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1.
Pediatr Emerg Care ; 36(12): e704-e708, 2020 Dec.
Article in English | MEDLINE | ID: mdl-30247454

ABSTRACT

OBJECTIVE: The objective of this study was to evaluate whether residents can accurately estimate children's weight using the Broselow tape. METHOD: We conducted a preplanned secondary analysis from an experimental trial. Participants were residents in pediatrics, family medicine, and emergency medicine rotating in the ED. Residents were randomly assigned to 2 sets of paired scenarios during 2 sessions. They were asked to estimate the weight of a manikin using the Broselow tape at the beginning of each scenario. The first scenario from the initial session and the last scenario from the second session were used for the current study. The primary analysis was the proportion of participants who accurately estimated manikin weight within a 10% margin of error. RESULTS: Forty residents were recruited. Thirty-two (80%) reported knowledge of the Broselow tape and 13 (32.5%) reported previous use. Weight estimation was accurate in 60% (24/40; 95% confidence interval [CI], 45%-74%) during the first scenarios. Error in weight estimation differed by greater than 25% in 28% (11/40). Error in estimation was not associated with previous knowledge (odds ratio, 6.2; 95% CI, 0.68-56) or previous use (odds ratio, 0.9; 95% CI, 0.23-3.5) of the Broselow tape. In the last scenario, 88% accurately estimated manikin weight (35/40; 95% CI, 73%-95%). CONCLUSIONS: Although most residents reported knowledge of the Broselow tape, 40% made erroneous weight estimations by at least 10% with the first use in this simulation study. With repeated use, they improved significantly over time. Teaching appropriate use of the Broselow tape should be part of residency-training curricula and pediatric advanced life support course.


Subject(s)
Anthropometry , Body Weight , Emergency Medicine , Manikins , Anthropometry/instrumentation , Anthropometry/methods , Child , Humans , Odds Ratio , Simulation Training
2.
Am J Med Genet A ; 179(2): 196-205, 2019 02.
Article in English | MEDLINE | ID: mdl-30569567

ABSTRACT

Prader-Willi syndrome (PWS) is a multi-system disorder resulting from a lack of paternal gene expression in the 15q11.2-q13 region. Using databases compiled through response questionnaires completed by families known to the Prader-Willi Syndrome Association (USA), this study tested the hypothesis that PWS genetic subtype, BMI, age of diagnosis, clinical symptoms, and growth hormone treatment differ among deceased and living individuals with PWS. Categorical and continuous variables were compared using chi-square and two-group t tests, respectively. Deceased individuals had higher rates of clinical features, including increased weight concerns, heart problems, sleep apnea, other respiratory complications, diabetes, osteoporosis, high pain tolerance, and severe skin picking, when compared to living individuals. Meanwhile, living individuals had higher rates of growth hormone use and early puberty. Obesity and subsequent consequences are the primary contributors to increased mortality in PWS. Additional emphasis on areas to decrease mortality is needed.


Subject(s)
Growth Hormone/therapeutic use , Heart Failure/mortality , Obesity/mortality , Prader-Willi Syndrome/mortality , Adolescent , Adult , Aged , Aged, 80 and over , Body Mass Index , Child , Child, Preschool , Chromosomes, Human, Pair 15/genetics , Female , Heart Failure/complications , Heart Failure/genetics , Heart Failure/therapy , Humans , Infant , Infant, Newborn , Longitudinal Studies , Male , Middle Aged , Obesity/complications , Obesity/genetics , Obesity/therapy , Paternal Inheritance/genetics , Prader-Willi Syndrome/complications , Prader-Willi Syndrome/genetics , Prader-Willi Syndrome/therapy , Young Adult
3.
Genet Med ; 16(2): 164-9, 2014 Feb.
Article in English | MEDLINE | ID: mdl-23928912

