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Updated: Jun 8, 2025
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Biomarkers to Differentiate Acute Chest Syndrome From Vaso-Occlusive Crisis in Children With Sickle Cell Disease
Karen Wang1, Nelida Olave2, Saurabh Aggarwal3
1Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Insights
Biomarkers can help distinguish Acute Chest Syndrome (ACS) from pain crises in children with sickle cell disease (SCD). Early identification of ACS using these markers can reduce mortality and morbidity in pediatric SCD patients.
Area of Science:
- Pediatric Hematology
- Critical Care Medicine
- Biomarker Discovery
Background:
- Acute Chest Syndrome (ACS) is a primary cause of mortality in pediatric sickle cell disease (SCD) in the US.
- Approximately 50% of children with ACS initially present with pain, complicating early diagnosis.
Purpose of the Study:
- To identify specific biomarkers that differentiate ACS from vaso-occlusive crises (VOC) in children with SCD presenting with pain.
- To enable earlier and more accurate diagnosis of ACS for improved patient outcomes.
Main Methods:
- Prospective cohort study of pediatric patients with SCD presenting to the emergency department (ED) with pain.
- Patients were categorized into ACS or VOC groups based on discharge diagnosis.
- Analysis of clinical data and specific biomarker levels (e.g., sPLA2, IFN-γ, IL-10, IL-12).
Main Results:
- 7 patients diagnosed with ACS and 19 with VOC were studied.
- ACS patients exhibited higher respiratory rates, lower oxygen saturation, and longer hospital stays.
- Elevated levels of white blood cell count, glucose, anion gap, sPLA2, IFN-γ, IL-10, and IL-12 were observed in the ACS group compared to the VOC group.
Conclusions:
- Specific biomarkers were identified that are significantly associated with the development of ACS in children with SCD presenting with pain.
- These biomarkers facilitate earlier intervention for ACS, potentially reducing mortality and morbidity in this vulnerable population.
Background:
Acute Chest Syndrome (ACS) is the leading cause of death in children with sickle cell disease (SCD) in the US-about half of the children who develop ACS present initially with pain.
Methods:
Here, we studied biomarkers to differentiate ACS from vaso-occlusive crises (VOC) in children with SCD who presented with pain to the emergency department (ED). We conducted a prospective cohort study of consecutive patients who presented to the ED with pain and were discharged with ACS or VOC between March, 2017 and February, 2020.
Results:
We identified 7 patients with ACS and 19 patients with VOC. The two groups were comparable in age and sex. All patients with ACS had asthma versus 42% of the VOC group. The ACS group had lower weight and BMI z-scores. Patients with ACS compared to VOC had significantly higher respiratory rates, lower O2 saturation, and longer hospital stays. They also had higher white blood cell count, glucose level (> 99 mg/dL), anion gap (> 9 mEq/L), sPLA2 (> 7 pg/mL), IFN-γ (> 17.8 pg/mL), IL-10 (1.54 pg/mL), and IL-12 (> 0.5 pg/mL) levels.
Conclusions:
We identified biomarkers associated with ACS development in children with SCD presenting with pain that allow for earlier ACS interventions to reduce mortality and morbidity.
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