Sainte-Justine Hospital Research Articles

Twenty-five years of selective dorsal rhizotomy based on clinical observations instead of intraoperative electrophysiological monitoring: The Sainte-Justine hospital experience

Twenty-five years of selective dorsal rhizotomy based on clinical observations instead of intraoperative electrophysiological monitoring: The Sainte-Justine hospital experience

2009 H1N1 pandemic

Pediatric Critical Care Unit, Department of Pediatrics, Sainte-Justine Hospital, Université de Montréal, Montréal, Canada *See also p.625. Dr. Jouvet received grant support from Johnson & Johnson and Hamilton Medical. Dr. Flechelles has not disclosed any potential conflicts of interest.

Suivi des concentrations sériques de la vancomycine en pédiatrie

ObjectivesThe authors wanted to determine if the current local practice (initial prescription and monitoring) in pediatrics allowed reaching vancomycin therapeutic concentrations. Patients and methodsThirty patients that had received vancomycin for at least five days with at least one evaluation of serum concentration, at the Sainte-Justine university hospital center, were retrospectively studied. Vancomycin trough and peak levels were analyzed. ResultsThe values of vancomycin serum concentration were within therapeutic ranges (local standards of 5 to 10mg/L for trough level and 20 to 40mg/L for peak level) in 60% and 33% of cases at the fifth day of treatment for trough and peak levels, respectively. ConclusionThe current practice does not allow reaching vancomycin serum concentrations in the target range. Using a wider range for the trough values could be considered.

Risk factors associated with increased length of mechanical ventilation in children

Invasive mechanical ventilation, if prolonged, may lead to high morbidity and mortality. To determine the incidence rate and early risk factors for prolonged acute invasive mechanical ventilation in children. Retrospective longitudinal cohort study over a 1-yr period. All consecutive episodes of invasive mechanical ventilation in the pediatric intensive care units of Sainte-Justine Hospital (Montreal, Canada) were included. Risk factors for long (≥96 hrs) vs. short (<96 hrs) duration of ventilation were determined by logistic regression. None. Among the 360 episodes of invasive ventilation, 36% had a length of ≥96 hrs. Following multivariate analysis, significant risk factors for prolonged acute invasive mechanical ventilation were age of <12 months (odds ratio 3.27, 95% confidence interval 1.90-5.63), Pediatric Risk of Mortality score of ≥15 at admission (odds ratio 3.41, 95% confidence interval 1.31-8.89), mean airway pressure of ≥13 cm H(2)O on day 1 (odds ratio 5.92, 95% confidence interval 3.08-11.36), use of continuous intravenous sedation on day 1 (odds ratio 1.75, 95% confidence interval 1.00-3.05), and use of noninvasive ventilation before intubation (odds ratio 6.56, 95% confidence interval 1.99-21.63). Among the risk factors identified, the use of noninvasive ventilation and continuous intravenous sedation on the first day of ventilation are the only two interventions that were associated with prolonged acute invasive mechanical ventilation. Further research is needed to study the impact of sedation protocols on the duration of mechanical ventilation in children.

Bardet-Biedl Syndrome, Crohn Disease, Primary Sclerosing Cholangitis, and Autoantibody Positive Thyroiditis: A Case Report and A Review of a Cohort of BBS Patients

Bardet-Biedel syndrome (BBS) is a rare autosomal recessive, genetically heterogeneous ciliopathy. Although the disease has been described in a patient with psoriasis, individuals with BBS are not known to be at risk of developing autoimmune disorders. Our objective was to describe a 14-year-old patient with BBS who presented with Crohn disease (CD), primary sclerosing cholangitis (PSC), and thyroiditis in the context of a cohort review at Sainte-Justine Hospital and to alert clinicians to the increased risk of autoimmune disorders in these patients. The cohort contained fifteen patients (9 boys), followed from 1968 to 2009 during a median period of 12 years (range 9 months–26 years). Three of the 15 patients (20%) developed a chronic autoimmune disease: one had juvenile rheumatoid arthritis; a second one had type 1 diabetes mellitus in association with Hashimoto thyroiditis and psoriasis; a third one developed CD, PSC, and Hashimoto thyroiditis. As chronic autoimmune diseases occurred in 20% of our cohort of children with BBS, it is appropriate to keep this association in mind during the followup.

