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Showing papers by "Montreal Children's Hospital published in 2004"


Journal ArticleDOI
TL;DR: The data indicate that FGF-23 induces phenotypic changes in mice resembling those of patients with ADHR, OOM, and XLH and that F GF-23 is an important determinant of Pi homeostasis and bone mineralization.
Abstract: Mutations in the fibroblast growth factor 23 gene, FGF23, cause autosomal dominant hypophosphatemic rickets (ADHR). The gene product, FGF-23, is produced by tumors from patients with oncogenic osteomalacia (OOM), circulates at increased levels in most patients with X-linked hypophosphatemia (XLH) and is phosphaturic when injected into rats or mice, suggesting involvement in the regulation of phosphate (Pi) homeostasis. To better define the precise role of FGF-23 in maintaining Pi balance and bone mineralization, we generated transgenic mice that express wild-type human FGF-23, under the control of the alpha1(I) collagen promoter, in cells of the osteoblastic lineage. At 8 wk of age, transgenic mice were smaller (body weight = 17.5 +/- 0.57 vs. 24.3 +/- 0.37 g), exhibited decreased serum Pi concentrations (1.91 +/- 0.27 vs. 2.75 +/- 0.22 mmol/liter) and increased urinary Pi excretion when compared with wild-type littermates. The serum concentrations of human FGF-23 (undetectable in wild-type mice) was markedly elevated in transgenic mice (>7800 reference units/ml). Serum PTH levels were increased in transgenic mice (231 +/- 62 vs. 139 +/- 44 pg/ml), whereas differences in calcium and 1,25-dihydroxyvitamin D were not apparent. Expression of Npt2a, the major renal Na(+)/Pi cotransporter, as well as Npt1 and Npt2c mRNAs, was significantly decreased in the kidneys of transgenic mice. Histology of tibiae displayed a disorganized and widened growth plate and peripheral quantitative computerized tomography analysis revealed reduced bone mineral density in transgenic mice. The data indicate that FGF-23 induces phenotypic changes in mice resembling those of patients with ADHR, OOM, and XLH and that FGF-23 is an important determinant of Pi homeostasis and bone mineralization.

481 citations


Journal ArticleDOI
TL;DR: It is suspected that high levels of prenatal stress exposure, particularly early in the pregnancy, may negatively affect the brain development of the fetus, reflected in the lower general intellectual and language abilities in the toddlers.
Abstract: Prenatal maternal stress has been shown to impair functioning in nonhuman primate offspring Little is known about the effects of prenatal stress on intellectual and language development in humans because it is difficult to identify sufficiently large samples of pregnant women who have been exposed to an independent stressor We took advantage of a natural disaster (January 1998 ice storm in Quebec, Canada) to determine the effect of the objective severity of pregnant women's stress exposure on general intellectual and language development of their children Bayley Mental Development Index (MDI) scores and parent-reported language abilities of 58 toddlers of mothers who were exposed to varying levels of prenatal stress were obtained at 2 y of age The hierarchical multiple regression analyses indicated that the toddlers' birth weight and age at testing accounted for 120% and 148% of the variance in the Bayley MDI scores and in productive language abilities, respectively More importantly, the level of prenatal stress exposure accounted for an additional 114% and 121% of the variance in the toddlers' Bayley MDI and productive language abilities and uniquely accounted for 173% of the variance of their receptive language abilities The more severe the level of prenatal stress exposure, the poorer the toddlers' abilities The level of prenatal stress exposure accounted for a significant proportion of the variance in the three dependent variables above and beyond that already accounted for by non-ice storm-related factors We suspect that high levels of prenatal stress exposure, particularly early in the pregnancy, may negatively affect the brain development of the fetus, reflected in the lower general intellectual and language abilities in the toddlers

446 citations


Journal ArticleDOI
TL;DR: The methylation of the Snrpn, Igf2r, Peg1 and Peg3 differentially methylated regions in postnatal growing mouse oocytes indicated that methylation was acquired asynchronously at these different genes, contributing to the understanding of the epigenetic mechanisms underlying imprint acquisition during female germ cell development and for the practice of assisted reproductive technologies.
Abstract: Imprinted genes are differentially marked during germ cell development to allow for their eventual parent-of-origin specific expression. A subset of imprinted genes becomes methylated during oocyte growth in both mouse and human. However the timing and mechanisms of methylation acquisition are unknown. Here, we examined the methylation of the Snrpn, Igf2r, Peg1 and Peg3 differentially methylated regions in postnatal growing mouse oocytes. Our findings indicate that methylation was acquired asynchronously at these different genes. Further analysis of Snrpn DMR1 revealed that parental alleles retain an epigenetic memory of their origin as the two alleles were recognized in a parental-specific manner in the absence of DNA methylation. In addition, we show that methylation acquisition was probably related to oocyte diameter and coincided with the accumulation of Dnmt3a, Dnmt3b and Dnmt3L transcripts. Methylation of the repetitive retroviral-like intracisternal A particle also occurred during this same window of oocyte growth. These findings contribute to our understanding of the epigenetic mechanisms underlying imprint acquisition during female germ cell development and have implications for the practice of assisted reproductive technologies.