ABSTRACT

PURPOSE: Prader-Willi syndrome is an imprinting disorder characterized by typical facial, physical, and cognitive/behavioral features, resulting from lack of paternally expressed genes on chromosome 15q11.2-q13. Studies have suggested an increased risk of other imprinting disorders in children conceived by assisted reproductive techniques. This study was designed to determine the association between assisted reproductive technology and Prader-Willi syndrome. METHODS: Data on individuals with Prader-Willi syndrome were collected from three distinct sources and the proportion of assisted reproductive technology births analyzed. RESULTS: The proportions of assisted reproductive technology births in the Prader-Willi Syndrome Association (USA), Rare Diseases Clinical Research Network, and University of California, Irvine Medical Center populations were 1.0% (18/1,736), 1.0% (1/98), and 2.0% (1/50), respectively (overall 1.1%; population frequency for the United States was 1.0%). Of note, 2.4% (45/1,898) of participants were co-twins (11 born after assisted reproductive technology procedures); US twin frequency is 1.6% (P = 0.007). The proportion of individuals with maternal disomy 15/imprinting defects born after assisted reproductive technology was higher than that in the total sample, 55.6% (10/18) and 34.5% (431/1,250), respectively. CONCLUSION: This study found no association between assisted reproductive technology and Prader-Willi syndrome. There was an increased frequency of twinning. The number of individuals with maternal disomy 15/imprinting defect was nearly double in the assisted reproductive technology group as compared with the total Prader-Willi syndrome participants.


Subject(s)
Prader-Willi Syndrome/epidemiology , Reproductive Techniques, Assisted/adverse effects , Chromosomes, Human, Pair 15 , Genomic Imprinting , Health Surveys , Humans , Prader-Willi Syndrome/genetics , Twins , Uniparental Disomy
4.
Genes (Basel) ; 10(7)2019 07 19.
Article in English | MEDLINE | ID: mdl-31331040

ABSTRACT

Prader-Willi Syndrome Association (USA) monitors the ongoing health and welfare of individuals with Prader-Willi syndrome (PWS) through active communication with members by membership surveys and data registries. Thromboembolism and blood clots have emerged in clinical studies as significant risk factors for injury and death in PWS. A 66-item questionnaire was developed by a panel of PWS medical and scientific experts, with input from Prader-Willi Syndrome Association (USA) leadership, so as to probe their membership on the frequency, risk, and protective factors for venous thromboembolism, pulmonary embolism, and related findings. The characteristics of those with and without a reported history of blood clots and related health factors were tabulated and analyzed. Responses were obtained for 1067 individuals with PWS (554 females and 513 males), and 38 (23 females and 15 males) had a history of blood clots. The individuals with clots did not differ by gender, but were significantly older 32.8 ± 15 years vs 20.4 ± 13 years, and were more likely to have a reported history of obesity (76%), edema (59%), hypertension (24%), vasculitis (33%), and family history of blood clots (33%) than those without clots. Growth hormone treatment was more common in individuals without clots. The risk factors for thromboembolism in PWS overlap those commonly observed for the general population.


Subject(s)
Prader-Willi Syndrome/complications , Venous Thromboembolism/etiology , Adolescent , Adult , Cause of Death , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged , Prader-Willi Syndrome/mortality , Risk Factors , Surveys and Questionnaires , Venous Thromboembolism/mortality , Young Adult
5.
Pediatr Emerg Care ; 24(5): 321-30, 2008 May.
Article in English | MEDLINE | ID: mdl-18496120

