Table 3.

Transfusion status at the time of enrollment and hemolytic triggers

Number of patients (n/N with known data)%Amish patients%Non-Amish patients%
Transfusion status at enrollment*       
 Historically on regular transfusions 79/198 40 32/46 70 47/152 31 
 Currently on regular transfusions 23§/198 12 0/46 23/152 15 
 Intermittent transfusions only 56/198 28 6/46 13 50/152 33 
 Never transfused 34/198 17 4/46 30/152 20 
 Historical transfusions with unknown transfusion frequency 6/198 4/46 2/152 
Hemolytic triggers       
 Presumed infections 137/228|| 60 41/53 30 96/175 70 
 Stress 59/207|| 29 36/54 61 23/153 39 
 Pregnancy 20/39 51 13/41 33 26/89 67 
 Transfused for an acute stressor 127/247|| 51 16/52 13 111/195 87 
Transfusions in the 12 mo prior to enrollment       
 Transfused at least once 92/254 36 5/55 87/199 44 
 Median hemoglobin (range) 8.1 (5.5-11)  8.8 (7.4-9)  8 (5.5-11)  
 Not transfused 162/254 64 50/55 91 112/199 56 
 Median hemoglobin (range) 9.6 (6.3-14.2)  9.5 (8-11.2)  9.6 (6.3-14.2)  
Number of patients (n/N with known data)%Amish patients%Non-Amish patients%
Transfusion status at enrollment*       
 Historically on regular transfusions 79/198 40 32/46 70 47/152 31 
 Currently on regular transfusions 23§/198 12 0/46 23/152 15 
 Intermittent transfusions only 56/198 28 6/46 13 50/152 33 
 Never transfused 34/198 17 4/46 30/152 20 
 Historical transfusions with unknown transfusion frequency 6/198 4/46 2/152 
Hemolytic triggers       
 Presumed infections 137/228|| 60 41/53 30 96/175 70 
 Stress 59/207|| 29 36/54 61 23/153 39 
 Pregnancy 20/39 51 13/41 33 26/89 67 
 Transfused for an acute stressor 127/247|| 51 16/52 13 111/195 87 
Transfusions in the 12 mo prior to enrollment       
 Transfused at least once 92/254 36 5/55 87/199 44 
 Median hemoglobin (range) 8.1 (5.5-11)  8.8 (7.4-9)  8 (5.5-11)  
 Not transfused 162/254 64 50/55 91 112/199 56 
 Median hemoglobin (range) 9.6 (6.3-14.2)  9.5 (8-11.2)  9.6 (6.3-14.2)  

N, total number of patients with known data; n, number of patients.

*

The sample size is 202 because 53 children age 5 years and younger were not included in this analysis.

Historically received regular transfusions is defined as not receiving regular transfusions in the 12 months before enrollment and .6 transfusions per year before the 12-month period before enrollment.

Currently receiving regular transfusions is defined as receiving $6 transfusions over the last 12-month period before enrollment.

§

Twenty-three patients were receiving regular transfusions at the time of enrollment; 17 had had a splenectomy.

||

The denominators are less than 254 because some patients had unknown data for presumed infections, stress, or transfusion for an acute stressor.

Thirty-nine female patients had at least 1 pregnancy.

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