Beneficial Effect of Intravenous Dexamethasone in Children With Mild to Moderately Severe Acute Chest Syndrome Complicating Sickle Cell Disease

Abstract

Acute chest syndrome (ACS) in patients with sickle cell disease (SCD) has historically been managed with oxygen, antibiotics, and blood transfusions. Recently high-dose corticosteroid therapy was shown to reduce the duration of hospitalization in children with SCD and vaso-occlusive crisis. Therefore, we chose to assess the use of glucocorticoids in ACS. We conducted a randomized, double-blind placebo-controlled trial to evaluate the efficacy and toxicity of intravenous dexamethasone (0.3 mg/kg every 12 hours × 4 doses) in children with SCD hospitalized with mild to moderately severe ACS. Forty-three evaluable episodes of ACS occurred in 38 children (median age, 6.7 years). Twenty-two patients received dexamethasone and 21 patients received placebo. There were no statistically significant differences in demographic, clinical, or laboratory characteristics between the two groups. Mean hospital stay was shorter in the dexamethasone-treated group (47 hours v 80 hours; P = .005). Dexamethasone therapy prevented clinical deterioration and reduced the need for blood transfusions (P < .001 and = .013, respectively). Mean duration of oxygen and analgesic therapy, number of opioid doses, and the duration of fever was also significantly reduced in the dexamethasone-treated patients. Of seven patients readmitted within 72 hours after discharge (six after dexamethasone; P = .095), only one had respiratory complications (P = 1.00). No side effects clearly related to dexamethasone were observed. In a stepwise multiple linear regression analysis, gender and previous episodes of ACS were the only variables that appeared to predict response to dexamethasone, as measured by lengh of hospital stay. Intravenous dexamethasone has a beneficial effect in children with SCD hospitalized with mild to moderately severe acute chest syndrome. Further study of this therapeutic modality is indicated. © 1998 by The American Society of Hematology.

Acute chest syndrome: sickle cell disease

“…The role of corticosteroids in the treatment for ACS is controversial. A small, randomized, controlled trial (82) demonstrated that corticosteroid administration resulted in the rapid resolution of mild-to moderate-ACS episodes. A short course of dexamethasone therapy may attenuate the course of ACS of sickle cell disease, but it also increases the risk of early readmission after discharge.…”

The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy

“…A clinical trial of hydroxyurea revealed that leukocyte counts correlated strongly with pain crisis rates (32). Acute vasoocclusive crisis has been precipitated inadvertently by administration of granulocytosis-inducing growth factors to sickle patients (1,5,138,191), and antiinflammatory steroids have an ameliorating effect on pain crisis (62) and acute chest syndrome (18). Acute painful episodes are not uncommonly accompanied by fever.…”

Red blood cell storage duration is not associated with clinical outcomes for acute chest syndrome in children with sickle cell disease

“…The median storage duration of the oldest unit of transfused RBCs was 17 days (interquartile range [IQR], [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26], with a range of 2 to 41 days. When evaluated as a categorical variable, 36.8% of the transfused units were stored for less than 14 days, 25.6% were stored between 14 and 20 days, 17.1% were stored between 21 and 27 days, and 20.5% were stored for 28 days or more.…”

“…A validated ACS severity score does not exist, so a combination of reported severity scores and clinical experience was used to create the score depicted in Table 1. 24,25 A history of asthma was determined by reviewing diagnoses and past medical history in the medical records. Concomitant complications were included if the diagnosis was documented by a provider or there was a confirmatory laboratory result.…”