This set of recommendations is designed to assist the pediatrician in caring for children with Williams syndrome (WS) who were diagnosed by using clinical features and with chromosome 7 microdeletion confirmed by fluorescence in situ hybridization, chromosome microarray, or multiplex ligation-dependent probe amplification. The recommendations in this report reflect review of the current literature, including previously peer-reviewed and published management suggestions for WS, as well as the consensus of physicians and psychologists with expertise in the care of individuals with WS. These general recommendations for the syndrome do not replace individualized medical assessment and treatment.
To study the longitudinal associations of 12th-grade binge drinking with driving while impaired (DWI), riding with an impaired driver (RWI), blackouts, extreme binge drinking, and risky driving (self-reported Checkpoints Risky Driving Scale) among emerging adults up to 4 years after leaving high school.METHODS:
The data were all 7 waves (W 1 to W 7 of the NEXT Generation Health Study; a US nationally representative study (N = 2785) with a probability cohort of 10th-graders (mean age = 16.2 years; SE = 0.03) starting in the 2009–2010 year. Binary and ordinal logistic regressions were used for the analysis.RESULTS:
Binge drinking prevalence in W1 to W3 was 27.2%, 23.8%, and 26.8%, respectively. Twelfth-grade binge drinking was associated with a higher likelihood of DWI, RWI, blackouts, and risky driving in W4 to W7 and extreme binge drinking in W7. Adolescents who binged ≥3 times in high school were more likely to DWI, RWI, blackout (W4 to W7), be involved in extreme binge drinking (W7), and report riskier driving several years after high school. In some waves, parental practices appeared to have enduring effects in protecting against DWI, RWI, and blackouts.CONCLUSIONS:
Twelfth-grade binge drinking is a robust predictor of early adulthood DWI, RWI, blackout, extreme binge drinking, and risky driving. Our study suggests that ongoing parental practices could be protective against DWI, RWI, and blackouts once adolescents transition from high school into early adulthood. Prevention programs that incorporate binge drinking–focused screening and bolster parental practices may reduce the likelihood of later major alcohol-related health-risk behaviors and consequences in emerging adults.
Oropharyngeal dysphagia and aspiration may occur in infants and children. Currently, there is wide practice variation regarding when to feed children orally or place more permanent gastrostomy tube placement. Through implementation of an evidence-based guideline (EBG), we aimed to standardize the approach to these patients and reduce the rates of gastrostomy tube placement.METHODS:
Between January 2014 and December 2018, we designed and implemented a quality improvement intervention creating an EBG to be used by gastroenterologists evaluating patients ≤2 years of age with respiratory symptoms who were found to aspirate on videofluoroscopic swallow study (VFSS). Our primary aim was to encourage oral feeding and decrease the use of gastrostomy tube placement by 10% within 1 year of EBG initiation; balancing measures included total hospital readmissions or emergency department (ED) visits within 6 months of the abnormal VFSS.RESULTS:
A total of 1668 patients (27.2%) were found to have aspiration or penetration noted on an initial VFSS during our initiative. Mean gastrostomy tube placement in these patients was 10.9% at the start of our EBG implementation and fell to 5.2% approximately 1 year after EBG initiation; this improvement was sustained throughout the next 3 years. Our balancing measures of ED visits and hospital readmissions also did not change during this time period.CONCLUSIONS:
Through implementation of this EBG, we reduced gastrostomy tube placement by 50% in patients presenting with oropharyngeal dysphagia and aspiration, without increasing subsequent hospital admissions or ED visits.
Big data (BD) in pediatric medication safety research provides many opportunities to improve the safety and health of children. The number of pediatric medication and device trials has increased in part because of the past 20 years of US legislation requiring and incentivizing study of the effects of medical products in children (Food and Drug Administration Modernization Act of 1997, Pediatric Rule in 1998, Best Pharmaceuticals for Children Act of 2002, and Pediatric Research Equity Act of 2003). There are some limitations of traditional approaches to studying medication safety in children. Randomized clinical trials within the regulatory context may not enroll patients who are representative of the general pediatric population, provide the power to detect rare safety signals, or provide long-term safety data. BD sources may have these capabilities. In recent years, medical records have become digitized, and cell phones and personal devices have proliferated. In this process, the field of biomedical science has progressively used BD from those records coupled with other data sources, both digital and traditional. Additionally, large distributed databases that include pediatric-specific outcome variables are available. A workshop entitled "Advancing the Development of Pediatric Therapeutics: Application of ‘Big Data’ to Pediatric Safety Studies" held September 18 to 19, 2017, in Silver Spring, Maryland, formed the basis of many of the ideas outlined in this article, which are intended to identify key examples, critical issues, and future directions in this early phase of an anticipated dramatic change in the availability and use of BD.
Bevacizumab is a human monoclonal immunoglobulin G1 antibody to vascular endothelial growth factor indicated in several adult diseases. Emerging literature and expert opinion support the off-label use of intravitreal bevacizumab in the treatment of retinopathy of prematurity (ROP), a common disease process seen in premature neonates. One of the most common side effects of systemic therapy in adults is hypertension; however, this has not been well described in infants receiving bevacizumab for ROP. In this report, we review a case of a former 25-week premature infant treated for stage 3 ROP with administration of intravitreal bevacizumab. The immediate posttreatment course was uncomplicated; however, at 10 days posttreatment, he developed new-onset systemic hypertension. In addition, neuroimaging revealed new areas of vasogenic edema, which improved over time. To the best of our knowledge and after a review of the literature, neither of these effects has been described in neonates after intravitreal bevacizumab for ROP.