426 citations


Journal ArticleDOI
TL;DR: Overnight pulse oximetry can be used to estimate the severity of OSA, to shorten the diagnostic and treatment process for those with more severe disease, and to aid clinicians in prioritization of T&A and planning perioperative care.
Abstract: Objective. Obstructive sleep apnea (OSA) in children is usually effectively treated by adenotonsillectomy (TA 22%) had no additional sleep studies before T&A. Timing of T&A was based on oximetry score, leading to a significant reduction in waiting time for surgery for those with higher oximetry scores. Postoperative respiratory complications were more common with increasing oximetry score. Conclusions. Overnight pulse oximetry can be used to estimate the severity of OSA, to shorten the diagnostic and treatment process for those with more severe disease, and to aid clinicians in prioritization of T&A and planning perioperative care.

323 citations


Journal ArticleDOI
TL;DR: Gene therapy for RPE65 deficient dogs partially restored sight, and provides the first real hope of treatment for this devastating blinding condition.

254 citations


Journal ArticleDOI
TL;DR: In this paper, the authors investigated whether MMR vaccination is associated with an increased risk of autism or other pervasive developmental disorders, and they found that MMR vaccination was not associated with a higher risk of pervasive developmental disorder.

241 citations


Journal ArticleDOI
TL;DR: The results suggest that the mechanism of meiotic down-regulation differs between the sexes and provide clues to specific roles for the different DNMT family members in de novo and maintenance methylation in the developing testis and ovary.

204 citations


Journal ArticleDOI
TL;DR: New developments in the understanding of genomic imprinting including the mechanisms and timing of imprint erasure, acquisition and maintenance during germ cell development and early embryogenesis are discussed as well as the implications of this research for future epigenetic studies in reproduction and assisted reproductive technology.
Abstract: Recent studies suggest a possible link between human assisted reproductive technology and genomic imprinting disorders. Assisted reproductive technology includes the isolation, handling and culture of gametes and early embryos at times when imprinted genes are likely to be particularly vulnerable to external influences. Evidence of sex-specific differences in imprint acquisition suggests that male and female germ cells may be susceptible to perturbations in imprinted genes at specific prenatal and postnatal stages. Imprints acquired first during gametogenesis must be maintained during preimplantation development when reprogramming of the overall genome occurs. In this review, we will discuss both new developments in our understanding of genomic imprinting including the mechanisms and timing of imprint erasure, acquisition and maintenance during germ cell development and early embryogenesis as well as the implications of this research for future epigenetic studies in reproduction and assisted reproductive technology.

202 citations


Journal ArticleDOI
TL;DR: In stimulant-responsive young children with ADHD without learning and conduct disorders, there is no support for academic assistance and psychotherapy to enhance academic achievement or emotional adjustment.
Abstract: Objective To test the hypothesis that intensive multimodal psychosocial intervention (that includes academic assistance and psychotherapy) combined with methylphenidate significantly enhances the academic performance and emotional status of children with attention-deficit/hyperactivity disorder (ADHD) compared with methylphenidate alone and with methylphenidate combined with nonspecific psychosocial treatment (attention control). Method One hundred three children with ADHD (ages 7–9), free of conduct and learning disorders, who responded to short-term methylphenidate were randomized for 2 years to receive one of three treatments: (1) methylphenidate alone, (2) methylphenidate plus psychosocial treatment that included academic remediation, organizational skills training, and psychotherapy as well as parent training and counseling and social skills training, or (3) methylphenidate plus attention control treatment. Children's function was assessed through academic testing, parent ratings of homework problems, and self-ratings of depression and self-esteem. Results No advantage was found on any measure of academic performance or emotional status for the combination treatment over methylphenidate alone and over methylphenidate plus attention control. Significant improvement occurred across all treatments and was maintained over 2 years. Conclusions In stimulant-responsive young children with ADHD without learning and conduct disorders, there is no support for academic assistance and psychotherapy to enhance academic achievement or emotional adjustment. Significant short-term improvements were maintained over 2 years.

