Racial and Ethnic Composition of Populations Served by Freestanding Children's Hospitals and Disparities in Outcomes of Pediatric Lupus.

OBJECTIVE: Health disparities may be driven by hospital-level factors. We assessed whether racial and ethnic composition of populations hospitals serve explain or modify disparities in hospital outcomes of children with systemic lupus erythematosus (SLE). METHODS: In this retrospective cohort study of patients 5 to 26 years old with SLE at 47 children's hospitals in the Pediatric Health Information System (2006-2021), race and ethnicity were assessed at the patient level and hospital level (proportion of total admissions composed of Black or Hispanic patients, respectively). Outcomes included intensive care unit (ICU) admission or adverse renal outcome (end-stage renal disease, dialysis, or transplant) during follow-up. We estimated racial and ethnic disparities, adjusted or stratified by hospital racial or ethnic composition. RESULTS: Of 8,125 patients with SLE, 2,293 (28%) required ICU admission, and 698 (9%) had an adverse renal outcome. Black and non-Hispanic White disparities in ICU admission were observed only at hospitals serving higher proportions of Black patients (odds ratio [OR] 1.29, 95% confidence interval [95% CI] 1.04-1.59 vs OR 1.07, 95% CI 0.83-1.38). Larger Black and non-Hispanic White disparities in adverse renal outcomes were observed at hospitals with higher Black racial composition (OR 2.0, 95% CI 1.4-2.8 vs OR 1.7, 95% CI 1.1-2.4). Conversely, Hispanic versus non-Hispanic disparities in renal outcomes persisted after adjustment for hospital-reported Hispanic ethnic composition but were observed only at hospitals with lower proportions of Hispanic patients. CONCLUSION: Worse Black and White disparities in SLE outcomes are observed at children's hospitals serving more Black children, whereas distinct patterns are observed for Hispanic and non-Hispanic disparities. Reporting of hospital characteristics related to populations served is needed to identify modifiable drivers of hospital-level variation.

Updated Case Definition of MIS-C and Implications for Clinical Care.

OBJECTIVES: A broad, surveillance case definition was implemented when multisystem inflammatory syndrome in children (MIS-C) emerged in 2020. In 2023, a revised MIS-C case definition was constructed to improve specificity and reduce misclassification with other pediatric inflammatory conditions. This study aims to describe the impact of the updated definition on the classification of patients with MIS-C and Kawasaki Disease (KD). METHODS: Patients hospitalized from March 2020 to November 2022 with clinician-diagnosed KD and MIS-C at a single center were studied retrospectively. Specificity and positive predictive value were assessed; McNemar test was used to compare specificity. RESULTS: Among 119 patients with MIS-C per the 2020 definition, 20 (17%) did not fulfill the 2023 definition. Six of these 20 (30%) had shock or cardiac involvement. Of 59 KD patients, 10 (17%) met the 2020 MIS-C definition. Five patients (8%) met the 2023 MIS-C definition. Specificity for the 2020 and 2023 MIS-C definitions among KD patients were 83.1% and 91.5% respectively (McNemar, P = .0736). Positive predictive value was higher for the 2023 MIS-C case definition compared with the 2020 MIS-C case definition (95.2% vs 92.2%). CONCLUSIONS: Approximately 1 in 5 patients diagnosed with MIS-C using the 2020 case definition did not meet the 2023 definition, including patients with cardiovascular dysfunction. Overlap persisted between patients meeting KD and 2023 MIS-C case definitions, with a false positive rate of 8%. Implications for treatment should be considered, particularly in settings where presumed MIS-C may be treated with corticosteroid monotherapy.

An examination of characteristics, social supports, caregiver resilience and hospital readmissions of children with medical complexity.

