Acute chest syndrome in sickle cell disease in Saudi Arab children in the Eastern Province

Background: This study was conducted to define the clinical features and outcome of acute chest syndrome (ACS) in sickle cell disease (SCD) patients in the Eastern Province of Saudi Arabia Patients and Methods: This was a prospective study involving patients who were 12 years or younger, admitted to Qatif Central Hospital with ACS (or developed ACS during hospitalization) between July 1992 and July 1997. Chest x-ray, CBC, cultures (blood, sputum and throat), mycoplasma titers and blood gases were performed at the onset of ACS. Oxygen therapy, antibiotics, blood transfusion and mechanical ventilation were used as required Results: One hundred and thirty-two patients with episodes of ACS (154 admissions which accounted for 7.7% of SCD admissions) were studied. Fever, cough and chest pain were the most common symptoms. Raised temperature, tachypnea and tachycardia were the most common findings. ACS was associated with painful crisis (46.8%) and infections (13%). It was mild in 31.2%, moderate in 57.1% and severe in 11.7% of admissions. Radiological studies revealed unilateral infiltrate in 69.5%, bilateral infiltrate in 20.8% and pleural effusion in 3.3%. There was a significant drop in Rb and platelets, and a rise in WBC. Significant hypoxia was found in 10.4% and bacteremia was found in 7.1%. Cephalosporine was required for 37%, simple blood transfusion for 74%, exchange transfusion for 2%, and mechanical ventilation for 0.7% of admissions. None of our patients died. Mean duration of hospitalization was 6.7 days. Conclusion: Acute chest syndrome in children with sickle cell disease in the Eastern Province of Saudi Arabia is relatively mild and infrequent, and rarely associated with bacteremia.