Critical pertussis: An example of pediatric disease that necessitates a large collaborative network*

Division of Pediatric Critical Care, Department of Pediatrics, Sainte-Justine Hospital, Université de Montréal, Montréal, Canada Dr. Jouvet received grant support for research from Hamilton Medical. Dr. Emeriaud has not disclosed any potential conflicts of interest.

Prediction of developmental performance in preterm infants at two years of corrected age: Contribution of the neurological assessment at term age

BackgroundThe population of preterm infants is increasing and resources available for follow-up are limited. Early markers are needed to identify children who will show major as well as more subtle neurodevelopmental impairments. Such a challenge could be achieved with the Amiel-Tison Neurological Assessment at Term (ATNAT). AimsThis study assesses the usefulness of the ATNAT in the prediction of developmental problems at two years of corrected age (CA) in infants born between 29 and 37 weeks of gestation. MethodInclusion criteria were: gestational age between 290/7 and 366/7 weeks inclusively, birth weight below 2500g and minimal 24-hour stay in the Neonatal Intensive Care Unit of Sainte-Justine Hospital. A sample of 147 was prospectively recruited and assessed at two ages: at term with the ATNAT and at 24months CA with Bayley Scales of Infant Development–II. ResultsNo major impairment such as cerebral palsy and no neurosensory impairment were observed. Developmental delay defined by an index<70 on the mental or psychomotor scale was reported respectively in 6.2% and 5.4% of the cohort. Significant differences in mental, psychomotor and behavioral performances were found according to neurological status. Neurological status was the only variable to enter the predictive model for psychomotor and behavioral indexes. Gender and neurological status remained in the predictive model for mental performance. ConclusionThis study supports the inclusion of the ATNAT among the eligibility criteria for systematic neurodevelopmental surveillance as it allows early identification of infants at higher risk of low developmental performances at 24months CA.

Tachycardia in a Patient with Hypertrophic Cardiomyopathy and Suspected Ventricular Preexcitation

Pacing and Clinical ElectrophysiologyVolume 34, Issue 1 p. 104-106 EP ROUNDS Tachycardia in a Patient with Hypertrophic Cardiomyopathy and Suspected Ventricular Preexcitation KATIA DYRDA M.Sc., M.D., KATIA DYRDA M.Sc., M.D. Montreal Heart InstituteSearch for more papers by this authorANNE FOURNIER M.D., ANNE FOURNIER M.D. Sainte-Justine Hospital, University of Montreal, Montreal, Quebec, CanadaSearch for more papers by this authorFRANÇOIS MARCOTTE M.D., FRANÇOIS MARCOTTE M.D. Montreal Heart InstituteSearch for more papers by this authorPAUL KHAIRY M.D., Ph.D., PAUL KHAIRY M.D., Ph.D. Montreal Heart Institute Sainte-Justine Hospital, University of Montreal, Montreal, Quebec, CanadaSearch for more papers by this author KATIA DYRDA M.Sc., M.D., KATIA DYRDA M.Sc., M.D. Montreal Heart InstituteSearch for more papers by this authorANNE FOURNIER M.D., ANNE FOURNIER M.D. Sainte-Justine Hospital, University of Montreal, Montreal, Quebec, CanadaSearch for more papers by this authorFRANÇOIS MARCOTTE M.D., FRANÇOIS MARCOTTE M.D. Montreal Heart InstituteSearch for more papers by this authorPAUL KHAIRY M.D., Ph.D., PAUL KHAIRY M.D., Ph.D. Montreal Heart Institute Sainte-Justine Hospital, University of Montreal, Montreal, Quebec, CanadaSearch for more papers by this author First published: 07 January 2011 https://doi.org/10.1111/j.1540-8159.2010.02831.x Address for reprints: Paul Khairy, M.D., Ph.D., Montreal Heart Institute, 5000 Belanger St. E., Montreal, Quebec, Canada, H1T 1C8. Fax: 514-593-2581; e-mail: paul.khairy@umontreal.ca Potential conflicts of interest: The authors have no potential conflicts of interest to disclose. This work was supported in part by a Canada research chair in electrophysiology and adult congenital heart disease (PK). Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Volume34, Issue1January 2011Pages 104-106 RelatedInformation

Large congenital melanocytic nevi and neurocutaneous melanocytosis: One pediatric center's experience