BACKGROUND: Children with medical complexity (CMC) account for 1% of children in the United States. These children experience frequent hospital readmissions, high healthcare costs and poor health outcomes. A link between CMC caregiver social support, resilience and hospital readmissions has never been fully investigated. This study examines the feasibility of a prospective, descriptive, repeated measures research design to characterize CMC and their caregivers, social supports, caregiver resilience and hospital readmissions to inform a larger prospective investigation. METHODS: Caregivers of CMC with unplanned hospitalizations completed surveys at the index hospitalization and 30 and 60 days after discharge. CMC caregiver and child characteristics, social supports and hospital readmissions were examined using an investigator-developed survey. Resilience was measured using the Resilience Scale-14© (7-Point Likert Scale, score range 14-98), and feasibility was measured by calculating enrolment, attrition, survey completion and item response. Analysis included descriptive statistics and qualitative data visualization. RESULTS: Of caregivers who were approached for participation, 81.1% consented  and completed 76 surveys. Attrition was 31%. Item response rates were ≥ 90% for all but one item. A total of 62.1% of children had hospital readmissions within 90 days and 37.9% within 30 days. Additionally, 70% of caregivers had home care nursing, but the approved hours were only partially filled. More than 70% of caregiver resilience scores were moderate to high (score range 74-98) and were stable across repeated measures and hospital readmissions. Open-ended question responses revealed the following five categories: All-consuming, Family Reliance, Impact of Covid, Taking Action and Broken System. CONCLUSIONS: Studying CMC caregiver social supports and resilience using repeated measures is feasible. CMC caregivers reported stressors including coordinating their child's substantial healthcare needs and managing partially filled home care nursing hours. Caregiver resilience remained stable over time, amidst frequent CMC hospital readmissions. Findings can inform future research priorities and power analyses for CMC caregiver resilience.

Assessment of Physical Tests in 6-11 Years Old Children: Findings from the Play Lifestyle and Activity in Youth (PLAY) Study.

The purpose was to evaluate selected physical tests in children and to compare the outcomes by sex. A cross-sectional study design was used to evaluate children 6-11 years who completed five physical tests: hand grip, vertical jump, sit and reach, Y-balance, and obstacle course (time and score). The outcome measures including test results were descriptively examined and compared by sex. The study participants consisted of 133 children (62 males and 71 females, with a median age of 7.8 years). Girls showed superior sit and reach performance (p = 0.002) compared with boys. Boys demonstrated better Y-balance scores (p = 0.007) and faster obstacle time (p = 0.042) than girls. Sex comparison within three age groups (6-<8 years, 8-<10 years, and 10-<12 years) showed that girls performed better on the sit and reach compared with boys in the in 6-<8 years (p = 0.009). Boys demonstrated higher Y-balance scores (p = 0.017) and faster obstacle time (p = 0.007) compared with girls in the 8-<10-year age group. These data will serve to guide future efforts to evaluate normative measures of physical literacy and guide targeted training interventions to promote sustained physical activity in children with deficits relative to their age and sex norms.

Equity factors in pediatric transplant listing: Initial findings from a single center review.

BACKGROUND: In order to improve transparency within the patient selection process, a transplant listing advisory committee was formed within the Boston Children's Hospital Pediatric Transplant Center. Its mission is to promote equity in access to organ transplantation by ensuring that the institutional transplant selection criteria are fair, unbiased, and nondiscriminatory. The committee conducts comprehensive case and data review of individual characteristics and reviews in aggregate to identify potential systems bias. METHODS: Charts for 256 patients evaluated for transplant from 3/2016 to 3/2019 were reviewed. Among these, 64 (25%) patients were declined for transplant. Univariate logistic regression analysis was used to identify demographic variables and vulnerable status factors associated with being declined. Odds ratios (OR) are reported. RESULTS: Among all patients, median age was 8.5 years and 58% were male. Asian patients were more likely to be declined than White patients (OR = 5.3, Wald p = .007). Socioeconomic factors that affected likelihood of listing decline included concerns for caregivers' ability to manage and understand care requirements (OR = 3.8, p = .011), caregiver employment status (OR = 1.9, p = .042), and use of public assistance programs (OR = 2.2, p = .05). Patients with severe neurodevelopmental delay were more likely to be declined for listing (OR = 3.7, p = .019). CONCLUSION: This analysis identified areas of potential bias related to race, socioeconomic status, and neurodevelopmental delay where initiatives can be targeted. Advisory committees are an important aspect of evaluating equity in transplant center selection policy and practice.