193 citations


Journal ArticleDOI
01 Mar 2004-Autism
TL;DR: This study aimed to determine if relatives of children with autism and less severe pervasive developmental disorders (PDDs) have higher rates of various components of the broad autistic phenotype.
Abstract: This study aimed to determine if relatives of children with autism and less severe pervasive developmental disorders (PDDs) have higher rates of various components of the broad autistic phenotype. Psychiatric and medical disorders were investigated. Parents of children with PDDs were selected from an epidemiological survey and compared with parents of control children with non-autistic developmental problems. Rates of abnormalities and disorders were compared in relatives of 79 cases and 61 controls. Medical and autoimmune disorders in both groups were endorsed by few relatives. Specific developmental disorders were commoner in parents of controls. Depression and anxiety were significantly more prevalent in mothers of children with PDDs. Significantly more PDD children had at least one first-degree relative with anxiety and one second-degree relative with OCD. PDDs were commoner in first-degree relatives. The implications of the findings for the definition of the broad phenotype of autism are discussed.

182 citations


Journal ArticleDOI
TL;DR: This study provides evidence that the positive predictive value of a diagnosis of autism recorded in the GPRD is high.
Abstract: We report on the validity of the computerized diagnoses of autism in a large case-control study investigating the possible association between autism and the measles, mumps and rubella vaccine in the UK using the General Practitioner Research Database (GPRD). We examined anonymized copies of all relevant available clinical reports, including general practitioners' (GP) notes, consultant, speech therapy and educational psychologists reports, on 318 subjects born between 1973 and 1997 with a diagnosis of autism or a related disorder recorded in their electronic general practice record. Data were abstracted to a case validation form allowing for the identification of developmental symptoms relevant to the diagnosis of pervasive developmental disorders (PDDs). Information on other background clinical and familial features was also abstracted. A subset of 50 notes was coded independently by 2 raters to derive reliability estimates for key clinical characteristics. For 294 subjects (92.5%) the diagnosis of PDD was confirmed after review of the records. Of these, 180 subjects (61.2%) fulfilled criteria for autistic disorder. The mean age at first recording of a PDD diagnosis in the GPRD database was 6.3 years (SD = 4.6). Consistent with previous estimates, the proportion of subjects experiencing regression in the course of their development was 19%. Inter-rater reliability for the presence of a PDD diagnosis was good (kappa = .73), and agreement on clinical features such as regression, age of parental recognition of first symptoms, language delay and presence of epilepsy was also good (kappas ranging from .56 to 1.0). This study provides evidence that the positive predictive value of a diagnosis of autism recorded in the GPRD is high.

Journal ArticleDOI
TL;DR: The results suggest that family separation can be understood as an ambiguous loss, in that the temporary absence of other family members cannot be fully acknowledged because of the perpetual uncertainty and permanent risk to them.

Journal ArticleDOI
TL;DR: A multivariate prediction rule shows promise for the accurate prediction of pneumonia in children, and patients with focal infiltrates were more likely in this study to have a history of fever, tachypnea, increased heart rate, retractions, grunting, crackles, or decreased breath sounds.
Abstract: Objective. To determine predictive factors for the presence of focal infiltrates in children with clinically suspected pneumonia in a pediatric emergency department. Methods. Children (1–16 years) with clinically suspected pneumonia were studied prospectively. The presenting features were compared between the children with and without focal infiltrates using χ 2 analysis, t test, and odds ratio with 95% confidence intervals. A multivariate prediction rule was developed using logistic regression. Results. A total of 570 were studied. Risk factors (odds ratio; 95% confidence interval) for the presence of focal infiltrates included history of fever (3.1; 1.7–5.3), decreased breath sounds (1.4; 1.0–2.0), crackles (2.0; 1.4–2.9), retractions (2.8; 1.0–7.6), grunting (7.3; 1.1–48.1), fever (1.5; 1.2–1.9), tachypnea (1.8; 1.3–2.5), and tachycardia (1.3; 1.0–1.6). We then used logistic regression to develop a candidate prediction rule for the variables of fever, decreased breath sounds, crackles, and tachypnea, which had an area under the receiver operating curve of 0.668. This rule had excellent sensitivity (93.1%–98%) yet poor specificity (5.7%–19.4%). Conclusions. Multiple predictive factors for children with suspected pneumonia have been identified. Patients with focal infiltrates were more likely in our study to have a history of fever, tachypnea, increased heart rate, retractions, grunting, crackles, or decreased breath sounds. A multivariate prediction rule shows promise for the accurate prediction of pneumonia in children. However, the prospective evaluation of this multivariate prediction rule in a clinical setting is still required.

