Published online August 11, 2005
PEDIATRICS Vol. 116 No. 3 September 2005, pp. e420-e425 (doi:10.1542/peds.2004-2107)
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ELECTRONIC ARTICLE

Serum Biomarkers for Identifying Acute Chest Syndrome Among Patients Who Have Sickle Cell Disease and Present to the Emergency Department

James T. Naprawa, MD*, Bema K. Bonsu, MBChB*, Deborah G. Goodman, RN* and Mark A. Ranalli, MD{ddagger}

* Department of Medicine, Division of Emergency Medicine
{ddagger} Division of Hematology-Oncology, Children’s Hospital, Columbus, Ohio

Objective. To compare the accuracy of biomarkers for identifying acute chest syndrome (ACS) in patients with sickle cell disease presenting to a pediatric emergency department (ED).

Methods. We conducted a 13-month-long (2002–2003) cohort study with nested case-control in patients with sickle cell disease presenting to the pediatric ED with vaso-occlusive crises or fever in which we compared levels of secretory phospholipase A2 (sPLA2), endothelin-1, interleukin-6 (IL-6), and peripheral white blood cell count (WBC) in cases that were complicated by ACS and in control subjects with uncomplicated illnesses. For diagnosis, a test was considered to be accurate when the area under its receiver operator characteristic curve (AUC) was >0.70. Laboratory tests with AUC values ≥0.70 were entered into a binary recursive partitioning model for diagnosis.

Results. For the period of study, samples from 72 visits were obtained from 51 patients who presented with vaso-occlusive crises (range: 1–4 visits per patient; 15 were enrolled more than once). ACS complicated 19 of 72 visits (26%, 95% confidence interval: 17%–38%). At an AUC value of 0.79, only the sPLA2 test was accurate for diagnosing ACS. AUC values for peripheral WBC, endothelin-1, and IL-6 were 0.68, 0.51, and 0.52, respectively. Binary recursive partitioning retained only sPLA2 at a cutoff of 13.7 ng/mL to be accurate for diagnosis. This cutoff had a sensitivity of 74% (14 of 19), a specificity of 87% (46 of 53), a positive likelihood ratio of 5.6, and a negative likelihood ratio of 0.18.

Conclusions. Secretory phospholipase A2 but not endothelin-1, IL-6, or WBC is an accurate test for identifying present or incipient ACS in young patients who present to the ED with sickle cell pain crises.

Key Words: emergency medicine • sickle cell disease

Abbreviations: ACS, acute chest syndrome • SCD, sickle cell disease • VOC, vaso-occlusive crises • sPLA2, secretory phospholipase A2 • IL, interleukin • ED, emergency department • WBC, white blood cell count • ROC, receiver operator characteristic • AUC, area under the curve • SaO2, oxygen saturation

Accepted Mar 31, 2005.