Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.

Interpretation Accuracy and Interrater Agreement of Pediatric Skin and Soft Tissue Point-of-Care Ultrasound Images among Residents and Faculty.

Background: Point-of-care ultrasound (POCUS) is commonly used to facilitate care in the emergency department. Acquired images are often reviewed by local experts for educational and quality assurance purposes. However, no published study has examined the accuracy and reliability of POCUS image interpretation by multiple reviewers.

Changes in child functioning pre-to post-neuropsychological evaluation.

Although a key goal of neuropsychological evaluation is to improve patient outcomes, research regarding changes in child functioning following neuropsychological evaluation is very limited. This project examined changes from before neuropsychological evaluation to several months afterward in parent report of their child's functioning and ability to participate across home, school, and community settings. Fifty-one parents of children and adolescents receiving their first outpatient neuropsychological evaluation completed questionnaires prior to initial parent interview and following the final report (of n = 162 who were mailed the follow-up questionnaire).

Evaluation of an After-Hours Child Passenger Safety Resource Guide.

Motor vehicle crashes are a leading cause of unintentional injury deaths for children in the United States. Child safety seats are effective in reducing the rate and severity of injury for children. Families seen in an emergency department (ED) outside of injury prevention (IP) operational hours may not have the same opportunity to obtain a child safety seat due to the unavailability of IP resources.

Neurologic Injury in Neonates Undergoing Cardiac Surgery.

Neurodevelopmental outcomes after neonatal congenital heart surgery are significantly influenced by brain injury detectable by MRI imaging techniques. This brain injury can occur in the prenatal and postnatal periods even before cardiac surgery. Given the significant incidence of new MRI brain injury after cardiac surgery, much work is yet to be done on strategies to detect, prevent, and treat brain injury in the neonatal period in order to optimize longer-term neurodevelopmental outcomes.

Dexmedetomidine extraction by the extracorporeal membrane oxygenation circuit: results from an in vitro study.

Background: Dexmedetomidine is a sedative administered to minimize distress and decrease the risk of life threatening complications in children supported with extracorporeal membrane oxygenation. The extracorporeal membrane oxygenation circuit can extract drug and decrease drug exposure, placing the patient at risk of therapeutic failure.

Objective: To determine the extraction of dexmedetomidine by the extracorporeal membrane oxygenation circuit.

Adherence to Orthodontic Treatment in Youth With Cleft Lip and/or Palate.

Objective: To identify rates of nonadherence to orthodontic treatment among youth with cleft lip and/or palate (CL/P) and to compare demographic and clinical characteristics of patients categorized as adherent or nonadherent.

Design: Retrospective chart review.

Setting: Orthodontic treatment program affiliated with a US pediatric hospital-based craniofacial team.

Oral Narrow-Spectrum Antibiotics for the Treatment of Urinary Tract Infection in Infants Younger Than 60 Days.

Data on the outcome of infants younger than 60 days treated with a combination of intravenous and oral antibiotics for urinary tract infection are limited. This study examined susceptibility and successful outcomes of treatment with narrow-spectrum oral antibiotics. In addition, we describe the effects of antimicrobial stewardship education intervention on prescribing practices.

Growth and Changing Characteristics of Pediatric Intensive Care 2001-2016.

Objectives: We assessed the growth, distribution, and characteristics of pediatric intensive care in 2016.

Design: Hospitals with PICUs were identified from prior surveys, databases, online searching, and clinician networking. A structured web-based survey was distributed in 2016 and compared with responses in a 2001 survey.

Adherence to Orthodontic Treatment in Youth With Craniofacial Conditions: A Survey of US Orthodontists.

Objective: (1) To explore orthodontists' perceptions of nonadherence and related factors in their patients with craniofacial conditions; (2) to examine differences in adherence perceptions by provider characteristics; (3) to evaluate current adherence interventions.

Design: Cross-sectional.

Setting: United States-based orthodontists affiliated with a nonprofit association for providers treating oral cleft and craniofacial conditions received survey invitations via list-serv, e-mail, and social media.

Guidelines for synoptic reporting of surgery and pathology in Hirschsprung disease.

Background/purpose: Synoptic, or standardized, reporting of surgery and pathology reports has been widely adopted in surgical oncology. Patients with Hirschsprung disease may experience morbidity related to surgical factors or underlying pathology and often undergo multiple operations. Our aim is to improve the postoperative outcome and care of patients with Hirschsprung disease by proposing a standardized set of data that should be included in every surgery and pathology report.