Journal ArticleDOI
TL;DR: Lipopolysaccharide inhibits allergic inflammation in nasal mucosa of atopic children by skewing local immune responses from Th2 to Th1 and upregulating production of interleukin 10.

Journal ArticleDOI
TL;DR: Gagnon et al. as discussed by the authors compared the balance skills of children after mild traumatic brain injury (TBI) with that of noninjured children matched for age, sex, and premorbid level of physical activity.

Journal ArticleDOI
TL;DR: This combination of multiple congenital abnormalities has not been previously described and probably represents a new autosomal recessive syndrome involving a genetic abnormality that interferes with normal islet development and whose aetiology is as yet unknown.
Abstract: Aims/hypothesis Neonatal diabetes is a rare disease with several identified molecular aetiologies. Despite associations with other malformations, neonatal diabetes with intestinal and biliary anomalies has not been described. The current study aims to describe a new syndrome, and to examine a possible link with one of three genes known to cause neonatal diabetes.

Journal ArticleDOI
TL;DR: IL-13 might be an important autocrine growth factor for IgE-producing B lymphocytes and neutralization of IL-13 impairs IgE synthesis, and real-time PCR demonstrated a 10- to 15-fold increase in IL- 13 mRNA.
Abstract: Background Our laboratory has demonstrated previously that human tonsillar B lymphocytes express IL-13 mRNA Objective We sought to investigate IL-13 production by human B cells and the association between B cell–derived IL-13 and IgE secretion. Methods Human B lymphocytes were isolated from tonsils and purified by means of rosetting with sheep RBCs or positive or negative selection with magnetic beads. They were stimulated with anti-CD40 antibodies with or without recombinant IL-4. Total mRNA was extracted, and IL-13 mRNA was measured by means of standard RT-PCR or by means of real-time PCR with commercially available primers. B cells were cultured with or without IL-13 neutralizing antibodies, and Ce transcripts and supernatant IgE levels were measured. Results IL-13 mRNA was detected in human B lymphocytes stimulated with anti-CD40 antibodies and IL-4 or IL-2 but not in unstimulated B cells. Real-time PCR demonstrated a 10- to 15-fold increase in IL-13 mRNA, maximizing at 36 hours. IL-13 protein was detected from B lymphocytes on day 3 and accumulated through day 7. The synthesis of IL-13 required both CD40 and IL-4 stimulation. The presence of IL-13 was confirmed by means of intracellular staining of cultured B lymphocytes and antigen-stimulated nasal biopsy specimens from atopic individuals. Addition of IL-13 neutralizing antibodies to purified B-cell cultures inhibited IgE production by up to 80% and diminished IgE (Ce) transcripts by 50%. Conclusion Human B lymphocytes express IL-13 mRNA after ligation of CD40 and the addition of cytokines. Human B lymphocytes produce significant IL-13, and neutralization of IL-13 impairs IgE synthesis. IL-13 might be an important autocrine growth factor for IgE-producing B lymphocytes.

Journal ArticleDOI
TL;DR: This review summarizes what is known about the neurobiology of specific language impairment and suggests that atypical patterns of asymmetry of language cortex, white-matter abnormalities, and cortical dysplasia may be associated with Specific language impairment.
Abstract: This review summarizes what is known about the neurobiology of specific language impairment. Despite its name, specific language impairment is frequently not specific. It is common to find associated impairments in motor skills, cognitive function, attention, and reading in children who meet criteria for specific language impairment. There is evidence that limitation in phonologic working memory may be a core deficit in specific language impairment. Both genetic and environmental factors have been shown to be important etiologic factors in specific language impairment. Structural neuroimaging studies suggest that atypical patterns of asymmetry of language cortex, white-matter abnormalities, and cortical dysplasia may be associated with specific language impairment. Abnormalities in the later stages of auditory processing have been demonstrated using auditory event-related potentials. Functional neuroimaging may cast further light on the neurobiology of specific language impairment and serve as a means of developing and evaluating therapy. A better understanding of the neurobiology of specific language impairment is critical for the rational development of therapeutic strategies to treat this common disorder.

Journal ArticleDOI
TL;DR: Univariate analyses provide evidence of an association between elevated UHg level and young age, and a multiple logistic regression model shows that the presence of amalgam fillings leads to increased odds of high UHG in children.

Journal ArticleDOI
01 Mar 2004-Chest
TL;DR: The single-breath DLNO/DLCO ratios are independent of exercise intensity, suggesting that using either nitric oxide or carbon monoxide as transfer gases are valid in the study of lung diffusion during any level of exercise.

