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

Summary 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.

Prevalence of hidradenitis suppurativa among patients with Down syndrome: a population-based cross-sectional analysis

“…This method has also been used to establish disease burden in the U.S.A . The use of administrative codes to identify the DS cohort has been validated previously with a demonstrated PPV of 79%, and this method has also been applied recently in epidemiological and registry studies involving DS …”

“…22 This method has also been used to establish disease burden in the U.S.A. 6,7 The use of administrative codes to identify the DS cohort has been validated previously with a demonstrated PPV of 79%, 23 and this method has also been applied recently in epidemiological and registry studies involving DS. [24][25][26][27][28][29] In secondary analyses, we recorded counts of first ever HS diagnoses within the last 5 years for the range of 19 age groups at 5-year intervals (ranging from 0-4 years to ≥ 90 years) in order to compare the age of HS onset in patients with and without DS. Human Subjects Committee review was waived as there are no identifiers associated with the data.…”

Comparable on‐therapy mortality and supportive care requirements in Black and White patients following initial induction for pediatric acute myeloid leukemia

“…Patient characteristics included age, sex, insurance type (private, public, or other), and time period of diagnosis (2004–2009 vs 2010–2014). ICU‐level resource utilization during the first 72 hours following admission for initial AML chemotherapy was also included as a covariate, where the timeframe was chosen a priori to evaluate clinical acuity at presentation rather than acuity resulting from chemotherapy toxicity . Acuity of presentation was categorized into three groups (none, 1 system, and ≥2 systems) based on the number of organ systems requiring ICU‐level resources within the first 72 hours of the diagnostic admission.…”

“…ICU-level resource utilization during the first 72 hours following admission for initial AML chemotherapy was also included as a covariate, where the timeframe was chosen a priori to evaluate clinical acuity at presentation rather than acuity resulting from chemotherapy toxicity. 11,19 Acuity of presentation was categorized into three groups (none, 1 system, and ≥2 systems) based on the num-…”

Unusual lymphoid malignancy and treatment response in two children with Down syndrome

“…[6][7][8]22 Treatment-related mortality and morbidity, chiefly due to infection, is elevated during all phases of ALL treatment in children with DS, including the maintenance phase. 3,23 Infusions of high-dose MTX in particular, which during highrisk ALL treatment typically occur over 24 hours at doses ranging from 2 to 5 g/m 2 , 24,25 can be associated with severe mucositis and stomatitis in children with DS. 26 With this background, there was significant motivation to reduce the dose of HD-MTX for this patient with DS and BL from the start.…”