Burden Experienced by Primary Caregivers of Children With Psychotic Disorders and at Clinical High Risk for Psychosis.

BACKGROUND: Despite the existing research exploring caregiver burden in adult psychosis, few studies have examined the experience of providing care to children diagnosed with psychotic disorders (PDs) and those identified as having clinical high risk for psychosis (CHR-P). OBJECTIVE: This study measured the level of burden in caregivers of children with PD and CHR-P and examined associated risk factors, including social support, caregiver-child relationship, severity of illness, and frequency of psychiatric hospitalizations. METHODS: A total of 56 caregivers completed validated measures and provided demographic information. Measures included the Zarit Burden Interview, the Multidimensional Scale of Perceived Social Support, the Behavior Assessment System for Children, Third Edition, Parenting Relationship Questionnaire-Child and Adolescent Form (BASC-3 PRQ-CA), and the Clinical Global Impression-Severity scale. RESULTS: The majority of caregivers were women (86%), mothers (84%), White (63%), married (66%), working full-time (50%), college-educated (79%), and whose mean age was 45.7 years (SD = 8.09). Nearly half of the caregivers (45%) reported a high level of caregiver burden, 39% rated their burden in the mild to moderate range, and 16% reported little to no burden. There was no significant difference in mean burden between PD and CHR-P groups. Higher caregiver burden was associated with lower levels of social support (r = -.408, p = .002), lower levels of parenting confidence (r = -.514, p < .001), higher levels of relational frustration (r = .612, p < .001), and higher severity of illness (r = .316 p = .025). CONCLUSIONS: These findings underscore the critical unmet need for support for caregivers of children with PD and CHR-P. Applications to clinical practice are discussed.

Postdischarge Glucocorticoid Use and Clinical Outcomes of Multisystem Inflammatory Syndrome in Children.

Importance: Minimal data are available regarding the postdischarge treatment of multisystem inflammatory syndrome in children (MIS-C). Objectives: To evaluate clinical characteristics associated with duration of postdischarge glucocorticoid use and assess postdischarge clinical course, laboratory test result trajectories, and adverse events in a multicenter cohort with MIS-C. Design, Setting, and Participants: This retrospective cohort study included patients with MIS-C hospitalized with severe illness and followed up for 3 months in an ambulatory setting. Patients younger than 21 years who were admitted between May 15, 2020, and May 31, 2021, at 13 US hospitals were included. Inclusion criteria were inpatient treatment comprising intravenous immunoglobulin, diagnosis of cardiovascular dysfunction (vasopressor requirement or left ventricular ejection fraction ≤55%), and availability of complete outpatient data for 3 months. Exposures: Glucocorticoid treatment. Main Outcomes and Measures: Main outcomes were patient characteristics associated with postdischarge glucocorticoid treatment, laboratory test result trajectories, and adverse events. Multivariable regression was used to evaluate factors associated with postdischarge weight gain (≥2 kg in 3 months) and hyperglycemia during illness. Results: Among 186 patients, the median age was 10.4 years (IQR, 6.7-14.2 years); most were male (107 [57.5%]), Black non-Hispanic (60 [32.3%]), and Hispanic or Latino (59 [31.7%]). Most children were critically ill (intensive care unit admission, 163 [87.6%]; vasopressor receipt, 134 [72.0%]) and received inpatient glucocorticoid treatment (178 [95.7%]). Most were discharged with continued glucocorticoid treatment (173 [93.0%]); median discharge dose was 42 mg/d (IQR, 30-60 mg/d) or 1.1 mg/kg/d (IQR, 0.7-1.7 mg/kg/d). Inpatient severity of illness was not associated with duration of postdischarge glucocorticoid treatment. Outpatient treatment duration varied (median, 23 days; IQR, 15-32 days). Time to normalization of C-reactive protein and ferritin levels was similar for glucocorticoid duration of less than 3 weeks vs 3 or more weeks. Readmission occurred in 7 patients (3.8%); none was for cardiovascular dysfunction. Hyperglycemia developed in 14 patients (8.1%). Seventy-five patients (43%) gained 2 kg or more after discharge (median 4.1 kg; IQR, 3.0-6.0 kg). Inpatient high-dose intravenous and oral glucocorticoid therapy was associated with postdischarge weight gain (adjusted odds ratio, 6.91; 95% CI, 1.92-24.91). Conclusions and Relevance: In this multicenter cohort of patients with MIS-C and cardiovascular dysfunction, postdischarge glucocorticoid treatment was often prolonged, but clinical outcomes were similar in patients prescribed shorter courses. Outpatient weight gain was common. Readmission was infrequent, with none for cardiovascular dysfunction. These findings suggest that strategies are needed to optimize postdischarge glucocorticoid courses for patients with MIS-C.

