EFFECT OF TRANSFUSION IN ACUTE CHEST SYNDROME OF 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 sick le cell disease admitted with a total of 40 episodes of acute chest sy ndrome were examined, Patients were given a clinical severity score in dicative of the degree of respiratory distress. Arterial blood gas val ues were determined 4 to 24 hours before and 12 to 24 hours after tran sfusion, 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 trans fusion was administered during 27 episodes (67.5%); 20 children receiv ed a simple packed cell transfusion, four had a partial packed cell ex change transfusion, and three had a simple transfusion followed by who le blood exchange transfusion because of worsening clinical symptoms. Although there was no significant change in oxygenation indexes for th e six patients not treated with transfusion, there was significant imp rovement in all indexes after transfusion. The transfused group had mo re severe disease on admission, but there was no significant differenc e in duration of fever, tachypnea, retractions, or hospital stay betwe en the transfusion and the nontransfusion groups. Conclusion: Blood tr ansfusion, even simple transfusion of packed erythrocytes, significant ly improves oxygenation in children with acute chest syndrome and is a valuable adjunct to therapy.