Safety of pediatric sedation and analgesia during burn dressing changes by pediatric residents.

BACKGROUND: Acute pain and anxiety management during pediatric burn dressing change is very challenging. There are limited data regarding feasibility and safety of sedation administration by nonanesthesiologists. We sought to describe the implementation of a sedation protocol for pediatric residents during burn dressing changes in the pediatric ward. METHODS: An analgesia and sedation protocol was designed and implemented in the pediatric wards in 2015. Retrospective data were collected on all children who were sedated by pediatric residents for burns in the pediatric wards over a 4-year period in our hospital. Demographics, burn characteristics, and data regarding adverse events were collected from patients' electronic medical records. The main outcomes were successful procedure completion and safety. RESULTS: During the study period, 1130 sedations were performed in 272 patients by pediatric residents. The median age was 2.5 years (IQR 1.3-9.1). Sixty-two percent (695/1130) of the patients were male. The majority of the burns (84%, 955/1130) were <20%, and the majority of the patients (91%, 1030/1130) had an ASA (American Society of Anesthesiologists) score of 1. The incidence rate of adverse events was 4.3% (49/1130) and 1.3% (15/1130) for serious adverse events . There were no statistically significant differences in gender, age, ASA, or burn degree between patients with or without adverse events. CONCLUSIONS: We designed and implemented an analgesia and sedation protocol for pediatric residents to be applied in patients during burn dressing change. The implemented protocol in the pediatric wards was found to be feasible and with a low incidence of adverse events.

Whole exome sequencing in childhood-onset lupus frequently detects single gene etiologies.

BACKGROUND: Systemic lupus erythematosus (SLE) comprise a diverse range of clinical manifestations. To date, more than 30 single gene causes of lupus/lupus like syndromes in humans have been identified. In the clinical setting, identifying the underlying molecular diagnosis is challenging due to phenotypic and genetic heterogeneity. METHODS: We employed whole exome sequencing (WES) in patients presenting with childhood-onset lupus with severe and/or atypical presentations to identify cases that are explained by a single-gene (monogenic) cause. RESULTS: From January 2015 to June 2018 15 new cases of childhood-onset SLE were diagnosed in Edmond and Lily Safra Children's Hospital. By WES we identified causative mutations in four subjects in five different genes: C1QC, SLC7A7, MAN2B1, PTEN and STAT1. No molecular diagnoses were established on clinical grounds prior to genetic testing. CONCLUSIONS: We identified a significant fraction of monogenic SLE etiologies using WES and confirm the genetic locus heterogeneity in childhood-onset lupus. These results highlight the importance of establishing a genetic diagnosis for children with severe or atypical lupus by providing accurate and early etiology-based diagnoses and improving subsequent clinical management.

The effect of chart speed on fetal monitor interpretation.

OBJECTIVE: Electronic fetal heart monitor chart speeds vary between countries, and it is unclear whether differing chart speeds affect physician tracing interpretation. METHODS: Twenty-minute segments of 19 tracings were displayed on both 1 and 3 cm/min strips and interpreted by 14 physicians at the particular speed they were accustomed to reading. Interpretations of tracing characteristics were compared between groups using free margin kappa, a measure of interobserver agreement. RESULTS: Compared to 3 cm/min tracings, 1 cm/min tracings were significantly more often identified as having absent than minimal variability, and minimal than moderate variability. Accelerations were significantly more often identified in 1 versus 3 cm/min strips. There were no significant differences between groups with respect to baseline fetal heart rate, prolonged or repetitive decelerations, or American College of Obstetricians and Gynecologists tracing category. Neither chart speed had substantial interobserver agreement in tracing variables; however, agreement was consistently higher in 3 versus 1 cm/min tracings (all p < 0.05). CONCLUSIONS: Tracing interpretation is significantly affected by fetal monitor chart speed with regards to variability, acceleration and deceleration. Further studies are required to determine if differences in chart speed interpretation affect clinical management.