Association of Race and Ethnicity With Medication Use for Pediatric Lupus in the Childhood Arthritis and Rheumatology Research Alliance Registry.

OBJECTIVE: Black and Hispanic children with pediatric lupus (pSLE) have higher morbidity and mortality than non-Hispanic White children. The extent to which differences in outcomes are due to treatment disparities, including medication use, is unknown. We aimed to determine whether medication use in pSLE is associated with race and ethnicity in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. METHODS: Patients with pSLE enrolled in the CARRA Registry from 2017 to 2020 were included. Multivariable mixed-effect logistic regression, adjusted for site of care, was used to compare use of antimalarials, high-dose oral glucocorticoids, and rituximab in Black and Hispanic children. RESULTS: We identified 639 children with pSLE, of whom 480 had at least 1 year of follow-up. At enrollment, 89% of patients were prescribed an antimalarial and 50% were on high-dose glucocorticoids. Of those with 1 year of follow-up, 12% received rituximab. Nephritis, shorter disease duration, and higher Systemic Lupus Erythematosus Disease Activity Index 2000 scores were associated with high-dose glucocorticoid use. Antimalarial use was higher among those with nephritis and lower in children with no insurance. Rituximab use was associated with Black race in the fixed-effects model but not when adjusted for site of care. CONCLUSION: We identified differences in medication use by race and insurance status. Site of care was associated with the racial differences observed in rituximab use. Further research is needed to optimize pSLE treatments particularly where use is highly variable, including glucocorticoid dosing and use of rituximab, and understand the impact of practice variation on disparities in pSLE outcomes.

Eating disorders in adolescents and young adults with cystic fibrosis.

INTRODUCTION: There is evidence for increased risk of eating disorders in individuals with diet-treated chronic illnesses, however, data in patients with cystic fibrosis (CF) is less clear. No studies have evaluated avoidant/restrictive food intake disorder (ARFID) in the CF population. We investigated the prevalence of eating disorders, including ARFID, in adolescents and young adults with CF. METHODS: Patients with CF aged 14-35 years were recruited to complete three validated surveys: (1) Eating Disorder Examination Questionnaire (EDE-Q), (2) Nine-Item Avoidant/Restrictive Food Intake Disorder Scale (NIAS), and (3) Cystic Fibrosis Questionnaire-Revised (CFQ-R). Univariate linear regression analysis identified baseline risk factors associated with these survey scores. Variables with univariate p < 0.20 were considered for inclusion in a multivariable linear regression model. Backwards stepwise linear regression was used to identify the final model. RESULTS: A total of 52 patients enrolled. The prevalence of a positive screen on the EDE-Q was 9.6%, and on the NIAS was 13.5%. The CFQ-R eating and weight subscales were associated with scores on the EDE-Q, and CFQ-R eating subscale and being dF508 homozygous were correlated with the NIAS total score. DISCUSSION: A clinically significant number of participants screened positive for eating disorders on the EDE-Q and NIAS. Scores on the eating and weight scales of the CFQ-R were associated with the scores on these surveys. Further work is needed to better understand the optimal way to use such tools to screen and treat for eating disorders in individuals with CF.