ABSTRACT

OBJECTIVE: To evaluate which treatment could be effective in the emergency department (ED) for children with migraine and status migrainosus, we carried out a qualitative systematic review of randomized controlled trials (RCTs) that evaluated treatment that could be used for those conditions. METHODS: Databases (Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects, Cochrane Controlled Trials Register, MedLine, and EMBASE) were searched for RCTs that evaluated treatment of migraine in children (<18 years of age). Guidelines published on the subject were checked for missed references. Characteristics of the identified studies as well as primary outcome (headache relief), other recognized primary outcomes, and adverse events were abstracted. Quality of the RCTs was evaluated using the Jadad score. RESULTS: Of the 14 trials included in the review, only 1 was performed in an ED after other treatments have failed. In that situation, prochlorperazine was more effective than ketorolac in relieving pain at 1 hour. Other treatments were evaluated by neurologists on their outpatients who started the studied drugs early at the beginning of the migraine without previous treatment. In that situation, ibuprofen (n = 3) and acetaminophen (n = 1) were better than placebo for pain relief. The efficacy of intranasal sumatriptan (n = 4), oral rizatriptan (n = 3), and oral zolmitriptan (n = 2) for pain relief was unclear. Oral sumatriptan (n = 1) and oral dihydroergotamine (n = 1) were not effective. CONCLUSIONS: There is a lack of studies addressing the question of treatment in the ED for children experiencing migraine. Although other treatments were found effective in children with migraine, none was evaluated in the ED except prochlorperazine and ketorolac.


Subject(s)
Analgesics, Non-Narcotic/therapeutic use , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Emergency Service, Hospital/statistics & numerical data , Migraine Disorders/drug therapy , Vasoconstrictor Agents/therapeutic use , Adolescent , Child , Humans , Migraine Disorders/epidemiology , Prevalence , Randomized Controlled Trials as Topic , Treatment Outcome
6.
Pediatrics ; 139(3)2017 Mar.
Article in English | MEDLINE | ID: mdl-28246338

ABSTRACT

OBJECTIVE: To evaluate whether a clinical aid providing precalculated medication doses decreases prescribing errors among residents during pediatric simulated cardiopulmonary arrest and anaphylaxis. METHODS: A crossover randomized trial was conducted in a tertiary care hospital simulation center with residents rotating in the pediatric emergency department. The intervention was a reference book providing weight-based precalculated doses. The control group used a card providing milligram-per-kilogram doses. The primary outcome was the presence of a prescribing error, defined as a dose varying by ≥20% from the recommended dose or by incorrect route. Residents were involved in 2 sets of paired scenarios and were their own control group. Primary analysis was the difference in mean prescribing error proportions between both groups. RESULTS: Forty residents prescribed 1507 medications or defibrillations during 160 scenarios. The numbers of prescribing errors per 100 bolus medications or defibrillations were 5.1 (39 out of 762) and 7.5 (56 out of 745) for the intervention and control, respectively, a difference of 2.4 (95% confidence interval [CI], -0.1 to 5.0). However, the intervention was highly associated with lower risk of 10-fold error for bolus medications (odds ratio 0.27; 95% CI, 0.10 to 0.70). For medications administered by infusion, prescribing errors occurred in 3 out of 76 (4%) scenarios in the intervention group and 13 out of 76 (22.4%) in the control group, a difference of 13% (95% CI, 3 to 23). CONCLUSIONS: A clinical aid providing precalculated medication doses was not associated with a decrease in overall prescribing error rates but was highly associated with a lower risk of 10-fold error for bolus medications and for medications administered by continuous infusion.


Subject(s)
Drug Dosage Calculations , Emergency Service, Hospital , Formularies, Hospital as Topic , Medication Errors/prevention & control , Adult , Anaphylaxis/drug therapy , Cross-Over Studies , Electric Countershock , Emergency Medicine/education , Female , Humans , Internship and Residency , Male , Manikins , Medication Errors/statistics & numerical data , Pediatrics/education , Quebec , Simulation Training , Tachycardia, Ventricular/therapy , Ventricular Fibrillation/therapy , Young Adult
7.
Pediatrics ; 124(1): 16-22, 2009 Jul.
Article in English | MEDLINE | ID: mdl-19564278

