Dexmedetomidine as Single Continuous Sedative During Noninvasive Ventilation: Typical Usage, Hemodynamic Effects, and Withdrawal.

Objectives: Dexmedetomidine use in pediatric critical care is increasing. Its prolonged effects as a single continuous agent for sedation are not well described. The aim of the current study was to describe prolonged dexmedetomidine therapy without other continuous sedation, specifically the hemodynamic effects, discontinuation strategies, and risk factors for withdrawal.

Cysteinyl leukotriene receptor 1 expression identifies a subset of neutrophils during the antiviral response that contributes to postviral atopic airway disease.

Background: Viral respiratory tract infections increase the risk of development and exacerbation of atopic disease. Previously, we demonstrated the requirement for a neutrophil (PMN) subset expressing CD49d to drive development of postviral atopic airway disease in mice.

Objective: We sought to determine whether human CD49d PMNs are present in the nasal mucosa during acute viral respiratory tract infections and further characterize this PMN subset in human subjects and mice.

Zoledronic acid in pediatric metabolic bone disorders.

Zoledronic acid (ZA), a highly potent intravenous bisphosphonate (BP), has been increasingly used in children with primary and secondary osteoporosis due to its convenience of shorter infusion time and less frequent dosing compared to pamidronate. Many studies have also demonstrated beneficial effects of ZA in other conditions such as hypercalcemia of malignancy, fibrous dysplasia (FD), chemotherapy-related osteonecrosis (ON) and metastatic bone disease. This review summarizes pharmacologic properties, mechanism of action, dosing regimen, and therapeutic outcomes of ZA in a variety of metabolic bone disorders in children.

The effect of body position on esophageal reflexes in cats: a possible mechanism of SIDS?

BackgroundIt has been hypothesized that life-threatening events are caused by supraesophageal reflux (SER) of gastric contents that activates laryngeal chemoreflex-stimulated apnea. Placing infants supine decreases the risk of sudden infant death syndrome (SIDS). The aim of this study was to determine whether body position affects esophageal reflexes that control SER.

Prospective research on infants with mild encephalopathy: the PRIME study.

Objective: To determine short-term outcomes of infants with evidence of hypoxia-ischemia at birth and classified as mild neonatal encephalopathy (NE) at <6 h of age.

Study Design: Prospective multicenter study. Mild NE was defined as ⩾1 abnormal category in modified Sarnat score.

Asfotase alfa treatment for 1 year in a 16 year-old male with severe childhood hypophosphatasia.

We describe the clinical outcome of asfotase alfa therapy in a 16-year-old boy with severe childhood hypophosphatasia (HPP), who began therapy at age 15 years. The patient was diagnosed with HPP at age 2 years when he presented with genu varum and premature loss of primary teeth. He had a history of multiple fractures requiring 16 orthopedic surgeries with rod and pin placement in his lower extremities.

Pulmonary sequelae and functional limitations in children and adults with bronchopulmonary dysplasia.

Preterm infants with bronchopulmonary dysplasia (BPD) often suffer from life-long pulmonary impairments in pulmonary physical function. This review summarizes our current understanding of the chronic pulmonary impairments and physical functional limitations associated with BPD from preterm birth to adulthood. It also identifies opportunities for intervention in children and adults living with chronic lung disease (CLD) after preterm birth.

Mobile Application vs Paper Pictorial Blood Assessment Chart to Track Menses in Young Women: A Randomized Cross-over Design.

Study Objective: Heavy menstrual bleeding is a common symptom reported by approximately 30% of women. The Pictorial Blood Assessment Chart (PBAC) score is often used to quantify severity of menstrual bleeding. However, the traditional PBAC paper diary might be subject to recall bias and compliance issues, especially in adolescents.

Whole exome sequencing frequently detects a monogenic cause in early onset nephrolithiasis and nephrocalcinosis.

The incidence of nephrolithiasis continues to rise. Previously, we showed that a monogenic cause could be detected in 11.4% of individuals with adult-onset nephrolithiasis or nephrocalcinosis and in 16.

Clinically severe CACNA1A alleles affect synaptic function and neurodegeneration differentially.

Dominant mutations in CACNA1A, encoding the α-1A subunit of the neuronal P/Q type voltage-dependent Ca2+ channel, can cause diverse neurological phenotypes. Rare cases of markedly severe early onset developmental delay and congenital ataxia can be due to de novo CACNA1A missense alleles, with variants affecting the S4 transmembrane segments of the channel, some of which are reported to be loss-of-function. Exome sequencing in five individuals with severe early onset ataxia identified one novel variant (p.

