Objectives:The aim of the study was to evaluate the impact of a residentled patient safety council. This study measured change in resident perceptions and knowledge of safety issues for 3 years, as well as behavioral choices to participate in patient safety activities during and after residency.Methods: Pediatric residents formed a resident-led safety council to engage their peers in patient safety activities. Surveys were distributed annually from 2013 to 2015 to measure residents' perception and knowledge surrounding patient safety. The number of patient safety reports submitted by residents was tracked for the same period. In addition, recent graduates were surveyed to assess the influence of the council on postresidency involvement in patient safety.Results: Resident perception of the institutional culture of safety improved and knowledge of basic patient safety concepts increased. The number of resident-submitted safety reports increased from 6.2 to 15.2 reports per month in the 2013 and 2015 academic years, respectively. Surveys of recent graduates suggest that involvement with the safety council during residency fostered future engagement in patient safety.Conclusions: This resident-led council models successful involvement of trainees in system-based patient safety. Such involvement can help shape the safety culture within a training program and encourages continued participation in patient safety after residency completion.

Aicardi syndrome (AS) is a rare neurodevelopmental disorder, predominantly seen in female individuals, which appears to have an increased risk of both benign and malignant neoplasia. We report the case of a child with AS who presented with metastatic malignant sacrococcygeal tumor (with yolk sac elements) which recurred and then was treated with 3 cycles of high-dose chemotherapy with autologous stem cell rescue. The patient tolerated therapy with acceptable toxicity and remains in clinical remission 3 months after the completion of therapy. Her neurological status remains similar to that before diagnosis with the exception of chemotherapy induced hearing loss. This is the first description a sacrococcygeal teratoma in a patient with Aicardi, as well as the first use of intensified consolidation chemotherapy in a patient with Aicardi, which was well tolerated and resulted in remission. The use of chemotherapy should be considered for all patients with AS and malignancy.

Background: Previous evidence has shown that low mean nocturnal oxyhemoglobin saturation is significantly associated with a higher number of pain days per year Hargrave et al. (Blood. 2003; 101(3)846-8). We tested the primary hypothesis that sleep disordered breathing, described by low mean nocturnal oxygen saturation (SpO2), is associated with increased rates of severe vaso-occlusive pain and acute chest syndrome (ACS). Our secondary hypothesis investigated the association between high obstructive apnea hypopnea index (OAHI; the number of obstructive apneas and hypopneas with >3% SpO2desaturations or arousals per hour of sleep) with increased incidence of severe pain requiring hospitalization and ACS episodes. Study design: A prospective cohort study of children and adolescents from the Sleep and Asthma Cohort (SAC) was assembled. Children with sickle cell anemia (SCA), defined as HgbSS or Hb Sβ°, were enrolled from 4 to 18 years of age at three clinical centers, Washington University School of Medicine in St. Louis, Missouri; Case Western Reserve University in Cleveland, Ohio; and University College London in London, UK (which recruited from three London hospitals). At each site, SAC study-certified technicians performed full 12 channel in-laboratory research polysomnography (PSG) including continuous measurements of nocturnal oxygen saturation using standardized protocols according to American Academy of Sleep Medicine guidelines for data acquisition and scoring. All PSGs were read centrally at Vanderbilt University School of Medicine. Negative binomial regression was used to test the association between nocturnal SpO2 and the incidence rate of severe pain requiring hospitalization and ACS episodes. Covariates in the primary models for pain and ACS included: age at polysomnography, sex, hemoglobin, white blood cell count (WBC), OAHI, and mean nocturnal SpO2. All covariates meeting a significance criteria of p<0.20 were subsequently included in the final model for pain or ACS. Pain rate was defined as number of vaso-occlusive pain events that required hospitalization per year. ACS event was defined as an episode of acute respiratory distress associated with a new radiodensity on chest roentgenogram and at least one of the following: temperature greater than 38° Celsius, increased respiratory effort, decreased oxygen saturation, or increased respiratory rate documented in the medical record. A diagnosis of pneumonia was considered an ACS episode. If a pain event occurred with an ACS episode, the participant was counted as having an ACS event. Results: A total of 252 participants with SCA underwent polysomnography and after quality control, 243 were included; of these 223 had values for pain and ACS after polysomnography; missing data were due to lack of follow-up. Of the 223 participants, 18 on hydroxyurea therapy at the time of PSG and one with missing hydroxyurea status were excluded in primary analyses as hydroxyurea treatment is known to significantly influence pain incidence. The final sample included 204 participants, median age 10.4 years (interquartile range 7.3). Participants were followed for median 5.0 years (interquartile range 1.8 after PSG). After adjusting for age, sex, WBC, and hemoglobin, higher nocturnal SpO2 was associated with increased incidence of pain, which is in the opposite direction of our hypothesis (incidence rate ratio 1.11, 95% CI 1.06-1.17, p<0.001). Mean nocturnal SpO2 was not associated with ACS (incidence rate ratio 1.02, CI 0.96-1.09, p=0.500). In separate models, adjusting for age, sex, WBC, and hemoglobin, OAHI was not associated with future pain using either the log OAHI (IRR 0.99, CI 0.88-1.10, p=0.805) or with OAHI ≥2 (IRR 0.87, CI 0.55-1.41, p=0.569). After adjustment for covariates, OAHI was not associated with ACS using either the log OAHI (IRR 1.06, CI 0.92-1.21, p=0.450) or with OAHI ≥2 (IRR 0.86, CI 0.53-1.39, p=0.541). In an attempt to replicate the Hargrave analysis (Blood. 2003; 101(3)846-8), we investigated whether pain days per year was associated with nocturnal SpO2. Nocturnal SpO2was associated with pain days per year, but in the opposite direction of our hypothesis (IRR 1.12, 95% CI 1.05-1.20, p= 0.001). Conclusion: In an unselected group of children with SCA, low mean nocturnal SpO2 and elevated OAHI are not associated with clinically relevant increased incidence rates of vaso-occlusive pain or ACS. Disclosures No relevant conflicts of interest to declare.