Successful Treatment of Rapidly Progressive Interstitial Lung Disease in Juvenile Dermatomyositis.

Juvenile dermatomyositis (JDM) is a rare systemic autoimmune disease characterized by cutaneous findings, muscle inflammation, and vasculopathy. Patients with antimelanoma differentiation associated gene 5 (anti-MDA5) JDM may have subtle muscle weakness, absence of pathognomonic rashes, and more polyarthritis and ulcerative skin lesions when compared with other JDM subtypes. Although there is a known association of rapidly progressive interstitial lung disease (RP-ILD) in patients with anti-MDA5 dermatomyositis, few case reports describe this association in the pediatric literature. Even fewer reports describe successful treatment. We report an unusual case of RP-ILD in a pediatric patient with a hypomyopathic presentation complicated by prolonged intubation and pulmonary hemorrhage. A 4-year-old underweight female presented in respiratory distress and quickly progressed to severe hypoxic and hypercarbic respiratory failure requiring intubation. She experienced fatigue, intermittent fever, and transaminitis. The diagnosis was delayed because of recent travel history and a lack of typical cutaneous features of JDM, except a small erythematous papule on her antihelix. Her pulmonary hemorrhage and ear lesion prompted additional rheumatic workup. The myositis-specific antibody panel revealed high positive anti-MDA5 antibodies that prompted aggressive combination therapy with plasmapheresis, systemic steroids, inhibitors of Janus kinase, agents to deplete B-cells, and intravenous immunoglobulin. The patient responded well to treatment and was ultimately extubated and discharged. To our knowledge, this is the first thoroughly documented case of anti-MDA5 JDM with RP-ILD presenting with pulmonary hemorrhage and lacking typical cutaneous features. Early recognition of this highly fatal condition is important for improved prognosis.

Steering the Titanic: One tertiary care children's hospital's experience navigating safe sleep for hospitalized infants.

BACKGROUND: Sudden Unexpected Infant Death (SUID) is the leading cause of death in infants 1 month to 1 year of age in the United States. Despite extensive efforts in research and public education, rates of sleep-related infant death have plateaued since the late-1990s, largely due to unsafe sleep practices and environments. LOCAL PROBLEM: A multidisciplinary team assessed our institution's compliance with its own infant safe sleep policy. Data was collected on infant sleep practices, nurses' knowledge and training on the hospital policy, and teaching practices for parents and caregivers of hospitalized infants. Zero crib environments from our baseline observation met all the American Academy of Pediatrics recommendations for infant safe sleep. METHODS: A comprehensive safe sleep program was implemented in a large pediatric hospital system. The purpose of this quality improvement project was to improve compliance with safe sleep practice from 0% to 80%, documentation of infant sleep position and environment every shift from 0% to 90%, and documentation of caregiver education from 12% to 90% within 24 months. INTERVENTIONS: Interventions included revision of hospital policy, staff education, family education, environmental modifications, creation of a safe sleep taskforce, and electronic health record modifications. RESULTS: Documented compliance with infant safe sleep interventions at the bedside improved from 0% to 88%, while documentation of family safe sleep education improved from 12% to 97% during the study period. CONCLUSIONS: A multifaceted, multidisciplinary approach can lead to significant improvements in infant safe sleep practices and education in a large tertiary care children's hospital system.

Reasons and predictors for early termination of pediatric polysomnography: one children's hospital's experience.

