Beyond terminology: the policy impact of a grassroots movement.

This article discusses the history of the grassroots movement led by self-advocates and their families to replace the stigmatizing term "mental retardation" with "intellectual disability" in federal statute. It also describes recent and pending changes in federal regulations and policy to adopt the new terminology for Social Security and Medicaid.

Can magnetic resonance spectroscopy predict neurodevelopmental outcome in very low birth weight preterm infants?

OBJECTIVE: To determine if metabolite ratios at near-term age predict outcome in very low birth weight preterm infants at 18 to 24 months adjusted age. STUDY DESIGN: Thirty-six infants (birth weight

Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada.

BACKGROUND: An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. METHODS: Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes. RESULTS: Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK. CONCLUSIONS: This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.

Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies.

A collaborative study of human transmissible spongiform encephalopathies has been carried out from 1993 to 2000 and includes data from 10 national registries, the majority in Western Europe. In this study, we present analyses of predictors of survival in sporadic (n = 2304), iatrogenic (n = 106) and variant Creutzfeldt-Jakob disease (n = 86) and in cases associated with mutations of the prion protein gene (n = 278), including Gerstmann-Sträussler-Scheinker syndrome (n = 24) and fatal familial insomnia (n = 41). Overall survival for each disease type was assessed by the Kaplan-Meier method and the multivariate analyses by the Cox proportional hazards model. In sporadic disease, longer survival was correlated with younger age at onset of illness, female gender, codon 129 heterozygosity, presence of CSF 14-3-3 protein and type 2a prion protein type. The ability to predict survival based on patient covariates is important for diagnosis and counselling, and the characterization of the survival distributions, in the absence of therapy, will be an important starting point for the assessment of potential therapeutic agents in the future.

Variation in utilization of computed tomography scanning for the investigation of minor head trauma in children: a Canadian experience.

OBJECTIVES: To compare the utilization rates of CT scans in investigating minor head trauma in children in Canada, to identify the injuries determined by these scans, and to identify clinical findings that are highly associated with its diagnosis and the injury itself. METHODS: A retrospective cohort study involving nine pediatric hospitals in Canada was conducted. A structured data collection method was used. Inclusion criteria included age 16 years or less, history of blunt head trauma, and a Glasgow Coma Scale score (GCS) greater than or equal to 13. Data collected included demographic information, type of injury, relevant clinical information, computed tomography (CT) scan data, and clinical outcome. Clinical findings associated with CT scan and positive CT scan were identified using logistic regression. RESULTS: One thousand one hundred sixty-four children were included in the study. One hundred seventy-one (15%) had a CT scan, of which 60 (35%) were abnormal. There was a significant difference in the rate of ordering of CT scans among the participating hospitals, but no significant difference in the rate of abnormal CT scans. Mechanism of injury, GCS, and loss of consciousness were significantly related to the presence of an abnormal CT scan. CONCLUSIONS: Although there is a significant difference in the utilization of CT scans to investigate minor head trauma in children across Canada, there is no significant difference in the frequency of head injuries in these patients. This suggests that it may be possible to determine clinical criteria that are predictive of a head injury in these patients.

Blepharophimosis, minor facial anomalies, genital anomalies, and mental retardation: report of two sibs with a unique syndrome.

We report on two sibs, a 2.5-year-old girl and a 10-month-old boy, with a hitherto unreported combination of congenital anomalies: blepharophimosis, ptosis, midface hypoplasia, abnormal palate, low anterior and posterior hairlines, displaced hair whorl, apparently low-set and abnormally shaped ears, trigonocephaly, dental anomalies, laryngomalacia, sensorineural hearing loss, genital anomalies, hypotonia, and mental retardation. The occurrence of a similar pattern of anomalies in two sibs of opposite sex suggests autosomal recessive inheritance. To our knowledge, this combination of anomalies has not been reported previously, and thus we propose it to be a formal genesis syndrome.