The Impact of Psychological Capital and Workplace Social Support on Pediatric Oncology Nurses' Post Traumatic Stress Disorder (PTSD) Symptomology.

Introduction: Work-related post traumatic stress disorder (PTSD) can develop in nurses. Pediatric oncology nursing is a potentially high-risk subspecialty for PTSD secondary to the nature of the work. This study aimed to describe the prevalence of PTSD symptomology and explore relationships between nurse psychological capital, workplace social supports, and PTSD symptomology in pediatric oncology nurses. Methods: The study utilized a cross-sectional correlational survey design. Pediatric oncology nurses working in direct patient care in the United States completed a demographics questionnaire, Psychological Capital Questionnaire (PCQ), Coworker Support Scale, Supervisor Support Scale, and the Post traumatic Checklist for Diagnostic and Statistical Manual of Mental Disorders (PCL-5). Descriptive and inferential statistics, including logistic regression models, were used to analyze data. A cutoff score of ≥31 on the PCL-5 was used to determine the prevalence of PTSD symptomology. Results: The sample included 424 nurses. The prevalence of PTSD symptomology was 13.4%. Work setting (inpatient), decreased coworker and leadership social support, and psychological capital were independently associated with PTSD symptomology. After controlling for covariates, only psychological capital was associated with PTSD symptomology. For a 1 unit decrease in PCQ score, pediatric oncology nurses were 4.25 times more likely to have PTSD symptomology. Discussion: PTSD prevalence rates in pediatric oncology nurses are aligned with rates found in other nursing specialties. Nurse psychological capital may play a protective role against PTSD symptomology. Implications for Future Research: Findings support PTSD as a serious workplace concern for nurses. Workplace programs that foster nurses' psychological capabilities should be considered to protect against the development of PTSD symptomology.

Health Impairments in Children and Adolescents After Hospitalization for Acute COVID-19 or MIS-C.

OBJECTIVES: To evaluate risk factors for postdischarge sequelae in children and adolescents hospitalized for acute coronavirus disease 2019 (COVID-19) or multisystem inflammatory syndrome in children (MIS-C). METHODS: Multicenter prospective cohort study conducted in 25 United States pediatric hospitals. Patients <21-years-old, hospitalized May 2020 to May 2021 for acute COVID-19 or MIS-C with follow-up 2 to 4 months after admission. We assessed readmissions, persistent symptoms or activity impairment, and new morbidities. Multivariable regression was used to calculate adjusted risk ratios (aRR) and 95% confidence intervals (CI). RESULTS: Of 358 eligible patients, 2 to 4 month survey data were available for 119 of 155 (76.8%) with acute COVID-19 and 160 of 203 (78.8%) with MIS-C. Thirteen (11%) patients with acute COVID-19 and 12 (8%) with MIS-C had a readmission. Thirty-two (26.9%) patients with acute COVID-19 had persistent symptoms (22.7%) or activity impairment (14.3%) and 48 (30.0%) with MIS-C had persistent symptoms (20.0%) or activity impairment (21.3%). For patients with acute COVID-19, persistent symptoms (aRR, 1.29 [95% CI, 1.04-1.59]) and activity impairment (aRR, 1.37 [95% CI, 1.06-1.78]) were associated with more organ systems involved. Patients with MIS-C and pre-existing respiratory conditions more frequently had persistent symptoms (aRR, 3.09 [95% CI, 1.55-6.14]) and those with obesity more frequently had activity impairment (aRR, 2.52 [95% CI, 1.35-4.69]). New morbidities were infrequent (9% COVID-19, 1% MIS-C). CONCLUSIONS: Over 1 in 4 children hospitalized with acute COVID-19 or MIS-C experienced persistent symptoms or activity impairment for at least 2 months. Patients with MIS-C and respiratory conditions or obesity are at higher risk of prolonged recovery.

Attitudes and behaviors of physical activity in children: Findings from the Play, Lifestyle & Activity in Youth (PLAY) Questionnaire.