The tuberculin skin test (TST) has been preferred for screening young children for latent tuberculosis infection (LTBI) because of concerns that interferon- release assays (IGRAs) may be less sensitive in this high-risk population. In this study, we compared the predictive value of IGRAs to the TST for progression to tuberculosis disease in children, including those <5 years old.METHODS:
Children <15 years old at risk for LTBI or progression to disease were tested with TST, QuantiFERON-TB Gold In-Tube test (QFT-GIT), and T-SPOT.TB test (T-SPOT) and followed actively for 2 years, then with registry matches, to identify incident disease.RESULTS:
Of 3593 children enrolled September 2012 to April 2016, 92% were born outside the United States; 25% were <5 years old. Four children developed tuberculosis over a median 4.3 years of follow-up. Sensitivities for progression to disease for TST and IGRAs were low (50%–75%), with wide confidence intervals (CIs). Specificities for TST, QFT-GIT, and T-SPOT were 73.4% (95% CI: 71.9–74.8), 90.1% (95% CI: 89.1–91.1), and 92.9% (95% CI: 92.0–93.7), respectively. Positive and negative predictive values for TST, QFT-GIT, and T-SPOT were 0.2 (95% CI: 0.1–0.8), 0.9 (95% CI: 0.3–2.5), and 0.8 (95% CI: 0.2–2.9) and 99.9 (95% CI: 99.7–100), 100 (95% CI: 99.8–100), and 99.9 (95% CI: 99.8–100), respectively. Of 533 children with TST-positive, IGRA-negative results not treated for LTBI, including 54 children <2 years old, none developed disease.CONCLUSIONS:
Although both types of tests poorly predict disease progression, IGRAs are no less predictive than the TST and offer high specificity and negative predictive values. Results from this study support the use of IGRAs for children, especially those who are not born in the United States.
Provision of high-quality care to acutely ill and injured children is a challenge to US hospitals because many have low pediatric volume. Delineating national trends in definitive pediatric acute care would inform improvements in care.METHODS:
We analyzed emergency department (ED) visits by children between 2008 and 2016 in the Nationwide Emergency Department Sample, a weighted sample of 20% of EDs nationally. For each hospital annually, we determined the Hospital Capability Index (HCI) to determine the frequency of definitive acute care, defined as hospitalization instead of ED transfer. Hospitals were classified annually according to 2008 HCI quartiles to understand shifts in pediatric capability.RESULTS:
The national median HCI was 0.06 (interquartile range: 0.01–0.17) in 2008 and 0.02 (interquartile range: 0.00–0.09) in 2016 (P < .001). Definitive care became less common regardless of annual pediatric volume, urban or rural designation, or condition frequency. In 2016, 2171 EDs (49.0%) had HCIs <0.013, which represented the lowest 25% of ED HCIs in 2008. Pediatric visits to EDs categorized in the bottom 2008 capability quartile more than doubled from 2.5 million in 2008 to 5.3 million in 2016. Despite decreasing capability, centers with higher annual pediatric volume and urban centers provided more definitive inpatient care and had fewer inter-ED transfers than lower-volume and rural centers.CONCLUSIONS:
Across the United States from 2008 to 2016, hospital provision of definitive acute pediatric care decreased, and ED visits to the hospitals least likely to provide definitive care increased. Systems improvements are needed to support hospital-based acute care of children.
Antibiotics are among the most common prescriptions in children, and non–β-lactam antibiotics (NBLAs) account for almost half of those prescribed in Australian pediatric hospitals. Despite this, data on NBLA hypersensitivity in children are limited. This study describes reported hypersensitivity reactions to NBLAs in children and the results of allergy evaluation.METHODS:
Children with a suspected NBLA allergy who had skin testing and/or an intravenous or oral challenge test (OCT) between May 2011 and June 2018 were included. Patients were excluded if they were >18 years old or did not complete the allergy evaluation for any reason other than allergic reaction.RESULTS:
Over the 7-year study period, 141 children had 150 allergy evaluations of 15 different NBLAs. The median time from the initial reported reaction to allergy evaluation was 1.9 (range 0.1–14.9) years. Overall, 27 of the 150 (18.0%) challenge tests to NBLAs had positive results, with the rate of positive OCT results being highest for trimethoprim-sulfamethoxazole (15 of 46; 32.6%) and macrolides (8 of 77; 10.4%). Although 4 children reported initial anaphylactic reactions, no patients had severe symptoms on rechallenge or required adrenaline. Of the challenges that had positive results, the majority of children (23 of 27; 85.2%) had symptoms on repeat challenge similar to those that were initially reported.CONCLUSIONS:
Overall, 8 of 10 children with NBLA allergy could be delabeled. On average, patients waited 1.9 years to be rechallenged. Timely access to allergy evaluation to delabel these patients is needed to preserve first-line antibiotics.
This is a joint policy statement from the American Academy of Pediatrics, American College of Emergency Physicians, Emergency Nurses Association, National Association of Emergency Medical Services Physicians, and National Association of Emergency Medical Technicians on pediatric readiness in emergency medical services systems.