Patterns of Emergency Department Care for Newly Diagnosed Immune Thrombocytopenia in United States Children's Hospitals.

We used the Pediatric Health Information Systems database to ascertain treatment patterns of immune thrombocytopenia across the US. Despite the recently published guidelines by the American Society of Hematology, most patients are still being hospitalized for immune thrombocytopenia, even in the absence of documented bleeding symptoms.

Effect of Depth and Duration of Cooling on Death or Disability at Age 18 Months Among Neonates With Hypoxic-Ischemic Encephalopathy: A Randomized Clinical Trial.

Importance: Hypothermia for 72 hours at 33.5°C for neonatal hypoxic-ischemic encephalopathy reduces death or disability, but rates continue to be high.

Objective: To determine if cooling for 120 hours or to a temperature of 32.

Self-management and Transition to Adult Health Care in Adolescents and Young Adults: A Team Process.

As health care continues to evolve, the need for more effective health care transition (HCT) for all youth, but particularly children with chronic conditions and special health care needs, becomes even more important. With more than 90% of adolescents with chronic medical conditions now surviving into adulthood, suboptimal transition can lead to poorer quality of life and less successful adulthood.Through a series of clinical vignettes, the challenges of HCT are presented herein and accompanied by comments that underscore how these adolescents can best be helped to transition to successful adulthood.

The Relationship Between Stressful Life Events and Axis I Diagnoses Among Adolescent Offspring of Probands With Bipolar and Non-Bipolar Psychiatric Disorders and Healthy Controls: The Pittsburgh Bipolar Offspring Study (BIOS).

Background: Previous studies have explored the role of stressful life events in the development of mood disorders. We examined the frequency and nature of stressful life events as measured by the Stressful Life Events Schedule (SLES) among 3 groups of adolescent offspring of probands with bipolar (BD), with non-BD psychiatric disorders, and healthy controls. Furthermore, we examined the relationship between stressful life events and the presence of DSM-IV Axis I disorders in these offspring.

Renal Calcium Oxalate Deposits Induce a Pro-Atherosclerotic and Pro-Osteoporotic Response in Mice.

Urinary stone disease (USD) is increasing in adult and pediatric populations. Adult and pediatric studies have demonstrated decreased bone mineral density and increased fracture rates. USD has also been independently linked to increased rates of myocardial infarction and cerebral vascular accidents.

Challenges in Implementation of the New Accreditation System.

Despite major changes in US Graduate Medical Education, from Core Competencies (2002) to the Next Accreditation System (2012), few studies have evaluated the role of the Residency Coordinator in program accreditation. This role may be especially challenging in child neurology, which involves separate, accredited child and adult neurology residencies. The present study of Child Neurology Program Coordinators evaluated workforce factors and first-year implementation of new training requirements.

Hormonal Contraception and Risk of Thromboembolism in Women With Diabetes.

Objective: To investigate safety of hormonal contraception with regard to thromboembolic events in women with type 1 or 2 diabetes.

Research Design And Methods: We used data from 2002-2011 in Clinformatics Data Mart to identify women in the U.S.

Behavioral and Molecular Genetics of Reading-Related AM and FM Detection Thresholds.

Auditory detection thresholds for certain frequencies of both amplitude modulated (AM) and frequency modulated (FM) dynamic auditory stimuli are associated with reading in typically developing and dyslexic readers. We present the first behavioral and molecular genetic characterization of these two auditory traits. Two extant extended family datasets were given reading tasks and psychoacoustic tasks to determine FM 2 Hz and AM 20 Hz sensitivity thresholds.

Characterization and mechanisms of the pharyngeal swallow activated by stimulation of the esophagus.

Stimulation of the esophagus activates the pharyngeal swallow response (EPSR) in human infants and animals. The aims of this study were to characterize the stimulus and response of the EPSR and to determine the function and mechanisms generating the EPSR. Studies were conducted in 46 decerebrate cats in which pharyngeal, laryngeal, and esophageal motility was monitored using EMG, strain gauges, or manometry.

Preparedness of pediatric residents for fellowship: a survey of US neonatal-perinatal fellowship program directors.

Objective: To evaluate the preparedness of pediatric residents entering accredited neonatal-perinatal medicine (NPM) fellowships in the United States.

Study Design: A multi-domain, validated survey was distributed to Program Directors (PDs) of US NPM fellowship programs. The 47-item survey explored 5 domains: professionalism, independent practice, psychomotor ability, clinical evaluation, and academia.