Dexamethasone Associated With Significantly Shorter Length of Hospital Stay Compared With a Prednisolone-Based Regimen in Pediatric Patients With Mild to Moderate Acute Asthma Exacerbations.

A retrospective chart review was done to evaluate the efficacy of a course of dexamethasone for pediatric patients hospitalized with a mild to moderate acute asthma exacerbation compared with a prednisone-based regimen. Patients were identified based on International Classification of Diseases (ICD-9 and ICD-10) discharge diagnosis codes for asthma and cross-referenced with pharmacy dispense reports during the study period of June 2011 to January 2016. Baseline characteristics were similar among the 2 groups.

Pediatric Diffuse Large B-Cell Lymphoma of the Frontal Sinus: A Case Report.

An 11-year-old male presented with right proptosis, bulbar conjunctivitis, and diplopia. Computerized tomography (CT) and magnetic resonance imaging revealed an enhancing mass involving the superio-medial orbit, ethmoids, frontal sinus, and anterior cranial fossa with skull base destruction. Diffuse large B-cell lymphoma was diagnosed via CT-guided biopsy.

Comparing Single-Photon Emission Computed Tomography (SPECT), Electroencephalography (EEG), and Magneto-encephalography (MEG) Seizure Localizations in Pediatric Cases of Laser Ablation.

Intractable epilepsy may have a more severe effect on children in comparison to adults because the motor, sensorial, and cognitive functions of children are still undergoing development. For this same reason, however, children maintain a greater potential for recovery from intractable epilepsy because of the remaining cerebral plasticity. Thus, after 2 unsuccessful antiepileptic medications, surgical intervention is recommended.

Metabolic syndrome is a sequela of radiation exposure in hypothalamic obesity among survivors of childhood brain tumors.

Survivors of childhood brain tumors may be at risk for early onset of metabolic syndrome, possibly secondary to surgery and/or radiation exposure. This study examines effects of radiation exposure to hypothalamus-pituitary-adrenal axis (HPA) on metabolic risk among survivors of childhood brain tumors. One hundred forty-two met inclusion criteria; 60 had tumor surgery plus radiation exposure (1 Gray (Gy)) to HPA.

Non-Radiographic Severity Measurement of Pectus Excavatum.

Background: To avoid the radiation exposure of CT imaging and the expense of CT or MRI studies, we sought to develop a non-radiographic severity measurement of pectus excavatum based on 3D photogrammetric imaging.

Methods: Over 28 mo, ten consecutive patient volunteers with pectus excavatum underwent 3D stereophotogrammetric imaging. The surface width to surface depth ratio (Surface Lengths Pectus Index), the chest deformity's surface area to total chest surface area (Pectus Surface Area Ratio), and the chest deformity's volume to total chest volume (Pectus Volume Ratio) were calculated.

Consensus Statement on Urgent Care Centers and Retail Clinics in Acute Care of Children.

This article provides recommendations for pediatric readiness, scope of services, competencies, staffing, emergency preparedness, and transfer of care coordination for urgent care centers (UCCs) and retail clinics that provide pediatric care. It also provides general recommendations for the use of telemedicine in these establishments. With continuing increases in wait times and overcrowding in the nation's emergency departments and the mounting challenges in obtaining timely access to primary care providers, a new trend is gaining momentum for the treatment of minor illness and injuries in the form of UCCs and retail clinics.

Randomized Clinical Trial Comparing Breath-Enhanced to Conventional Nebulizers in the Treatment of Children with Acute Asthma.

Objective: To compare the efficacy of a breath-enhanced and a conventional jet nebulizer in the treatment of children with moderate to severe acute asthma.

Study Design: We enrolled subjects between 6 and 18 years of age presenting to the emergency department (ED) with acute asthma and an initial forced expiratory volume in 1 second (FEV) <70% of predicted. We excluded patients with chronic disease, who required immediate resuscitation, or failed spirometry.

The use of point of care ultrasound in the evaluation of pediatric soft tissue neck masses.

Objective: Most soft tissue neck masses represent benign inflammatory or infectious processes; however, in some cases the diagnosis is not clear and a broader differential must be considered. The aim of this study was to compare point-of-care ultrasound (POCUS) to radiology department imaging (RDI) in the diagnosis of soft tissue neck masses.

Methods: This prospective pilot study involved a convenience sample of patients ranging in age from 1 month to 18 years of age presenting to the Pediatric Emergency Department (PED) with a soft tissue neck mass.