from insufficient training and by the existence of an "oral tradition" and a professional culture that are resistant to change. BT practices differ within and across units. Many dimensions intervene in the decision to transfuse, from individual clinical appreciation and local unit "culture" to formal and ad hoc training. Consistent change in BT can only occur with the implementation of norms and guidelines that are endorsed by key influential figures. An extensive multicentered study is necessary to better understand how social and cultural factors affect BT practices. This knowledge will sustain an enlightened clinical practice and lead to the recognition that peer practices are also embedded in professional cultures.

Incidence of hypotension and acute hypotensive transfusion reactions following platelet concentrate transfusions.

Platelet concentrates (PCs) are associated with transfusion reactions involving hypotension, particularly bradykinin-mediated acute hypotensive transfusion reactions. This study aims to determine the incidence of hypotensive events and more specifically acute hypotensive transfusion reaction associated with PC transfusions. We also sought to ascertain whether these reactions are associated with elevated bradykinin levels. This is a prospective descriptive study of PCs administered at Sainte-Justine Hospital over 28 months. All PCs administered during this period were screened for hypotension through review of all transfusion-associated reaction reports (TARRs) sent to the blood bank. All residual PC bags were returned to the blood bank. TARRs associated with hypotension were reviewed by adjudicators that established the imputability of the PC transfusion to the reaction. Bradykinin levels were sampled in the first 168 PC bags returned to the blood bank. Levels were compared between PCs associated with hypotension and control PCs not associated with hypotension. A total of 3672 PC bags were returned to the blood bank; 25 PCs were associated with hypotension. Adjudicators ascertained that five hypotensive events were imputable to PCs of which one was an acute hypotensive transfusion reaction (incidence: 0·03%). Bradykinin level in the latter PC was 10 pg/ml, whereas levels were 226·2 ± 1252 pg/ml in the 143 control PCs. Our results show a low incidence of hypotension after PC transfusion. We identified only one acute hypotensive transfusion reaction. No correlation between bradykinin level and the occurrence of acute hypotensive reactions could be observed given that only one event was identified.

Detection of Epstein-Barr virus in leucoreduced blood products.

This study examined the prevalence of three human herpesviruses (HHV), namely HHV-4 (Epstein-Barr virus/EBV), HHV-6b and HHV-7 in leucoreduced blood products obtained from the Sainte-Justine Hospital blood bank. A total of 100 specimens, including 34 red blood cell concentrates, 33 platelet bags and 33 plasma units, were collected and screened by a sensitive PCR assay using virus-specific primers. Positive units were then retested by quantitative PCR. Of the 100 specimens, one platelet unit tested positive for EBV.

Resective Epilepsy Surgery for Tuberous Sclerosis in Children: Determining Predictors of Seizure Outcomes in a Multicenter Retrospective Cohort Study.

There are no established variables that predict the success of curative resective epilepsy surgery in children with tuberous sclerosis complex (TSC). We performed a multicenter observational study to identify preoperative factors associated with seizure outcome in children with TSC undergoing resective epilepsy surgery. A retrospective chart review was performed in eligible children at New York Medical Center, Miami Children's Hospital, Cleveland Clinic Foundation, BC Children's Hospital, Hospital for Sick Children, and Sainte-Justine Hospital between January 2005 and December 2013. A time-to-event analysis was performed. The "event" was defined as seizures after resective epilepsy surgery. Seventy-four patients (41 male) were included. The median age of the patients at the time of surgery was 120 months (range, 3-216 months). The median time to seizure recurrence was 24.0 ± 12.7 months. Engel Class I outcome was achieved in 48 (65%) and 37 (50%) patients at 1- and 2-year follow-up, respectively. On univariate analyses, younger age at seizure onset (hazard ratio [HR]: 2.03, 95% confidence interval [CI]: 1.03-4.00, P = .04), larger size of predominant tuber (HR: 1.03, 95% CI: 0.99-1.06, P = .12), and resection larger than a tuberectomy (HR: 1.86, 95% CI: 0.92-3.74, P = .084) were associated with a longer duration of seizure freedom. In multivariate analyses, resection larger than a tuberectomy (HR: 2.90, 95% CI: 1.17-7.18, P = .022) was independently associated with a longer duration of seizure freedom. In this large consecutive cohort of children with TSC and medically intractable epilepsy, a greater extent of resection (more than just the tuber) is associated with a greater probability of seizure freedom. This suggests that the epileptogenic zone may include the cortex surrounding the presumed offending tuber.