Reference EntryDOI
TL;DR: In steroid-naive patients with mild to moderate airway obstruction, the initiation of inhaled corticosteroids in combination with long-acting beta2-agonists does not significantly reduce the rate of exacerbations over that achieved with inhaled Corticosteroid alone; it does improve lung function and symptom-free days but does not reduce rescuebeta2-agonist use as compared to inhaled steroids alone.
Abstract: Background Consensus statements recommend the addition of long-acting inhaled beta2-agonists only in asthmatic patients who are inadequately controlled on inhaled corticosteroids. Objectives To compare the efficacy of initiating anti-inflammatory therapy using the combination of inhaled corticosteroids and long-acting beta2-agonists (ICS+LABA) as compared to inhaled corticosteroids alone (ICS alone) in steroid-naive children and adults with persistent asthma. Search strategy We identified randomised controlled trials (RCTs) through electronic database searches (Cochrane Airways Group Specialised Register, Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE and CINAHL) until April 2004, bibliographies of identified RCTs and correspondence with manufacturers. Selection criteria RCTs comparing the combination of inhaled corticosteroids and long-acting beta2-agonists (ICS + LABA) to inhaled corticosteroids (ICS) alone in steroid-naive children and adults with asthma. Data collection and analysis Studies were assessed independently by each reviewer for methodological quality and data extraction. Confirmation was obtained from the trialists when possible. The primary endpoint was rate of asthma exacerbations requiring systemic corticosteroids. Secondary endpoints included pulmonary function tests (PFTs), symptoms, use of other measures of asthma control, adverse events, and withdrawal rates. Main results Eighteen trials met the inclusion criteria; nine (totaling 1061 adults) contributed sufficient data to be analysed. Baseline forced expiratory volume in one minute (FEV1) was less than 80% predicted value in four trials and equal to or greater than 80% in five trials. The long-acting beta2-agonists (LABA) formoterol (N=2) or salmeterol (N=7) were added to a dose of at least 800 microg/day of beclomethasone dipropionate (BDP) equivalent of inhaled corticosteroids (ICS) in three trials and to at least 400 microg/day in the six remaining trials. Treatment with ICS plus LABA was not associated with a lower risk of exacerbations requiring oral corticosteroids than ICS alone (relative risk (RR) 1.2; 95% confidence interval (CI) 0.8 to 1.9). FEV1 improved significantly with LABA (weighted mean difference (WMD) 210 ml; 95% CI 120 to 300), as did symptom-free days (WMD 10.74%; 95% CI 1.86 to 19.62), but the change in use of rescue fast-acting beta2-agonists was not significantly different between the groups (WMD -0.4 puff/day, 95% CI -0.9 to 0.1). There was no significant group difference in adverse events (RR 1.1; 95% CI 0.8 to 1.5), withdrawals (RR 0.9; 95% CI 0.6 to 1.2), or withdrawals due to poor asthma control (RR 1.3; 95% CI 0.5 to 3.4). Authors' conclusions In steroid-naive patients with mild to moderate airway obstruction, the initiation of inhaled corticosteroids in combination with long-acting beta2-agonists does not significantly reduce the rate of exacerbations over that achieved with inhaled corticosteroids alone; it does improve lung function and symptom-free days but does not reduce rescue beta2-agonist use as compared to inhaled steroids alone. Both options appear safe. There is insufficient evidence at present to recommend use of combination therapy rather than ICS alone as a first-line treatment.

Journal ArticleDOI
TL;DR: An important role for choline and betaine depletion in the pathogenesis of homocystinuria due to MTHFR deficiency is emphasized, and the dramatic effects of betaine on survival and growth are emphasized.
Abstract: MTHFR (methylenetetrahydrofolate reductase) catalyses the synthesis of 5-methyltetrahydrofolate, the folate derivative utilized in homocysteine remethylation to methionine. A severe deficiency of MTHFR results in hyperhomocysteinaemia and homocystinuria. Betaine supplementation has proven effective in ameliorating the biochemical abnormalities and the clinical course in patients with this deficiency. Mice with a complete knockout of MTHFR serve as a good animal model for homocystinuria; early postnatal death of these mice is common, as with some neonates with low residual MTHFR activity. We attempted to rescue Mthfr−/− mice from postnatal death by betaine supplementation to their mothers throughout pregnancy and lactation. Betaine decreased the mortality of Mthfr−/− mice from 83% to 26% and significantly improved somatic development from postnatal day 1, compared with Mthfr−/− mice from unsupplemented dams. Biochemical evaluations demonstrated higher availability of betaine in suckling pups, decreased accumulation of homocysteine, and decreased flux through the trans-sulphuration pathway in liver and brain of Mthfr−/− pups from betaine-supplemented dams. We observed disturbances in proliferation and differentiation in the cerebellum and hippocampus in the knockout mice; these changes were ameliorated by betaine supplementation. The dramatic effects of betaine on survival and growth, and the partial reversibility of the biochemical and developmental anomalies in the brains of MTHFR-deficient mice, emphasize an important role for choline and betaine depletion in the pathogenesis of homocystinuria due to MTHFR deficiency.