Descriptive study of sledding injuries in Canadian children.

OBJECTIVES: (1) To describe the characteristics of sledding injuries presenting to a pediatric emergency department and (2) To describe the sledding environment that leads to childhood sledding injuries. SETTING: A pediatric hospital emergency department in Ottawa, Canada and identified sledding sites in the region. METHODS: All patients less than 18 years with sled related injuries were included. Questionnaires were completed gathering information on the sled operator, the sled, the sledding site, and the injury. Site visits were made to designated and non-designated sledding hills in the Ottawa region to record data regarding sled operators, sleds, and the sledding environment. RESULTS: Ninety-five patients were identified with sledding injuries and 81 (85%) completed the questionnaire. The mean age was 9.9 years (range 8 months to 17 years). The majority were male (63%). Most injuries occurred on non-designated sledding hills in the community (70%). Mild to moderate injuries were most common, however nine patients (11%) were admitted to hospital. Fifty-one per cent had adult supervision at the time of injury compared with 86% observed at the site visits. Common mechanisms of injuries were collisions with objects (33%), falls in icy conditions (28%), and going off jumps (16%). Most serious injuries occurred with contact with motor vehicles. There was no relationship between the type of sled used and the likelihood of injury. CONCLUSIONS: Sledding hills which have obstacles, icy conditions, jumps, or proximity to roads may result in more childhood injuries. Children with no adult supervision are likely at higher risk of injury.

A randomized, clinical trial comparing butylcyanoacrylate with octylcyanoacrylate in the management of selected pediatric facial lacerations.

OBJECTIVE: To compare two tissue adhesives, butylcyanoacrylate and octylcyanoacrylate, in the treatment of small (<4 cm) superficial linear traumatic facial lacerations in children. METHODS: This was a randomized, clinical trial with parallel design. 94 children <18 years of age seen in the ED of a tertiary care pediatric hospital with a facial laceration suitable for tissue adhesive closure underwent laceration closure using either butylcyanoacrylate or octylcyanoacrylate. The primary outcome was the cosmetic result at three months rated from photographs by a plastic surgeon on a visual analog scale (VAS). Secondary outcomes included the time to perform the procedure, the perceived difficulty of the procedure, the pain perceived by the patient, and a wound evaluation score at ten to 14 days and three months. RESULTS: Ninety-four patients were randomized with 47 in each group. The two groups were similar for baseline demographic and clinical characteristics. There was no difference in the three-month cosmesis VAS (median, 70.0 mm for n-butyl-2-cyanoacrylate vs 67.5 mm for octylcyanocrylate, p = 0.84). There was no difference between the groups for time to complete the procedure (p = 0.88), parent/patient-perceived pain of the procedure (p = 0.37), or physician-perceived difficulty of the procedure (p = 0.33). Similarly, there was no difference between the groups for the percentage of early (p = 0.58) or late (p = 0.71) optimal wound evaluation scores. CONCLUSIONS: In the closure of small linear pediatric facial lacerations, octylcyanoacrylate is similar to butylcyanoacrylate in ease of use and early and late cosmetic outcomes. The superior physical properties of octylcyanoacrylate appear to add little benefit to the management of these selected lacerations. Physician preference and differing costs may dictate use for these small selected lacerations.

Tissue adhesive versus suture wound repair at 1 year: randomized clinical trial correlating early, 3-month, and 1-year cosmetic outcome.

