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Williams syndrome is a complex syndrome characterized by developmental abnormalities, craniofacial dysmorphic features, and cardiac anomalies. Sudden death has been described as a very common complication associated with anesthesia, surgery, and procedures in this population. Anatomical abnormalities associated with the heart pre-dispose these individuals to sudden death. In addition to a sudden and rapid downhill course, lack of response to resuscitation is another significant feature seen in these patients. The authors report a five-year-old male with Williams syndrome, hypothyroidism, and attention deficit hyperactivity disorder. He suffered an anaphylactic reaction during CT imaging with contrast. Resuscitation was unsuccessful. Previous reports regarding the anesthetic management of patients with Williams are reviewed and the potential for sudden death or peri-procedure related cardiac arrest discussed in this report. The authors also review reasons for refractoriness to defined resuscitation guidelines in this patient population.

A sepsis scenario guided participants through active resuscitation of a sick child using hidden clues/puzzles that unlocked treatments enabling progression through the scenario. Participants were assessed on enjoyment of the task, frequency of use of CRM principles, and adherence to a protocolized based sepsis resuscitation algorithm. [Extracted from the article]

This JAMA Patient Page describes the causes, symptoms, diagnosis, and treatment of periorbital and orbital cellulitis. [ABSTRACT FROM AUTHOR]

The authors discuss the importance of a cohort study which investigated the link between the implementation of a mandated sepsis bundle and mortality among children in New York State in 2013. They cite the challenge of identifying pediatric sepsis early which the cohort study was able to address by providing needed data to develop protocols for improved sepsis outcomes. They also warn against merely adopting a bundle of care without a culture of safety and quality.

This JAMA Patient Page describes the symptoms, diagnosis, and treatment of epiglottitis in children. [ABSTRACT FROM AUTHOR]

This JAMA Patient Page describes the symptoms, diagnosis, and treatment of croup in children. [ABSTRACT FROM AUTHOR]

This JAMA Patient Page describes skin abscess—its symptoms, treatment, and warning signs. [ABSTRACT FROM AUTHOR]

The article describes the case of a six-day-old male patient who was admitted to a medical facility due to diarrhea, vomiting as well as abdominal distention. Medical professionals obtained samples of blood, urine and cerebrospinal fluid from the patient to rule out potential development of bacteremia, urinary-tract infection and meningitis. The case focuses on the need for medical experts to first consider life-threatening conditions in newborn patients.

Study Objective: To determine whether the receipt of more than or equal to 30 mL/kg of intravenous fluid in the first hour after emergency department (ED) arrival is associated with sepsis-attributable mortality among children with hypotensive septic shock., Methods: This is a retrospective cohort study set in 57 EDs in the Improving Pediatric Sepsis Outcomes quality improvement collaborative. Patients less than 18 years of age with hypotensive septic shock who received their first intravenous fluid bolus within 1 hour of arrival at the ED were propensity-score matched for probability of receiving more than or equal to 30 mL/kg in the first hour. Sepsis-attributable mortality was compared. We secondarily evaluated the association between the first-hour fluid volume and sepsis-attributable mortality in all children with suspected sepsis in the first hour after arrival at the ED, regardless of blood pressure., Results: Of the 1,982 subjects who had hypotensive septic shock and received a first fluid bolus within 1 hour of arrival at the ED, 1,204 subjects were propensity matched. In the matched patients receiving more than or equal to 30 mL/kg of fluid, 26 (4.3%) of 602 subjects had 30-day sepsis-attributable mortality compared with 25 (4.2%) of 602 receiving less than 30 mL/kg (odds ratio 1.04, 95% confidence interval 0.59 to 1.83). Among the patients with suspected sepsis regardless of blood pressure, 30-day sepsis-attributable mortality was 3.0% in those receiving more than or equal to 30 mL/kg versus 2.0% in those receiving less than 30 ml/kg (odds ratio 1.52, 95% confidence interval 0.95 to 2.44.) CONCLUSION: In children with hypotensive septic shock receiving a timely first fluid bolus within the first hour of ED care, receiving more than or equal to 30 mL/kg of bolus intravenous fluids in the first hour after arrival at the ED was not associated with mortality compared with receiving less than 30 mL/kg., (Copyright © 2022 American College of Emergency Physicians. Published by Elsevier Inc. All rights reserved.)