Journal ArticleDOI
TL;DR: An exposure to Brucella melitensis that occurred in a large microbiology laboratory is reported and the strategy chosen for antibiotic prophylaxis and serological follow-up of exposed workers is described.
Abstract: Brucellosis is one of the most common laboratory-acquired infections, mostly because aerosolization is a mechanism of transmission in this setting. We report an exposure to Brucella melitensis that occurred in a large microbiology laboratory and describe the strategy chosen for antibiotic prophylaxis and serological follow-up of exposed workers.

Journal ArticleDOI
TL;DR: Dynamic video rhinoscopy is more accurate at assessing adenoid hypertrophy, and the percentage of airway occlusion, as estimated by video rhinescopy, is better correlated to the severity of symptoms than are values obtained by lateral neck radiography.
Abstract: Objective: To evaluate the usefulness of adynamic lateral neck radiographs and dynamic video rhinoscopy in assessing adenoid size and the relationship of these methods to associated symptoms and thus the severity of the disease. Methods: Children with suspected adenoid hypertrophy underwent standard lateral neck soft tissue radiographs: the percentage of airway occlusion, adenoid to nasopharynx (AN) ratio, airway to soft palate ratio, and adenoid thickness were assessed by a radiologist. The percentage of airway closure was assessed by direct fibre-optic rhinoscopy in an ear, nose, and throat clinic. Associated clinical symptoms were assessed by parents using a standardized questionnaire, evaluating the severity of symptoms (snoring, sleep apnea, mouth breathing, and otitis media) to give a total symptom score out of 16. Results: Nonparametric statistical analysis using Spearman’s correlation coefficients was performed on 32 patients. There was a weak correlation, which approaches significance, between the percentage of airway occlusion assessed by fibre-optic rhinoscopy and the total symptom score (r = .344, p = .054). However, this correlation becomes significant when the frequency of otitis media is omitted (r = .367, p = .039). There was also a significant correlation between airway occlusion assessed by rhinoscopy and the percentage of airway occlusion as determined by lateral neck radiography (r = .431, p = .014). There was no correlation between any of the measurements taken by lateral soft tissue neck radiography and total symptom score. Conclusion: Dynamic video rhinoscopy is more accurate at assessing adenoid hypertrophy, and the percentage of airway occlusion, as estimated by video rhinoscopy, is better correlated to the severity of symptoms than are values obtained by lateral neck radiography. Sommaire

Journal ArticleDOI
TL;DR: A 5‐month‐old boy required sedation after a cleft lip repair and developed a metabolic acidosis followed by multiple cardiac dysrhythmias, hepatic and renal failure, and his multisystem organ failure gradually resolved after initiation of charcoal haemoperfusion.
Abstract: A 5-month-old boy required sedation after a cleft lip repair. He was sedated with propofol and intermittent fentanyl, requiring escalating doses over the subsequent 48 h. On the second post-operative day he developed a metabolic acidosis followed by multiple cardiac dysrhythmias, hepatic and renal failure. Propofol was stopped. His multisystem organ failure gradually resolved after initiation of charcoal haemoperfusion. Further investigation demonstrated an abnormality in acylcarnitine metabolism, similar to that found in one previous case report.

