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Clinical course and outcome for critically ill children with Down syndrome: a retrospective cohort study
Shane M Tibby1, Andrew Durward, Chong Tien Goh
1Paediatric Intensive Care Unit, Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, SE1 7EH, UK. Shane.tibby@gstt.nhs.uk
Insights
Children with Down syndrome (DS) admitted to intensive care units (ICUs) require more organ support. Their mortality risk increases with longer ICU stays, suggesting unique responses to critical illness.
Area of Science:
- Pediatric critical care medicine
- Genetics and genomics
- Down syndrome research
Background:
- Children with Down syndrome (DS) present unique genetic profiles due to trisomy 21, potentially impacting their physiological responses to critical illness.
- Understanding the intensive care unit (ICU) course for children with DS is crucial for optimizing treatment strategies and outcomes.
Purpose of the Study:
- To compare the intensive care unit (ICU) course and outcomes of children with Down syndrome (DS) against a cohort of children without DS.
- To investigate if children with DS have a different risk of ICU mortality compared to their non-DS counterparts, considering confounding factors.
Main Methods:
- A retrospective cohort study was conducted across four English pediatric ICUs between 2003 and 2009.
- A competing risks model was employed to analyze the subhazard (risk) for ICU mortality in children with DS, adjusting for key confounders.
Main Results:
- Children with DS (n=1,278) showed lower crude ICU mortality (4.2% vs. 6.2%).
- Despite lower initial disease severity, DS patients required more cardiovascular support. Their mortality risk, initially lower (subhazard 0.63), increased to match non-DS patients by day 10 and subsequently exceeded it.
- The competing risks model indicated that the length of ICU stay significantly influenced mortality risk for children with DS.
Conclusions:
- Children with DS necessitate a higher degree of organ support relative to their admission disease severity.
- The mortality risk for pediatric patients with Down syndrome is significantly influenced by the duration of their ICU stay.
- These findings highlight potential differences in how children with DS respond to critical illness, possibly due to genetic factors or variations in case mix.
Purpose:
Children with Down syndrome (DS) have several genetic anomalies within chromosome 21 which may influence their response to critical illness. We compared the intensive care course and outcome of children with DS versus those without.
Methods:
Retrospective cohort study in four English paediatric intensive care units (ICUs) (2003-2009, n = 33,485). We examined, via a competing risks model, whether risk (subhazard) for ICU mortality differed for children with DS, after adjusting for important confounders.
Results:
DS patients exhibited lower disease severity at ICU admission but subsequently required a higher proportion of cardiovascular support, and similar renal support to non-DS patients. Children with DS (n = 1,278) had lower crude mortality than those without (4.2 versus 6.2 %, p = 0.003). This was not significant when expressed as standardized mortality ratio: 0.83 [95 % confidence interval (CI) 0.63-1.09] versus 0.90 (95 % CI 0.86-0.94). However, the competing risks model showed that mortality risk was influenced by length of ICU stay. At admission, DS patients exhibited a subhazard for mortality of 0.63 (95 % CI 0.46-0.85), which increased to 1.00 by day 10 of admission, and continued rising above that of non-DS children thereafter.
Conclusions:
Children with DS require a higher proportion of organ support than expected by disease severity at ICU admission. In addition, the mortality risk for children with DS is dependent upon length of ICU stay. These findings could reflect differences in case mix, but are also compatible with different response to critical illness in this group.
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