Large congenital melanocytic nevi (LCMN) predispose to neurocutaneous melanocytosis (NCM), which is associated with significant morbidity and mortality. To identify risk factors for NCM in patients with LCMN and suggest guidelines for their management. Medical records of patients with LCMN were reviewed at Sainte-Justine Hospital between 1980 and 2006. Presence of multiple satellite nevi and posterior midline location were evaluated as risk factors for NCM using chi-square test. Magnetic resonance imaging scans were reviewed by a neuroradiologist. Twenty-six of 52 patients underwent radiologic investigation. Six of 26 (23%) had NCM. Patients with this condition are more likely to have multiple satellite nevi (100% vs 50%, P = .03) and have a trend to posterior midline location of their LCMN (100% vs 60%, P = .08). Patients with NCM are more likely to have both multiple satellite nevi and posterior midline location (100% vs 25%, P = .002). Radiologic findings are also presented. This was a retrospective case series with imprecise chart data in 38% of cases. The presence of multiple satellite nevi alone or with associated posterior midline location of LCMN is associated with a higher risk of NCM. We recommend magnetic resonance imaging testing before 4 months of age in patients with these features.

Organisation des soins spécialisés et intégrés en ambulatoire pour la prise en charge de la maladie rénale chronique en pédiatrie : expérience du CHU Sainte-Justine

The management and optimal care for the pediatric patient with chronic kidney disease requires attention not only to medical management, but also special focus on the psychosocial and developmental factors of children which is complicated by the presence of other disease-related complications. In recent years, specialized chronic kidney disease and predialysis clinics have been set up to facilitate and improve the quality of care of these patients with a multidisciplinary organisation and coordinated management approaches of a renal team. We present our experience in establishing such a renal management clinic named "Prévoir" for children with chronic kidney disease at Sainte-Justine Hospital.

Should we avoid transfusion in the pediatric intensive care unit?*

Division of Pediatric Critical Care Medicine; Department of Pediatrics; Sainte-Justine Hospital; Université de Montréal; Montreal, Quebec, Canada (Gauvin, Lacroix) The authors have not disclosed any potential conflicts of interest.

Évaluation des besoins pharmacothérapeutiques d’un service de pédopsychiatrie

Objective To develop a structured and reproducible approach to evaluate pharmaceutherapeutic needs. The approach should allow a pharmacist task description on the unit in order to optimize pharmaceutical care. Patients and methods Descriptive study of pharmaceutical care model. The selected unit is a pediatric psychiatry unit in Sainte-Justine hospital, a teaching hospital of 500 beds. The methodology includes a literature review conducted to describe the impact of clinical pharmacist on selected health indicators; a profile of patients and clinical activities; a description of pharmacotherapy needs and an update of task description. Results Studies demonstrate that pharmacists have an impact on selected clinical outcomes following clinical pharmacy activities: drug therapy monitoring, intervention to improve compliance, and education. Health indicators revealed that patients admitted in the pediatric psychiatry unit have a better prognosis than average patients in the hospital, but a longer length of stay (23.4 days versus 5.8 days). For pharmacy services, their drug cost per admission is 50 $ per admission (children) and 183 $ per admission (adolescents) versus 380 $ for all the patients treated at CHU Sainte-Justine. The approach helped us to identify interventions to manage outpatients, to provide medication history and clinical tools to reduce prescription error. Conclusion This is the first study to illustrate an approach for the evaluation of a pharmacotherapeutic needs in a pediatric psychiatry unit.

Dermatofibrosarcoma Protuberans in Childhood and Adolescence: Report of Eight Patients

We reviewed all occurrences of dermatofibrosarcoma protuberans in children under 17 years of age who were evaluated at Sainte-Justine Hospital, a tertiary-care pediatric center, between 1980 and 2002. The medical records and histologic features of all were reviewed. Eight patients were identified, three boys and five girls. The interval between apparent onset and diagnosis ranged from 3 months to 14 years. All lesions except one were removed by surgical excision with a margin of 1.5 to 3 cm including underlying fascia or by Mohs technique. Follow-up ranged from 2 to 15 years with median of 5 years. To date all the patients are alive and none has had a recurrence.