Purpose: Pediatric sepsis guidelines recommend rapid intravenous fluid (IVF) bolus administration rates (BAR). Recent sepsis studies suggest that rapid BAR may be associated with increased morbidity. We aimed to describe the association between emergency department (ED) IVF BAR and clinical outcomes in pediatric sepsis., Patients and Methods: Secondary post-hoc analysis of retrospective cohort data from 19 hospitals in the Pediatric Septic Shock Collaborative (PSSC) database. Patients with presumed septic shock were defined by severe sepsis/septic shock diagnostic codes, receipt of septic shock therapies, or floor-to-ICU transfers within 12 hours from ED admission for septic shock. Patients (2 months-21 years) with complete data on weight, antibiotic receipt, bolus timing, and bolus volumes were included. The primary outcome was 30-day mortality. Associations between BAR and mortality and secondary (intubation or non-invasive positive pressure ventilation = NIPPV) outcomes were assessed using unadjusted and adjusted logistic regression., Results: The PSSC database included 6731 patients; 3969 met inclusion and received a median ED volume of 40.2 mL/kg. Seventy-six (1.9%) patients died, 151 (3.8%) were intubated, and 235 (5.9%) had NIPPV administered. The median BAR was 25.7 mL/kg/hr. For each 20 mL/kg/hr increase in BAR, the adjusted odds ratio (aOR) for 30-day mortality [aOR = 1.11 (95% CI 1.01, 1.23)], intubation [aOR = 1.25 (95% CI 1.09, 1.44)], and NIPPV [aOR = 1.20 (95% CI 1.05, 1.38)] significantly increased., Conclusion: Faster ED IVF bolus administration rates in this pediatric sepsis database were associated with higher adjusted odds of death, intubation and NIPPV. Controlled trials are needed to determine if these associations are replicable., Competing Interests: The authors report no conflicts of interest in this work., (© 2022 Mullan et al.)

Background: Angiopoietin-1 and -2 are vascular growth factors that exert opposing effects on endothelial activation and dysfunction. The aim of this study was to assess the association of these biomarkers with outcomes in children with sepsis. Methods: Biomarkers were assayed from the blood collected in an emergency department prior to any intervention. Predictor variables were Ang-1 and Ang-2 levels and the Ang-2/Ang-1 ratio. Outcomes included mortality, length of time on vasopressors, and ICU and hospital lengths of stay. The Pediatric RISk of Mortality III Score was calculated. A vasoactive inotrope score was calculated every 12 hours. Results: Forty-five children with sepsis and 49 with septic shock were analyzed. The median Ang-2 was higher in septic shock. The Ang-2/Ang-1 ratio was approximately 2-fold greater in those with septic shock. The Ang-2/Ang-1 ratio was associated with higher doses of vasoactive agents at 12 hours and longer ICU length of stay. In septic shock, for every 0.35 unit increase in the Ang-2/Ang-1 ratio, the PRISM III score increased by 1. Conclusions: The Ang-2/Ang-1 ratio was higher in children with septic shock. Ang-2/Ang-1 was associated with higher vasoactive agents, longer ICU length of stay, and correlated with the severity of illness score., Competing Interests: Competing Interests: Dr. Liles is listed as an inventor on US patent, ''Biomarkers for Early Determination of a Critical or Life-Threatening Response to Illness and/or Treatment Response.” The remaining authors have no financial relationships relevant to this article to disclose.

Background: Sepsis is a leading cause of morbidity and mortality in children worldwide. Barriers exist for timely recognition and management in emergency care settings. This 1-year quality improvement collaborative sought to reduce mortality from sepsis., Methods: Fifteen hospitals participated initially. We included children with a spectrum of illness from sepsis to septic shock. The intervention bundle focused on recognition, escalation of care, and the first hour of resuscitation. We conducted monthly learning sessions and disseminated data reports of site-specific and aggregated metrics to drive rapid cycle improvement., Results: Seven sites contributed enough data to be analyzed. Of the 1,173 pediatric patients in the total cohort, 506 presented with severe sepsis/septic shock. Quarterly data demonstrated a mean improvement in initial clinical assessment from 46% to 60% ( P < 0.001) and in adherence to the administration of first fluid bolus within 15 minutes from 38% to 46% ( P < 0.015). There was no statistically significant improvement in other process metrics. There was no statistically significant improvement in mortality for the total cohort (sepsis to septic shock) or either of the subgroups in either 3- or 30-day mortality., Conclusions: A quality improvement collaborative focused on improving timely recognition and management of pediatric sepsis to septic shock led to some process improvements but did not show improvement in mortality. Future national efforts should standardize definitions and processes of care for sepsis to septic shock, including the identification of a "time zero" for measuring the timeliness of treatment.