STUDY OBJECTIVES: We sought to identify reasons for early polysomnography termination in pediatric patients. METHODS: We retrospectively reviewed all studies conducted at the Pediatric Sleep Center at UT Southwestern Medical Center from January 1, 2017, through December 31, 2019, that were terminated before study completion. We investigated reasons for early termination and gathered characteristics such as age, sex, presence of neurocognitive impairment, payor status, seasonal variability, and testing site location. RESULTS: There were 137 patients who terminated their studies before completion, comprising 1.05% of all patients who arrived for their scheduled polysomnographies during that time frame. The 3 main categories of reasons were equipment intolerance (47%), acute illness (33%), and parental refusal (20%). Parental refusal reasons included the patient's becoming combative, a lack of specialized equipment, patient's inability to fall asleep, forgetting the patient's nighttime medications, and the parent's inability to stay the night. Males represented a greater proportion of those who terminated due to intolerance of equipment (75%). Patients who terminated early due to equipment intolerance consisted of more neurologically impaired patients compared with those who terminated due to acute illness (56% vs 24%). Termination due to acute illness occurred more during the wintertime (44%) than in the summer (7%). In those who terminated due to parental refusal, there was a greater proportion of children under 6 years of age (75%). CONCLUSIONS: Determining factors that are associated with early polysomnography termination is an important step to help identify at-risk groups and implement strategies to improve study completion. CITATION: Luong S, Culp M, McCreary M, Wani A, Caraballo M. Reasons and predictors for early termination of pediatric polysomnography: one children's hospital's experience. J Clin Sleep Med. 2023;19(10):1711-1716.

Obstructive Sleep Apnea in Children With Autism.

STUDY OBJECTIVES: To describe the demographic and clinical characteristics of children with autism spectrum disorder (ASD) referred for polysomnography (PSG) and to look for predictors of obstructive sleep apnea (OSA) and severe OSA in these children. METHODS: This is a retrospective case series of children ages 2 to 18 years who underwent PSG between January 2009 and February 2015. Children were excluded if they had major comorbidities, prior tonsillectomy, or missing data. The following information was collected: age, sex, race, height, weight, tonsil size, and prior diagnosis of allergies, asthma, gastroesophageal reflux disease, seizure disorder, developmental delay, cerebral palsy, or attention deficit hyperactivity disorder. Predictors of OSA were evaluated. RESULTS: A total of 45 children were included with a mean (standard deviation [SD]) age of 6.1 years (2.8). The patients were 80% male, 49% Hispanic, 27% African American, 22% Caucasian, and 2.2% other. Of these children 26 (58%) had OSA (apnea-hypopnea index [AHI] > 1 event/h) and 15 (33%) were obese (body mass index, body mass index z-score ≥ 95th percentile). The mean (SD) AHI was 7.7 (15.0) events/h (range 1.0-76.6). A total of 9 (20%) had severe OSA (AHI ≥ 10 events/h). There were no demographic or clinical predictors of OSA in this group. However, increasing weight served as a predictor of severe OSA and African American or Hispanic children were more likely obese. CONCLUSIONS: The absence of demographic or clinical predictors of OSA supports using general indications for PSG in children with ASD.

Knowledge, Attitudes, and Risk for Sudden Unexpected Infant Death in Children of Adolescent Mothers: A Qualitative Study.

OBJECTIVE: To investigate practices, knowledge, attitudes, and beliefs regarding infant sleep among adolescent mothers, a demographic at high risk for sudden unexpected infant death, and to identify novel public health interventions targeting the particular reasons of this population. STUDY DESIGN: Seven targeted focus groups including 43 adolescent mothers were conducted at high school daycare centers throughout Colorado. Focus groups were recorded, transcribed, validated, and then analyzed in NVivo 10. Validation included coding consistency statistics and expert review. RESULTS: Most mothers knew many of the American Academy of Pediatrics recommendations for infant sleep. However, almost all teens reported bedsharing regularly and used loose blankets or soft bedding despite being informed of risks. Reasons for nonadherence to recommendations included beliefs that babies are safest and sleep more/better in bed with them, that bedsharing is a bonding opportunity, and that bedsharing is easier than using a separate sleep space. The most common justifications for blankets were infant comfort and concern that babies were cold. Participants' decision making was often influenced by their own mothers, with whom they often resided. Participants felt that their instincts trumped professional advice, even when in direct contradiction to safe sleep recommendations. CONCLUSIONS: Among focus group participants, adherence with safe sleep practices was poor despite awareness of the American Academy of Pediatrics recommendations. Many mothers expressed beliefs and instincts that infants were safe in various unsafe sleep environments. Future study should investigate the efficacy of alternative educational strategies, including education of grandmothers, who have significant influence over adolescent mothers.