Journal ArticleDOI
TL;DR: The analysis of the second harvest of the STS Congenital Heart Surgery Database produced meaningful outcome data and several critical lessons relevant to congenital heart surgery outcome analysis worldwide as discussed by the authors, including: (1) Death must be clearly defined. (2) Primary Procedure in a given operation must be documented.
Abstract: Objective: The analysis of the second harvest of the STS Congenital Heart Surgery Database produced meaningful outcome data and several critical lessons relevant to congenital heart surgery outcomes analysis worldwide. Methods: This data harvest represents the first STS multi-institutional experience with software utilizing the nomenclature and database requirements adopted by the STS and EACTS (April 2000 Annals of Thoracic Surgery). Members of the STS Congenital Heart Committee analyzed the STS data. Results: This STS harvest includes data from 16 centers (12787 cases, 2881 neonates, 4124 infants). In 2002, the EACTS reported similar outcome data utilizing the same database definitions (41 centers, 12736 cases, 2245 neonates, 4195 infants). Lessons from the analysis include: (1) Death must be clearly defined. (2) The Primary Procedure in a given operation must be documented. (3) Inclusionary and exclusionary criteria for all diagnoses and procedures must be agreed upon. (4) Missing data values remain an issue for the database. (5) Generic terms in the nomenclature lists, that is terms ending in Not Otherwise Specified (NOS), are redundant and decrease the clarity of data analysis. (6) Methodology needs to be developed and implemented to assure and verify data completeness and data accuracy. 'Operative Mortality' and 'Mortality Assigned to this Operation' were defined by the STS and EACTS; these definitions were not utilized uniformly. 'Thirty Day Mortality' was problematic because some centers did not track mortality after hospital discharge. Only 'Mortality Prior to Discharge' was consistently reported. Designation of Primary Procedure for a given operation determines its location for analysis. Until Complexity Scores lead to automated methodology for choosing the Primary Procedure, the surgeon must designate the Primary Procedure. Inclusionary and exclusionary criteria for all diagnoses and procedures have been developed in an effort to define acceptable concomitant diagnoses and procedures for each analysis. Improvements in data completeness can be achieved using a variety of techniques including developing more functional techniques of data entry at individual institutions and software improvements. Future versions of the STS Congenital Database will request that the coding of diagnoses and procedures avoid the terms ending in NOS. Conclusions: Lessons from this data harvest should improve congenital heart surgery outcome analysis.

Journal ArticleDOI
TL;DR: In nonconduct-disordered, stimulant-treated children with ADHD, parent training does not improve self-rated parental behavior and the benefits of brief stimulant treatment for negative parental behavior are sustained with extended treatment.
Abstract: Objective To test the hypothesis that multimodal psychosocial intervention, which includes parent training, combined with methylphenidate significantly enhances the behavior of parents of children with attention-deficit/hyperactivity disorder (ADHD), compared with methylphenidate alone and compared with methylphenidate and nonspecific psychosocial treatment (attention control). Method One hundred three children with ADHD (ages 7–9), free of conduct and learning disorders, who responded to short-term methylphenidate therapy were randomized for 2 years to receive either (1) methylphenidate treatment alone; (2) methylphenidate plus psychosocial treatment that included parent training and counseling, social skills training, academic assistance, and psychotherapy; or (3) methylphenidate plus attention control treatment. Parents rated their knowledge of parenting principles and negative and positive parenting behavior. Children rated their parents' behavior. Results Psychosocial treatment led to significantly better knowledge of parenting principles but did not enhance parenting practices, as rated by parents and children. Significant improvement in mothers' negative parenting occurred across all treatments and was maintained. Conclusions In nonconduct-disordered, stimulant-treated children with ADHD, parent training does not improve self-rated parental behavior. The benefits of brief stimulant treatment for negative parental behavior are sustained with extended treatment.

Journal ArticleDOI
TL;DR: Whether abutment of adenoids laterally against the torus tubaris affects the outcome of patients requiring pressure equalization tubes (PET) for OM is determined.
Abstract: Objective: Nasopharyngeal adenoids may serve as a mechanical obstruction to the eustachian tube and contribute to the pathophysiology of otitis media (OM). The purpose of this study was to determine whether abutment of adenoids laterally against the torus tubaris affects the outcome of patients requiring pressure equalization tubes (PET) for OM. Study Design: Randomized, controlled, prospective clinical trial. Method: Patients requiring PET for recurrent acute OM or OM with persistent effusion were randomized into two groups: 1) PET placement and 2) PET placement and adenoidectomy, regardless of whether the adenoids were abutting or not abutting the torus tubaris. Patients were followed for a minimum of 1 year to determine rate of treatment failure, defined as recurrence of acute OM (>3 times/year), OM with effusion, or reinsertion of PET. Results: Of the 34 patients in the abutting group, 16 patients underwent only PET insertion, of whom 8 (50%) failed, whereas 18 patients had combined PET placement and adenoidectomy, of whom 3 (17%) failed. There was a statistical difference between these two groups (P <.05). Of the 29 patients in the nonabutting group, 24 patients underwent only PET insertion, of whom 9 (37.5%) failed, whereas 5 patients underwent combined PET placement and adenoidectomy, of whom 2 (40%) failed. There was no statistical difference between these two groups (P =.92). Conclusion: This study demonstrates that the position of hypertrophied adenoids may alter the final otologic outcome of patients requiring PET insertion for OM. Patients with adenoids abutting the torus tubaris may benefit most from an adjuvant adenoidectomy.

