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Effect of transfusion in acute chest syndrome of sickle cell disease
U Emre1, S T Miller, M Gutierez
1Children's Medical Center of Brooklyn, State University of New York Health Science Center, USA.
Insights
Blood transfusions significantly improve oxygenation in children with sickle cell disease and acute chest syndrome. This treatment is a valuable addition to managing acute chest syndrome in pediatric patients.
Area of Science:
- Pediatric Hematology
- Pulmonary Medicine
- Sickle Cell Disease Management
Background:
- Acute chest syndrome (ACS) is a common and serious complication in children with sickle cell disease (SCD).
- Effective management strategies for ACS in pediatric SCD patients are crucial for improving outcomes.
Purpose of the Study:
- To evaluate the impact of blood transfusions on clinical course and oxygenation in pediatric patients with SCD and ACS.
- To determine if blood transfusion therapy improves oxygenation indices in children experiencing ACS.
Main Methods:
- A prospective study involving 36 children with SCD over a 2-year period, analyzing 40 episodes of ACS.
- Clinical severity scores and arterial blood gas values were assessed before and after transfusion.
- Comparison groups included transfused patients and a small cohort (n=6) not receiving transfusions.
Main Results:
- Blood transfusion was administered in 67.5% of ACS episodes, with various transfusion types used.
- Significant improvement in oxygenation indices was observed post-transfusion.
- No significant differences in clinical recovery metrics (fever, tachypnea, hospital stay) were noted between transfused and non-transfused groups.
Conclusions:
- Blood transfusion, including simple packed red blood cell transfusion, effectively enhances oxygenation in pediatric patients with ACS.
- Transfusion serves as a valuable therapeutic adjunct for managing acute chest syndrome in children with sickle cell disease.
Objective:
To study the effects of transfusion on the clinical course and oxygenation indexes of children with sickle cell disease and acute chest syndrome.
Methods:
During a 2-year period, 36 children with sickle cell disease admitted with a total of 40 episodes of acute chest syndrome were examined. Patients were given a clinical severity score indicative of the degree of respiratory distress. Arterial blood gas values were determined 4 to 24 hours before and 12 to 24 hours after transfusion, and indexes of oxygenation were calculated; six patients who were not given transfusions also had blood gases measured on admission and approximately 24 hours later for comparison.
Results:
Blood transfusion was administered during 27 episodes (67.5%); 20 children received a simple packed cell transfusion, four had a partial packed cell exchange transfusion, and three had a simple transfusion followed by whole blood exchange transfusion because of worsening clinical symptoms. Although there was no significant change in oxygenation indexes for the six patients not treated with transfusion, there was significant improvement in all indexes after transfusion. The transfused group had more severe disease on admission, but there was no significant difference in duration of fever, tachypnea, retractions, or hospital stay between the transfusion and the nontransfusion groups.
Conclusion:
Blood transfusion, even simple transfusion of packed erythrocytes, significantly improves oxygenation in children with acute chest syndrome and is a valuable adjunct to therapy.