Background and Objectives: Few studies have demonstrated improvement in adherence to Pediatric Advanced Life Support guidelines for severe sepsis and septic shock. We sought to improve adherence to national guidelines for children with septic shock in a pediatric emergency department with poor guideline adherence., Methods: Prospective cohort study of children presenting to a tertiary care pediatric emergency department with septic shock. Quality improvement (QI) interventions, including repeated plan-do-study-act cycles, were used to improve adherence to a 5-component sepsis bundle, including timely (1) recognition of septic shock, (2) vascular access, (3) administration of intravenous (IV) fluid, (4) antibiotics, and (5) vasoactive agents. The intervention focused on IV fluid delivery as a key driver impacting bundle adherence, and adherence was measured using statistical process control methodology., Results: Two-hundred forty-two patients were included: 126 subjects before the intervention (November 2009 to March 2011), and 116 patients during the QI intervention (October 2011 to May 2013). We achieved 100% adherence for all metrics, including (1) administration of 60 mL/kg IV fluid within 60 minutes (increased from baseline adherence rate of 37%), (2) administration of vasoactive agents within 60 minutes (baseline rate of 35%), and (3) 5-component bundle adherence (baseline rate of 19%). Improvement was sustained over 9 months. The number of septic shock cases between each death from this condition increased after implementation of the QI intervention., Conclusions: Using QI methodology, we have demonstrated improved adherence to national guidelines for severe sepsis and septic shock., (Copyright © 2014 by the American Academy of Pediatrics.)

Background and Objectives: Few studies have evaluated sepsis guideline adherence in a tertiary pediatric emergency department setting. We sought to evaluate (1) adherence to 2006 Pediatric Advanced Life Support guidelines for severe sepsis and septic shock (SS), (2) barriers to adherence, and (3) hospital length of stay (LOS) contingent on guideline adherence., Methods: Prospective cohort study of children presenting to a large urban academic pediatric emergency department with SS. Adherence to 5 algorithmic time-specific goals was reviewed: early recognition of SS, obtaining vascular access, administering intravenous fluids, delivery of vasopressors for fluid refractory shock, and antibiotic administration. Adherence to each time-defined goal and adherence to all 5 components as a bundle were reviewed. A detailed electronic medical record analysis evaluated adherence barriers. The association between guideline adherence and hospital LOS was evaluated by using multivariate negative binomial regression., Results: A total of 126 patients had severe sepsis (14%) or septic shock (86%). The median age was 9 years (interquartile range, 3-16). There was a 37% and 35% adherence rate to fluid and inotrope guidelines, respectively. Nineteen percent adhered to the 5-component bundle. Patients who received 60 mL/kg of intravenous fluids within 60 minutes had a 57% shorter hospital LOS (P = .039) than children who did not. Complete bundle adherence resulted in a 57% shorter hospital LOS (P = .009)., Conclusions: Overall adherence to Pediatric Advanced Life Support sepsis guidelines was low; however, when patients were managed within the guideline's recommendations, patients had significantly shorter duration of hospitalization.

Purpose of Review: Sepsis requires prompt recognition and aggressive therapy; early goal-directed therapy decreases morbidity and mortality. Recommendations on the specific management of pediatric sepsis have historically been extrapolated from adult literature and from expert/consensus opinion. This review serves to appraise recent recommendations and determine the applicability of newly promoted adult guidelines for pediatric sepsis., Recent Findings: The first and most recent recommendations on the management of pediatric sepsis were published in 2002 by expert consensus with the recognition that there were few data from randomized controlled trials or meta-analysis. Previous to this, adult guidelines were used in the management of pediatric sepsis. New definitions for pediatric sepsis provided by expert consensus take into account the specific physiologic changes. Prompt correction of hemodynamic derangements has been shown to improve outcome through aggressive volume resuscitation and early initiation of vasopressor therapy. Frequent reassessment has been emphasized to ensure appropriate management., Summary: Prompt recognition, institution of appropriate therapy, and continual assessment for children with sepsis and shock are essential for improved outcomes. The emerging new literature on early goal-directed therapy is most applicable to care in the emergency department.

Objective: To identify the clinical utility of obtaining blood, urine and cerebrospinal fluid for bacterial culture among febrile infants <90 days of age with clinical bronchiolitis., Design: Retrospective chart review from 1995 to 2000., Setting: Urban emergency department of a tertiary children's hospital., Participants: All infants <90 days of age presenting with fever and clinical bronchiolitis., Main Outcome Measures: Result of the cultures of blood, urine and cerebrospinal fluid., Results: Of 3051 (11%) febrile infants, 329 met criteria for clinical bronchiolitis. Blood for culture was obtained from 309 (94%), urine for culture was obtained from 273 (83%) and cerebrospinal fluid for culture was obtained from 200 (61%). One hundred eighty-seven (57%) infants had all 3 specimens sent for culture. No cases of bacteremia [0%; 95% confidence interval (CI), 0, 1.1%] or meningitis (0%; 95% CI 0, 1.8%) occurred among these infants. However, 6 infants (2%; 95% CI 0.8, 5.7%), all male, had a culture of urine consistent with infection (4 Escherichia coli, 1 Staphylococcus aureus, 1 viridans streptococci)., Conclusion: The risk of bacteremia or meningitis among infants <90 days with fever and bronchiolitis is low in this age group. The risk of urinary tract infection in this age group is also low, but not negligible, at 2%.