Langerhans’ cell histiocytosis of the temporal bone in children

Langerhans' cell histiocytosis (LCH) is a rare pathology that implies an abnormal proliferation of these kinds of cells associated with a granular infiltration that affects different structures of the human body, including the temporal bone. The authors present their series of LCH of the temporal bone in children at the Sainte-Justine university hospital. The twofold objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare these results with previously reported series. A retrospective study was conducted between 1984 and 2007 with patients diagnosed and treated for a LCH of the temporal bone at the Sainte-Justine university hospital, a paediatric tertiary care center. A chart review was performed to obtain demographic, clinical, paraclinical, and therapeutic data. They were analysed and compared to other published series. Through a MEDLINE query, we found that since 1966, 50 articles dealing with a LCH of the temporal bone have been published. Fifty-nine cases of LCH were diagnosed and among them, 10 children had temporal bone involvement. They were four females and six males with a mean age of 3.28 years. The two most frequent clinical manifestations were the presence of a mass in the temporal region (70%) and otitis (60%). Two of our patients had a unifocal lesion of the temporal bone implicated. Eight patients had a multisystem involvement among which, two showed evidence of organ dysfunction. In 80% of cases, the diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The common radiological finding on a skull CT scan is a lytic lesion in the temporal bone. Seven patients were treated by chemotherapy, two were treated by radiotherapy as a primary treatment, and one received radiotherapy for a recurrence on the pituitary gland. Finally, one patient was treated with local steroid injections. Two patients had a recurrence. All our patients were in total remission on a mean average of 1.6 years after the diagnosis. Our results concord with other studies in which the prognosis of unifocal bone disease is excellent and children with a multifocal disease have a survival rate of 65-100%. LCH is a rare disease. A high-index suspicion should be raised in the context of a temporal mass, chronic otitis, and otorrhea. A biopsy is recommended in the presence of a temporal bone lytic lesion. Chemotherapy is the preferred therapeutic modality.

Long‐term survival rate of pediatric patients after blood transfusion

Studies in adults report posttransfusion survival rate (PTSR) at 1 to 10 years of 41 to 67 percent. There are no published studies specifically addressing PTSR in pediatric patients. The objectives of this study were to evaluate PTSR and risk factors associated with death in children receiving transfusions. A database of all patients receiving their first transfusion between 1990 and 1992 at Sainte-Justine Hospital (SJH) was used. Patients' demographic data, primary diagnosis, surgical procedures, and information on all labile components transfused were collected. Death was confirmed by the SJH database, RAMQ (universal health care provider for Québec residents), and the Quebec Commission for Access to Information (death certificate). The study population consisted of 1100 children. Median age was 16 months (range, 0-204 months). The most frequent primary diagnoses were cardiac disease (22%), prematurity (21.5%), malignant disease (11%), and hematologic disease (9.5%). Patients received a median of three transfusions (range, 1-126). PTSR was 86.9 percent at 1 year and 82.3 percent at 10 years. For nonsurvivors, median survival time was 22 days. Multivariate analysis showed that significant risk factors for death are age of less than 1 month, a diagnosis of malignant disease, more than 20 transfusions, and administration of more than one type of blood component. PTSR in children receiving their first transfusion between 1990 and 1992 was considerably higher than the PTSR reported in adults. Death occurred early after the first transfusion; survival rate remained relatively stable thereafter.

Neuropsychologie, plasticité et épilepsie infantile

Epilepsy is one of the most frequent childhood disorders. While most cases are well controlled, approximately 30-50% are resistant to medical treatment. In these cases, neurosurgery may be an option. Since 1979, our team at the Sainte-Justine Hospital in Montreal has studied the impact of epilepsy on the psycho-motor and cognitive development of the affected children. The aim of a first series of studies was to explore the extent and limits of cerebral plasticity by investigating the neuropsychological sequelae of early versus late callosotomy and hemispherectomy. In keeping with the plasticity hypothesis, the results revealed that the children who were operated before puberty showed fewer deficits than those operated during adolescence or adulthood. However, the compensatory mechanisms available to them appeared to be limited with respect to the nature and complexity of the information they can process. For instance, young children having undergone section of the corpus callosum resembled individuals born without a corpus callosum (callosal agenesis) in that there did not show the typical disconnection deficits seen in adult ''split-brain'' patients. However, they exhibited deficits on tasks requiring interhemispheric integration of motor and visuo-motor information. By the same token, hemispherectomy patients were still able to make visual judgements in their ''blind'' visual field but they were found to be impaired on a variety of visual and auditory tasks (localization of a sound sources in space) requiring the participation of both hemispheres. In a second series of studies, carried out in collaboration with a Parisian team, we intended to describe the neuropsychological profile of focal epilepsies, specifically frontal and temporal epilepsy. Contrary to the common belief that childhood epilepsy would result in diffuse impairments, we were able to demonstrate that children manifest the same localized deficits as adult patients. In studies presently underway in our laboratories, we make use of functional neuroimaging techniques such as high density electrophysiology, optical imaging and magnetoencephalography to investigate pre-and postoperative language and memory functions and to study the impact of epilepsy on brain maturation.

