title: Supportive care utilization and treatment toxicity in children with Down syndrome and acute lymphoid leukaemia at free-standing paediatric hospitals in the United States.
abstract: Although inferior outcomes of children with Down syndrome (DS) and acute lymphoid leukaemia (ALL) are established, national supportive care patterns for these patients are unknown. A validated retrospective cohort of paediatric patients diagnosed with ALL from 1999 to 2011 was assembled from the US Pediatric Health Information System (PHIS) database to examine organ toxicity, sepsis, and resource utilization in children with and without DS. Among 10699 ALL patients, 298 had DS-ALL (2·8%). In a multivariate model, DS was associated with increased risk of cardiovascular (odds ratio [OR] 2·0, 95% confidence interval [CI] 1·6-2·7), respiratory (OR 2·1, 95% CI: 1·6-2·9), neurologic (OR 3·4, 95% CI 1·9-6·2), and hepatic (OR 1·4, 95% CI 1·0-1·9) dysfunction and sepsis (OR 1·8, 95% CI: 1·4-2·4). Children with DS-ALL used significantly more respiratory support, insulin, and anti-infectives, including broad-spectrum Gram-positive agents, quinolones, and azoles. They used significantly fewer analgesics and antiemetics compared to non-DS-ALL children. Ultimately, this study confirms the increased risk of infectious and end-organ toxicity in children with DS-ALL and quantifies important differences in resource utilization between children with DS and non-DS ALL. These findings highlight the importance of investigating the impact of these care variations and developing specific supportive care guidelines for this population.
authors.affiliation: Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
authors.forename: Elizabeth G
authors.initials: EG
authors.lastname: Salazar
authors.affiliation: Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.|The Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
authors.forename: Yimei
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.|The Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.|Division of Infectious Diseases, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.|Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Brian T
authors.initials: BT
authors.lastname: Fisher
authors.affiliation: Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.|Department of Anesthesia and Critical Care Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Susan R
authors.initials: SR
authors.lastname: Rheingold
authors.affiliation: Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.|Department of Anesthesia and Critical Care Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Julie
authors.initials: J
authors.lastname: Fitzgerald
authors.affiliation: Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.|Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Alix E
authors.initials: AE
authors.lastname: Seif
authors.affiliation: Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Yuan-Shung
authors.initials: YS
authors.lastname: Huang
authors.affiliation: Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.|Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Rochelle
authors.initials: R
authors.lastname: Bagatell
authors.affiliation: Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.|Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.|The Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.|Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Richard
authors.initials: R
authors.lastname: Aplenc
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D004314:Down Syndrome / Q000150:complications / Q000453:epidemiology / Q000628:therapy*; D064420:Drug-Related Side Effects and Adverse Reactions / Q000453:epidemiology; D005260:Female; D006295:Health Resources / Q000706:statistics & numerical data*; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000453:epidemiology / Q000628:therapy*; D012189:Retrospective Studies; D014481:United States
keywords: Down syndrome; acute lymphoid leukaemia; chemotherapy toxicity; paediatric leukaemia; supportive care