Table 2.

Characteristics of acute chest syndrome patients.*

Age at First Episode of Acute Chest Syndrome
CharacteristicAll Pts.(n=537)0-9 yr(n=264)10-19 yr(n=145)>20 yr(n=128)P Value**
* Because of rounding, percentages may not total 100. Data on one patient were excluded because the patient’s birth date was not known. 
** P values were calculated by chi-square analyses. 
+ Other reasons included a vaso-occlusive event, anemia, fever, infection, gastrointestinal or abdominal pain, asthma, urologic and orthopedic conditions, heart failure, and surgery. 
Reprinted with permission from Vichinsky E et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342:1855-65. 
Sex (%) 
    Male 58 63 57 48 0.02 
    Female 42 38 43 53  
Hemoglobinopathy (%) 
    SS 82 83 81 83 0.94 
    Other 18 17 19 17  
Mean age at first episode of acute chest syndrome (yr) 13.8 5.4 14.6 30.2 <0.001 
Medical history (%) 
    Vaso-occlusive event 80 72 85 89 <0.001 
    Transfusion 74 63 84 86 <0.001 
    Acute chest syndrome or pneumonia 67 63 66 76 0.03 
    Prophylactic antibiotic therapy 57 81 51 13 <0.001 
    Major surgery 40 26 48 61 <0.001 
    Neurologic disease 16 10 17 29 <0.001 
    Sleep apnea 14 14 17 11 0.41 
    Red-cell antibodies 12 7 13 21 <0.001 
    Aseptic necrosis or fracture 12 5 11 26 <0.001 
    Asthma 11 14 9 5 <0.001 
    Smoking 10 0 4 36 <0.001 
    Renal disease or urinary tract infection 7 3 7 14 <0.001 
    Chronic transfusion therapy 5 5 5 7 0.64 
    Cardiac disease 4 1 3 12 <0.001 
    Chronic lung disease 4 3 4 5 0.46 
    Pulmonary edema 4 1 3 9 <0.001 
    Clubbing 2 1 0 5 0.002 
    Deep-vein thrombosis 1 <1 0 3 0.01 
    Tuberculosis <1 0 0 1 0.20 
Reason for current admission (%) 
    Acute chest syndrome 52 61 46 42 <0.001 
     Other+ 48 39 54 58  
Symptoms at diagnosis of acute chest syndrome (%) 
    Fever 80 86 78 70 <0.001 
    Cough 62 69 58 54 0.006 
    Chest pain 44 27 67 55 <0.001 
    Tachypnea 45 47 67 55 <0.001 
    Shortness of breath 41 31 44 58 <0.001 
    Pain in arms and legs 37 22 44 59 <0.001 
    Abdominal pain 35 38 36 29 0.24 
    Rib or sternal pain 21 14 26 30 <0.001 
    Reactive airway disease 13 17 12 6 0.01 
    Neurologic dysfunction 4 3 3 7 0.11 
    Cyanosis 2 1 3 3 0.24 
    Heart failure 1 2 1 1 0.49 
Age at First Episode of Acute Chest Syndrome
CharacteristicAll Pts.(n=537)0-9 yr(n=264)10-19 yr(n=145)>20 yr(n=128)P Value**
* Because of rounding, percentages may not total 100. Data on one patient were excluded because the patient’s birth date was not known. 
** P values were calculated by chi-square analyses. 
+ Other reasons included a vaso-occlusive event, anemia, fever, infection, gastrointestinal or abdominal pain, asthma, urologic and orthopedic conditions, heart failure, and surgery. 
Reprinted with permission from Vichinsky E et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342:1855-65. 
Sex (%) 
    Male 58 63 57 48 0.02 
    Female 42 38 43 53  
Hemoglobinopathy (%) 
    SS 82 83 81 83 0.94 
    Other 18 17 19 17  
Mean age at first episode of acute chest syndrome (yr) 13.8 5.4 14.6 30.2 <0.001 
Medical history (%) 
    Vaso-occlusive event 80 72 85 89 <0.001 
    Transfusion 74 63 84 86 <0.001 
    Acute chest syndrome or pneumonia 67 63 66 76 0.03 
    Prophylactic antibiotic therapy 57 81 51 13 <0.001 
    Major surgery 40 26 48 61 <0.001 
    Neurologic disease 16 10 17 29 <0.001 
    Sleep apnea 14 14 17 11 0.41 
    Red-cell antibodies 12 7 13 21 <0.001 
    Aseptic necrosis or fracture 12 5 11 26 <0.001 
    Asthma 11 14 9 5 <0.001 
    Smoking 10 0 4 36 <0.001 
    Renal disease or urinary tract infection 7 3 7 14 <0.001 
    Chronic transfusion therapy 5 5 5 7 0.64 
    Cardiac disease 4 1 3 12 <0.001 
    Chronic lung disease 4 3 4 5 0.46 
    Pulmonary edema 4 1 3 9 <0.001 
    Clubbing 2 1 0 5 0.002 
    Deep-vein thrombosis 1 <1 0 3 0.01 
    Tuberculosis <1 0 0 1 0.20 
Reason for current admission (%) 
    Acute chest syndrome 52 61 46 42 <0.001 
     Other+ 48 39 54 58  
Symptoms at diagnosis of acute chest syndrome (%) 
    Fever 80 86 78 70 <0.001 
    Cough 62 69 58 54 0.006 
    Chest pain 44 27 67 55 <0.001 
    Tachypnea 45 47 67 55 <0.001 
    Shortness of breath 41 31 44 58 <0.001 
    Pain in arms and legs 37 22 44 59 <0.001 
    Abdominal pain 35 38 36 29 0.24 
    Rib or sternal pain 21 14 26 30 <0.001 
    Reactive airway disease 13 17 12 6 0.01 
    Neurologic dysfunction 4 3 3 7 0.11 
    Cyanosis 2 1 3 3 0.24 
    Heart failure 1 2 1 1 0.49 
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