STUDY OBJECTIVE: To compare the 1-year cosmetic outcome of wounds treated with octylcyanoacrylate tissue adhesive and monofilament sutures and to correlate the early, 3-month, and 1-year cosmetic outcomes. METHODS: We prospectively randomized 136 cases of traumatic laceration to repair with octylcyanoacrylate tissue adhesive or 5-0 or smaller monofilament suture. A wound score was assigned by a research nurse, and validated by a second nurse blinded to the treatment, at 5 to 10 days after injury (early), 3 months, and 1 year. Standardized photographs were taken at 3 months and 1 year and shown to a cosmetic surgeon blinded to the method of closure, who rated the wounds on a validated cosmesis scale. RESULTS: We were able to examine 77 lacerations at 1 year for follow-up. No differences were found in the demographic or clinical characteristics between groups. Likewise, at 1 year no difference was found in the optimal wound scores (73% versus 68%, P =.60) or in visual analog scale cosmesis scores (69 versus 69 mm, P =.95) for octylcyanoacrylate and sutures, respectively. Agreement was poor between early and 3-month wound scores (kappa=.34; 95% confidence interval [CI],.10 to.58) but a strong association existed between 3-month and 1-year wound scores (kappa=.71; 95% CI,.52 to.90). We noted a moderate correlation between 3-month and 1-year results on the visual analog cosmesis scale (intraclass correlation,.48; 95% CI, .30 to.63). CONCLUSION: One year after wound repair, no difference is noted in the cosmetic outcomes of traumatic lacerations treated with octylcyanoacrylate tissue adhesive and sutures. The assessment of wounds 3 months after injury and wound repair provides a good measure of long-term cosmetic outcome.

Nebulized budesonide and oral dexamethasone for treatment of croup: a randomized controlled trial.

CONTEXT: The effectiveness of glucocorticoids for patients with croup is well established but it remains uncertain which glucocorticoid regimen is most effective. OBJECTIVE: To determine the effectiveness of 3 glucocorticoid regimens in patients with croup. DESIGN: Randomized controlled trial with parallel design. SETTING: Emergency departments of 2 Canadian pediatric tertiary care hospitals. PARTICIPANTS: Children with a clinical syndrome consistent with croup, aged 3 months to 5 years, with a croup score of 2 or greater following at least 15 minutes of mist therapy. INTERVENTIONS: Oral dexamethasone, 0.6 mg/kg, and nebulized placebo; oral placebo and nebulized budesonide, 2 mg; or oral dexamethasone, 0.6 mg/kg, and nebulized budesonide, 2 mg. MAIN OUTCOME MEASURES: Westley croup score (primary outcome), hospital admission rates, time spent in the emergency department, return visits to the emergency department, or ongoing symptoms at 1 week. RESULTS: The mean change in the croup score from baseline to the final study assessment was -2.3 (95% confidence interval [CI], -2.6 to -2.0) in the budesonide group (n = 65), -2.4 (95% CI, -2.6 to -2.2) in the dexamethasone group (n = 69), and -2.4 (95% CI, -2.7 to -2.1) in the budesonide and dexamethasone group (n = 64, P = .70). CONCLUSIONS: Based on the similar outcomes in the 3 groups, oral dexamethasone is the preferred intervention because of its ease of administration, lower cost, and more widespread availability.

A randomized trial comparing octylcyanoacrylate tissue adhesive and sutures in the management of lacerations.

OBJECTIVE: To assess the effectiveness of a new tissue adhesive for laceration closure. DESIGN: A prospective, randomized controlled trial. SETTING: An adult teaching hospital. PARTICIPANTS: One hundred thirty patients with 136 lacerations who consented to enrollment during a 5-month period. The lacerations included all eligible nonmucosal facial lacerations, as well as selected extremity and torso lacerations (not on hands, feet, or joints). One hundred six lacerations were available for early follow-up, and 98 were available for 3-month evaluation. INTERVENTIONS: Lacerations were randomly allocated to have skin closure with octylcyanoacrylate adhesive or monofilament suture. MAIN OUTCOME MEASURE: A 3-month photograph of the wound was assigned a cosmesis score on a previously validated 100-mm visual analog cosmesis scale by a plastic surgeon who was unaware of the method of wound closure. RESULTS: There were no differences in the mean visual analog cosmesis scores (67 mm for octylcyanoacrylate vs 68 mm for sutures; P=.65). Similarly, there was no difference in the percentage of early (80% vs 82%; P=.80) or late (72% vs 75%; P=.74) optimal wound evaluation scores. The tissue adhesive was a faster method of wound repair (3.6 vs 12.4 minutes; P<.001) as well as being less painful (visual analog pain scores, 7.2 vs 18.0 mm; P<.001). CONCLUSIONS: Octylcyanoacrylate tissue adhesive effectively closes selected lacerations. This relatively painless and fast method of wound repair can replace the need for suturing several million lacerations each year.