BACKGROUND: The identified domains of child physical literacy include knowledge/understanding, behavior, motivation/confidence, and physical competency. Understanding physical literacy is critical to moving forward intervention efforts surrounding physical inactivity. OBJECTIVES: To investigate physical literacy domains by presenting responses to the Play, Lifestyle & Activity in Youth (PLAY) questionnaire and analyze the relationship between comparable questions in the child and parent sections. DESIGN: Cross-sectional questionnaire study. SETTING: Suburban youth program, United States. MAIN OUTCOME MEASURE: Parent and child responses to the PLAY questionnaire. RESULTS: Questionnaire data were collected for 106 children 6-11 years old and 91 parents. Children (93.3%, 83/91) reported enjoyment in physical education, despite 70% participation in physical education 0-1 day/week. 66.6% (26/91) of children preferred active to passive play and played outside most days/week (5.0 median). Similarly, a high proportion of children (93.3%, 83/91) reported feeling that they physically performed similar to or higher than their peers and took ownership to improve motor competency versus taking a more passive approach. Most children (96.7%, 87/91) reported feeling happy when they were able to play sports/run around, enjoyed gym class (93.3%, 83/91), and reported feeling sad if they cannot play sports/run around during the day (75.6%, 68/91). Parents and children reported rules around screen time and bed time, and reinforced these rules often. Child and parent responses were associated for comparable questions about bedtime and screen-time/media rules, moderate-to-vigorous physical activity per day, sport camp preference, and play equipment use (Fisher's p = .051 screen time, p < .001 other questions). CONCLUSIONS: PLAY questionnaire internal validity showed agreement between comparable parent and child responses and an association for select comparable questions.

Characteristics and outcomes of autoimmune hemolytic anemia after pediatric allogeneic stem cell transplant.

BACKGROUND: Autoimmune hemolytic anemia (AIHA) after allogeneic hematopoietic stem cell transplant (HSCT) is a rare but complex and serious complication. Detailed descriptions of cases and management strategies are needed due to lack of prospective trials. OBJECTIVES: Describe the incidence, clinical characteristics, and management of AIHA after HSCT in a pediatric cohort. METHODS: This is a retrospective cohort study of 33 pediatric patients with AIHA after HSCT at an academic tertiary care center from 2003 to 2019. RESULTS: The overall incidence of AIHA after allogeneic HSCT was 3.8% (33/868). AIHA was significantly more common after transplant for nonmalignant versus malignant diagnoses (7.0% [26/370] vs. 1.4% [7/498], p < .0001). AIHA developed at a median of 4.7 months (range 1.0-29.7) after transplant. Sixteen of 33 patients (48.5%) required new AIHA-directed pharmacologic therapy; 17 (51.5%) were managed on their current immunosuppression and supportive care. Patients managed without additional therapy were significantly older, more likely to have a malignant diagnosis, and tended to develop AIHA at an earlier time point after transplant. Patients received a median of two red blood cell transfusions within the first 2 weeks of diagnosis and a median of one AIHA-directed medication (range one to four), most commonly corticosteroids and rituximab. CONCLUSIONS: AIHA after HSCT is rare but occurs more commonly in patients transplanted for nonmalignant diagnoses. While some pediatric patients who develop AIHA after transplant can be managed on current immunosuppression and supportive care, many require AIHA-directed therapy including second-line medications.

Response to rituximab in children and adults with immune thrombocytopenia (ITP).