Comparison of pain management in paediatric surgical patients in two hospitals in France and Canada

Pain management in children has improved substantially over the last few years but continues to vary widely across institutions. Our objective was to describe the evaluation and treatment of pain in paediatric patients in two hospitals (Robert Debré in Paris, France, and Sainte-Justine, Montréal, Canada) and to compare conformity with quality criteria. Retrospective medical chart of a total of 200 patients with uncomplicated appendectomy, spinal fusion, ureteroneocystostomy, or laparoscopic cholecystectomy were analysed, with special attention to nurses' entries, prescriptions, and medication administration sheets. Patient characteristics and variables pertaining to pain evaluation (tool and result) and treatment (date, prescription and administration details) were collected. Quality criteria for evaluating conformity with guidelines were taken from the literature. Any change in medication, dosage, or dosing interval was taken as a new prescription. About 929 prescriptions and 2,388 numerical pain scores were recorded for 200 patients. Pain was recorded at 70.8% of vital-sign evaluations at the Robert-Debré Hospital compared to 30.9% at the Sainte-Justine hospital (P < 0.0001). A validated age-appropriate pain evaluation tool was used in 97.4 and 94.1% of evaluations in these two hospitals, respectively (P < 0.0001). Analgesic dosage was appropriate in 92.5% of prescriptions at Robert-Debré and 86.0% of those at Sainte-Justine (P = 0.002). This study documents differences in post-operative pain management between two paediatric hospitals. We found differences between the two hospitals regarding the frequency of pain evaluation, the use of validated tools for evaluating pain, and analgesic dosages. Continued efforts to educate prescribing physicians and other healthcare providers are needed to improve pain management in children.

A Novel System for the 3-D Reconstruction of the Human Spine and Rib Cage From Biplanar X-Ray Images

The main objective of this study was to develop a 3-D X-ray reconstruction system of the spine and rib cage for an accurate 3-D clinical assessment of spinal deformities. The system currently used at Sainte-Justine Hospital in Montreal is based on an implicit calibration technique based on a direct linear transform (DLT), using a sufficiently large rigid object incorporated in the positioning apparatus to locate any anatomical structure to be reconstructed within its bounds. During the time lapse between the two successive X-ray acquisitions required for the 3-D reconstruction, involuntary patient motion introduce errors due to the incorrect epipolar geometry inferred from the stationary object. An approach using a new calibration jacket and explicit calibration algorithm is proposed in this paper. This approach yields accurate results and compensates for involuntary motion occurring between X-ray exposures.

Acute lung injury: Is volume the question?*

Division of Pediatric Critical Care, Department of Pediatrics, Sainte-Justine Hospital, Université de Montréal, Montréal, Quebec, Canada *See also pp 317 and 324.

Wait Times for Paediatric Rehabilitation

Early therapy intervention for children with disabilities may improve functional outcomes. Access to paediatric rehabilitation services can sometimes be difficult. To describe waiting time to receive physical therapy (PT) and occupational therapy (OT) services at rehabilitation centres for young children with physical disabilities; to examine factors associated with these waiting times. Prospective cohort. Two hundred and six children with physical disabilities, aged 6 to 72 months, referred in 2002-2004 from the Montreal Children's Hospital and Sainte-Justine Hospital to paediatric rehabilitation centres. Data on date of referral, age, gender and diagnosis were obtained from the hospital databases. Data on date of first PT or OT appointments at the rehabilitation centre, family socio-demographics and disability severity (WeeFIM) were obtained during parental interviews. Half of the sample waited more than 7 and 11 months for PT and OT services, respectively. Shorter waiting time was significantly associated with younger child's age and referral to one particular rehabilitation centre. Children with physical disabilities experience long waiting times for PT and OT rehabilitation services. Strategies to improve timely service delivery are needed.