Journal ArticleDOI
TL;DR: The use of plain catgut absorbable sutures in the repair of traumatic lacerations in children appears to be an acceptable alternative to nonabsorbable suture material because the long-term cosmetic outcome seems to be at least as good.
Abstract: Objectives: To show that the use of absorbable sutures in pediatric traumatic lacerations affords good long-term cosmesis and no increase in complications (infection, dehiscence rates, and need for surgical scar revision) when compared with wounds sutured with nonabsorbable sutures. Methods:This was a randomized clinical trial conducted in a pediatric emergency department. Patients 1–18 years of age who presented to the emergency department with lacerations < 12 hours old were recruited between January 1999 and December 2001. Exclusion criteria were the following: wounds that could be approximated by tissue adhesives, animal/human bites, gross contamination, puncture/crush wounds, wounds crossing joints, lacerations of tendon/nerve/cartilage, collagen vascular disease, immunodeficiency, diabetes mellitus, bleeding disorder, and scalp lacerations. Patients were randomized into one of two groups: those receiving absorbable plain gut sutures (group A) and those receiving nonabsorbable nylon sutures (group NA). Board-eligible/certified pediatric emergency physicians or clinical fellows performed laceration repair in a standardized approach. All wounds were reevaluated within ten days by a single research nurse who assessed the wounds using a previously validated wound evaluation score (WES) composed of six items (presence of step-off, contour irregularities, margin separation, edge inversion, extensive distortion, and overall cosmetic appearance). A score of 6/6 was considered optimal. The study nurse also noted the presence of infection and dehiscence. The patients were then seen by a single blinded plastic surgeon at four or five months who evaluated the wound using a previously validated visual analog scale of cosmesis (VAS). In addition, the surgeon repeated the WES and assessed the need for surgical scar revision. Results: A total of 147 patients were eligible, and 52 patients declined to participate. Of the 95 patients enrolled, 50 were randomized to group A and 45 to group NA. The two groups had similar ages, gender distributions, rates of use of sedation or steri-strips, wound lengths and widths, mechanisms of injury, and wound locations. At the short-term follow-up, no difference was found in the proportion of optimal WES scores between group A (63% of patients) and group NA (49% of patients) (relative risk = 0.73; 95% confidence interval [95% CI] = 0.45 to 1.17). No difference was found in the rates of infection and dehiscence between the two groups. Sixty-three of the 95 patients presented for long-term follow-up. The groups remained similar with respect to patient and wound characteristics as well as wound location. The average VAS score at four months was 79 (95% CI = 73 to 85) for group A and 66 (95% CI = 55 to 76) for group NA. In addition, no differences were found in the proportion of optimal WES between group A (36% of patients) and group NA (28% of patients) at four months (relative risk = 0.88; 95% CI = 0.62 to 1.26). Surgical scar revision was recommended for only three patients, of whom two were in group A. No patients chose to have their scars revised. No differences were found between group A and group NA for the rates of dehiscence (2% vs. 11%; p = 0.07) and infection (0 vs. 2; p = 0.3). Conclusions: The use of plain catgut absorbable sutures in the repair of traumatic lacerations in children appears to be an acceptable alternative to nonabsorbable sutures because the long-term cosmetic outcome seems to be at least as good. In this study, plain gut suture material seemed to provide slightly better cosmesis. In addition, no difference was found in the rate of dehiscence or infection between the groups.

Journal ArticleDOI
TL;DR: Use of CAM is common among patients with juvenile idiopathic arthritis and is not related to any decrease in adherence to conventional medical treatment.
Abstract: Objective To describe the frequency of complementary and alternative medicine (CAM) use in patients with juvenile idiopathic arthritis (JIA) and to explore whether CAM was associated with patient-specific characteristics, parent-specific characteristics, and medical management factors. Methods Parents of children with JIA completed questionnaires that addressed the use of CAM, adherence and perceived problems, parental distress, and coping with childhood illness. Clinical variables were abstracted from the patients' medical files. Results One hundred-eighteen children with JIA, mean ± SD age of 10.4 ± 3.9 years and mean ± SD disease duration of 4.5 ± 3.5 years, participated in the study. Ever use of CAM was 33.9% and was higher in patients whose parents used CAM (odds ratio [OR] 5.1, 95% confidence interval [95% CI] 1.4–19.5) and among those who considered themselves as “Canadian” as opposed to belonging to a specific ethnic group (OR 10. 7, 95% CI 1.2–99.8). Adherence to conventional treatment was high for both users and nonusers of CAM. Conclusion Use of CAM is common among patients with JIA. CAM use is not related to any decrease in adherence to conventional medical treatment.