BACKGROUND: Rituximab is a monoclonal anti-CD20 antibody used as a second-line treatment for immune thrombocytopenia (ITP). As additional treatments for ITP emerge, identifying the most appropriate patients and optimal timing for rituximab are important but challenging without established predictors of response to therapy. OBJECTIVES: The purpose of this study was to describe demographic, clinical, and laboratory characteristics of pediatric and adult patients with ITP to identify differences in evaluation before rituximab administration and correlates of platelet response. METHODS: This is a retrospective cohort study describing the characteristics of patients with ITP treated with rituximab from 2010 to 2020 at two academic tertiary care centers. RESULTS: A total of 64 patients met criteria for inclusion. Complete rituximab response (56%) was not significantly different between children (58%, n = 24) and adults (55%, n = 40). Response rate was similar in those with primary versus secondary ITP (53% vs 62%). Among patients treated with rituximab, Evans Syndrome was more common in children than adults (42% vs 18%). Immunologic labs assessed before rituximab varied by age and were more commonly evaluated in children (lymphocyte subsets 88% vs 22%). Immunologic markers, including antinuclear antibody, direct antiglobulin testing, immunoglobulin levels, and lymphocyte subsets, did not predict response to rituximab in pediatric or adult patients with ITP. CONCLUSIONS: Pre-rituximab immunologic evaluation varied significantly between adults and children, which could represent institution-specific practice patterns or a more general practice difference. If the latter, underlying immunodeficiency in adults with ITP may be underrecognized. Standardized guidance for pre-rituximab immunologic evaluation is needed.

Attitudes towards involving children in decision-making surrounding lung transplantation.

BACKGROUND: Medical care has shifted from a paternalistic model towards one centered around patient autonomy and shared decision-making (SDM), yet the role of the pediatric patient in decision-making is unclear. Studies suggest that many children with chronic disease are capable of making medical decisions at a young age, yet no standardized approaches have been developed for involving children in these decisions. METHODS: This is a single-center survey study investigating the attitudes of pediatric pulmonologists towards involvement of children in decisions regarding lung transplantation, utilizing a hypothetical case scenario with systematic manipulation of age and maturity level. We evaluated physician belief regarding ultimate decision-making authority, reconciliation of parent-child discordance, and utility of ethics and psychiatry consultation services. RESULTS: The majority of pediatric pulmonologists at this center believe decision-making authority rests with the parents. The effects of age and maturity are unclear. In instances of parent-child disagreement, physicians are more likely to try to convince parents to defer to the child if the child is both older and more mature. Physicians are divided on the utility of ethics and psychiatry consultations. CONCLUSION: Involvement of children with cystic fibrosis in SDM is broadly supported but inconsistently implemented. Despite evidence that children with chronic disease may have decisional capacity at a young age, the majority of physicians still grant decisional authority to parents. There are numerous barriers to involving children in decisions, including legal considerations. The role of age and maturity level in influencing these decisions appears small and warrants further investigation.

High prevalence of periodic limb movements of sleep in children with Down syndrome.

STUDY OBJECTIVES: Increased periodic limb movements of sleep (PLMS), > 5 events/h, are present in 1.2% to 7.7% of healthy children and associated with hypertension, attention deficit, and hyperactivity. This study sought to determine the prevalence of elevated PLMS in a large cohort of children with Down syndrome (DS) and their correlation with OSA and ferritin levels. METHODS: Retrospective chart review of all children with DS ages 2 to 18 years in whom single baseline polysomnography (PSG) was performed at a pediatric hospital over 5 years. RESULTS: A total of 418 children met inclusion criteria. Three hundred fifty-six children (85%) were referred because of concerns about sleep-disordered breathing; 49 (12%) were referred for screening per American Academy of Pediatrics (AAP) guidelines; and 13 (3%) because of concerns about restless legs or periodic limb movement disorder. One hundred thirty-nine children (33.3%) had elevated PLMS; they were younger (6.3 years) than those without elevated PLMS (7.7 years). OSA was present in 176/418 (42.1%) children, including 13/49 (26.2%) asymptomatic children referred for screening PSG. Ferritin levels were only recorded in the charts of 65 of the children with elevated PLMS (46.7%); in 36 (55.4%) levels were < 50 ng/mL. CONCLUSIONS: PLMS were increased in a substantial number of this large cohort of children with DS. Additional studies are necessary to assess utility of laboratory testing to predicting PLMS in similar, at-risk, populations. Screening PSG has value in identifying OSA in young, ostensibly asymptomatic children with DS. The prevalence of OSA increased with age in this cohort, unlike in typical children, requiring health care providers to remain vigilant for its emergence across the lifespan.