Psychosurgery for stuttering.

Psychosurgery for stuttering Edgar Durand,1 Alexander G Weil,2 Marc Lévêque1,3 1Espace éthique de l’Assistance publique-Hôpitaux de Paris et Département de recherche en éthique, South Paris University, Paris, France; 2Pediatric Neurosurgical Department, Sainte-Justine Hospital, Montreal, QC, Canada; 3Service de Neurochirurgie, Hôpital de la Pitié-Salpêtrière, Paris, FranceDear editor We read with interest the article entitled “Anterior capsulotomy improves persistent developmental stuttering with a psychiatric disorder: a case report and literature review” published in Neuropsychiatric Disease and Treatment.1 The authors report on a 28-year-old man with persistent developmental stuttering who was treated by bilateral anterior capsulotomy in the People’s Republic of China. To our knowledge, this is a new and previously unreported application of this technique for this indication. Accordingly, as the authors highlight, “the evidence for surgical treatment of persistent developmental stuttering and associated psychiatric disorders is limited”, placing their approach within the clinical research forum. In this experimental context, this case report brings forward several important reflections on patient evaluation, technique utilized, and postoperative follow-up. Read the original article

Sa1133 Prevalence of Methotrexate Intolerance in Pediatric Inflammatory Bowel Disease

• Participation: 64 patients and 44 parents completed the MISS questionnaire. Two patients refused. • MTX intolerance: 32 patients (50%) and 27 (42%) were intolerant to MTX based on the responses of the child and parents, respectively. • Mean (±SD) MISS score: 6.2 ± 5.8 for children and 6.8 ± 6.9 according to parents. • Good correlation between parents’ and children’s answers: r=0.77. • Prevalence and timing of gastrointestinal and behavioral symptoms are indicated in Fig. 1 and 2. The other symptoms noted were headaches (n=4), fatigue (n=2), alopecia (n=1) and negative conditioning to ondansetron (n=1). Division of Gastroenterology, Sainte-Justine Hospital, Montreal, Quebec Sainte-Justine Research Center, Montreal, Quebec.

The psychosocial challenges of epilepsy and the role of the clinical nurse specialist

Epilepsy in children is a complex disease with significant psychosocial consequences for the child and their family. The intervention of a multidisciplinary team enables the care to be adapted and provides support for the families. At Sainte-Justine Hospital in Montreal, Canada, the nurse clinician specialised in epilepsy plays a key role in this team.

ABSTRACT 237

Background and aims: Red blood cell (RBC) transfusion is known to cause various respiratory complications. When respiratory dysfunction (RD) appears after a transfusion is administered, it is considered to be a transfusion-associated respiratory dysfunction (TARD). Aims: 1) to determine the incidence of TARD in a pediatric intensive care unit (PICU), 2) to compare the results obtained with two different definitions (diagnostic criteria) for RD. Methods: This is a prospective cohort study of all consecutive admissions to the PICU of Sainte-Justine Hospital over a one-year period. TARD was diagnosed when RD appeared or significantly worsened after the 1st RBC transfusion administered in PICU. Two independent adjudicators used the diagnostic criteria of Goldstein et al (Pediatr Crit Care Med 2005) and Proulx et al (Chest 1996) to diagnose RD. Agreement was measured using concordance percentage and kappa score. IRB approval was obtained for this study. Results: Among the 842 consecutive admissions to the PICU, 136 children received a transfusion. Among these, 58 (43%) patients presented a TARD according to Goldstein’s criteria, while 53 (39%) TARD were diagnosed according to Proulx’s criteria. Concordance between adjudicators for each item varied from 63% to 100%; kappa scores ranged from 0.63 to 1.00. Conclusions: TARD is frequent in PICU (39% to 43% depending on the definition used). While both definitions by Goldstein et al and Proulx et al gave similar results, the definition by Goldstein et al. was more sensitive.

Congenital esophageal stenosis associated with esophageal atresia

Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES-EA patients from Lille University Hospital, Sainte-Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non-specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1-3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1-7 dilations/patient). Esophageal perforation was encountered in three patients (18%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.