Dexamethasone in salbutamol-treated inpatients with acute bronchiolitis: a randomized, controlled trial.

OBJECTIVE: To determine the clinical benefit of oral dexamethasone in children admitted to the hospital with bronchiolitis treated with nebulized salbutamol. METHODS: Randomized, double-blind, placebo-controlled trial in the inpatient wards of a pediatric tertiary care hospital. The participants, children aged 6 weeks to 15 months, admitted with first-time wheezing, were eligible if their oxygen saturation was less than 95% on admission to the hospital and their Respiratory Distress Assessment Instrument (RDAI) score was greater than 6. Patients were excluded if they had any one of the following: an underlying disease that might affect cardiopulmonary status, asthma, recent treatment with steroids (within 2 weeks), or any history of adverse reaction to steroids. Patients were randomly assigned to receive either orally administered dexamethasone with 0.5 mg/kg as the first dose and 0.3 mg/kg for the next 2 mornings, or an equal volume of an orally administered placebo with an identical appearance. All patients received nebulized salbutamol at 0.15 mg/kg every 4 hours for the first 24 hours. The primary outcome measure was the change from baseline in the RDAI score at 24 hours. Secondary outcome measures were oxygen saturation, respiratory rate, RDAI measurement twice daily for the first 4 days, and the length of hospitalization. RESULTS: At 24 hours the mean change (SD) from baseline in the RDAI score was 1.6 (2.3) in the placebo group (n = 28) and 1.4 (2.0) in the dexamethasone group (n = 33; p = 0.74). There were no significant differences between the two groups in change in oxygen saturation, respiratory rate, and RDAI score at any assessment period. The median length of stay (95% confidence interval) for the placebo group was 48 (42, 54) hours compared with 57 (38, 76) hours in the dexamethasone group (p = 0.19). CONCLUSIONS: Oral dexamethasone therapy does not affect the clinical course of children hospitalized with bronchiolitis and therefore cannot be recommended in this clinical situation.

A prospective comparison of octylcyanoacrylate tissue adhesive and suture for the closure of head and neck incisions.

OBJECTIVE: To compare the tissue adhesive octylcyanoacrylate with subcuticular suture for the closure of head and neck incisions. DESIGN: A prospective comparison with a blinded assessment of cosmetic outcome. SUBJECTS: Fifty consecutive patients undergoing head and neck procedures at two University of Ottawa teaching hospitals. METHODS: Twenty-six patients underwent skin closure with monofilament suture and 24 were closed with tissue adhesive. At 4 to 6 weeks the incisions were evaluated with a validated wound scale. Photographs of the incisions were rated using a visual analogue scale by two facial-plastic otolaryngologists who were blinded to the method of skin closure. RESULTS: The adhesive provided faster skin closure (29.7 seconds vs 289.0 seconds, p < .0001), and there were no differences in complications between the two groups. The primary outcome measure was the cosmetic appearance of the incision at 4 to 6 weeks. Although the adhesive group scored higher on both cosmesis scales, the visual analogue scale (octylcyanoacrylate 58.7 mm vs suture 53.2 mm) and the wound evaluation scale (57% vs 50% optimal wound scores), there were no statistical or clinically significant differences on either scale. The two facial-plastic otolaryngologists had good intraobserver and interobserver agreement when rating the cosmetic outcomes (0.87 and 0.71 respectively). CONCLUSIONS: Octylcyanoacrylate was found to be an effective method of skin closure in clean head and neck incisions. The practical advantages of tissue adhesives are reviewed.