ABSTRACT

OBJECTIVE: The goal was to examine the feasibility of outpatient management for 1- to 3-month-old infants with febrile urinary tract infections. METHODS: A cohort study was performed with all children 30 to 90 days of age who were evaluated for presumed febrile urinary tract infections in the emergency department of a tertiary-care pediatric hospital between January 1, 2005, and September 30, 2007. Patients were treated with intravenously administered antibiotics as outpatients in a day treatment center unless they met exclusion criteria, in which case they were hospitalized. RESULTS: Of 118 infants included in the study, 67 (56.8%) were admitted to the day treatment center and 51 (43.2%) were hospitalized. The median age of day treatment center patients was 66 days (range: 33-85 days). The diagnosis of urinary tract infection was confirmed for 86.6% of patients treated in the day treatment center. Escherichia coli was identified in 84.5% of urine cultures; 98.3% of isolates were sensitive to gentamicin. Six blood cultures (10.3%) yielded positive results, 5 of them for E coli. Treatment with intravenously administered antibiotics in the day treatment center lasted a mean of 2.7 days. The mean number of visits, including appointments for voiding cystourethrography, was 2.9 visits. The rate of parental compliance with day treatment center visits was 98.3%. Intravenous access problems were seen in 8.6% of cases. Successful treatment in the day treatment center (defined as attendance at all visits, normalization of temperature within 48 hours, negative control urine and blood culture results, if cultures were performed, and absence of hospitalization from the day treatment center) was obtained for 86.2% of patients with confirmed urinary tract infections. CONCLUSIONS: Ambulatory treatment of infants 30 to 90 days of age with febrile urinary tract infections by using short-term, intravenous antibiotic therapy at a day treatment center is feasible.


Subject(s)
Ambulatory Care , Anti-Bacterial Agents/administration & dosage , Urinary Tract Infections/drug therapy , Bacteremia/epidemiology , Escherichia coli Infections/drug therapy , Feasibility Studies , Hospitalization/statistics & numerical data , Humans , Infant , Infusions, Intravenous , Multivariate Analysis , Urinary Tract Infections/microbiology
8.
J Immunol ; 179(12): 8026-34, 2007 Dec 15.
Article in English | MEDLINE | ID: mdl-18056342

ABSTRACT

T cell recognition of Ag can result in priming or tolerance depending on the context in which Ag is recognized. Previously, we have reported that these distinct functional outcomes are associated with marked differences in the amplitude, kinetics, and cellular localization of activated, pERK signals at the level of individual Ag-specific T cells in vitro. Here, we show that the GTPase Rap1, which can antagonize the generation of such pERK signals and has been reported to accumulate in tolerant cells, exhibits an inverse pattern of expression to pERK in individual Ag-specific primed and tolerized T cells. Although pERK is expressed by more primed than tolerized T cells when rechallenged with Ag in vitro, Rap1 is expressed by higher percentages of tolerant compared with primed Ag-specific T cells. Moreover, whereas pERK localizes to the TCR and lipid rafts in primed cells, but exhibits a diffuse cellular distribution in tolerized cells, Rap1 colocalizes with the TCR and lipid raft structures under conditions of tolerance, but not priming, in vitro. This inverse relationship between Rap1 and pERK expression is physiologically relevant, given that we observed the same patterns in Ag-specific T cells in situ, following induction of priming and tolerance in vivo. Together, these data suggest that the maintenance of tolerance of individual Ag-specific T cells may reflect the recruitment of up-regulated Rap1 to the immune synapse, potentially resulting in sequestration of Raf-1 and uncoupling of the TCR from the Ras-ERK-MAPK cascade.


Subject(s)
Antigen Presentation , CD4-Positive T-Lymphocytes/immunology , Extracellular Signal-Regulated MAP Kinases/metabolism , Immune Tolerance , rap1 GTP-Binding Proteins/metabolism , Animals , Antigens/immunology , CD4-Positive T-Lymphocytes/enzymology , Extracellular Signal-Regulated MAP Kinases/analysis , Extracellular Signal-Regulated MAP Kinases/antagonists & inhibitors , Mice , Mice, Transgenic , Receptors, Antigen, T-Cell/analysis , Receptors, Antigen, T-Cell/genetics , rap1 GTP-Binding Proteins/analysis
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