The efficacy of nebulized budesonide in dexamethasone-treated outpatients with croup.

OBJECTIVE: To determine the added clinical benefit of nebulized budesonide in children with mild to moderate croup treated with 0.6 mg/kg oral dexamethasone. DESIGN: Randomized, double-blind, placebo-controlled trial. SETTING: Emergency department of a tertiary-care pediatric hospital with 47,000 visits per year. PARTICIPANTS: Children 3 months to 5 years of age with a syndrome consisting of hoarseness, inspiratory stridor, and barking cough and a croup score of 3 or greater after at least 15 minutes of mist therapy. Patients were excluded from the study if they had diagnoses of epiglottitis, chronic upper or lower airway disease (not including asthma), or severe croup or had received corticosteroids within the preceding 2 weeks. INTERVENTION: All patients received 0.6 mg/kg oral dexamethasone and were randomly assigned to receive 4 mL (2 mg) of budesonide solution (n=25) or 4 mL of 0.9% saline solution (n=25) by updraft nebulizer with a continuous flow of oxygen at 5 to 6 L/min. MAIN OUTCOME MEASURES: The primary outcome measure was the proportion of patients in each group who had clinically important changes (two points) in the croup score during the 4 hours after treatment. RESULTS: Eighty-four percent (n=21) of the patients who received budesonide had clinically important responses, compared with 56% (n=14) in the placebo group. The number of patients who would need to be treated with nebulized budesonide for one patient to have a clinically important response is four patients. CONCLUSIONS: Despite receiving simultaneous oral dexamethasone, pediatric outpatients with mild to moderate croup have added, clinically important improvement in respiratory symptoms after treatment with budesonide.

Agreement in the interpretation of extremity radiographs of injured children and adolescents.

OBJECTIVES: To measure agreement beyond chance (kappa) for comparison interpretations of extremity radiographs by pediatric radiologists and emergency physicians (EPs) and to identify factors associated with disagreement. METHODS: A random sample of 205 radiographs was selected from 1,016 patients having x-rays of their extremities in the emergency and radiology departments of a tertiary care pediatric hospital. Interpretations by the "official" reporting pediatric radiologist (ORPR), the treating EP, and a pediatric radiologist blinded to all clinical information (BPR) were compared for three categories: "abnormal" (one or more of fracture, dislocation, or effusion); "possibly abnormal"; and "normal." RESULTS: The overall weighted kappa (Kw) for the ORPRs and the EPs was 0.55. For fractures alone, the Kw for the ORPRs vs the EPs was 0.77; and for effusions alone, the value was 0.34. The Kw for the ORPRs vs the BPR was 0.63 (range 0.43-0.83 for individual ORPRs). The main areas of disagreement were in the identification of joint effusions and of nondisplaced fractures of the phalanges, elbow joint, tarsals, or metatarsals. CONCLUSIONS: There is good agreement between EPs and pediatric radiologists in interpreting extremity radiographs of injured children and adolescents. Disagreement occurs mainly for effusions or minor fractures and for the elbow region. Because of the importance of recognizing abnormalities in this region, an educational intervention to improve this area of deficiency is recommended.

A randomized trial comparing the efficacy of epinephrine with salbutamol in the treatment of acute bronchiolitis.

This randomized, double-blind trial compared the efficacy of nebulized epinephrine with salbutamol in the treatment of infants with acute bronchiolitis. The mean percent oxygen saturation at 60 minutes was significantly higher in the epinephrine group. Thirty-three percent of the patients in epinephrine group were admitted to the hospital compared with 81% of the salbutamol group (p = 0.003). We conclude that nebulized epinephrine is more efficacious than salbutamol for infants with acute bronchiolitis seen in an emergency department.