Allison Ashley-Koch 1, Quanhe Yang 2, and Richard S. Olney 2
Tables
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TABLE 1: Frequency of beta globin gene variants among different ethnic groups in a California newborn population(a)
Asian |
0/207551 |
1/1336 |
0/207551 |
0/207551 |
|
|
Asian Indian |
0/15843 |
1/725 |
1/15843 |
0/15843 |
|
|
Black |
1/700 |
1/14 |
1/4056 |
1/1297 |
1/63885 |
1/63885 |
Hispanic |
1/45622 |
1/183 |
1/729953 |
1/364976 |
1/1459906 |
1/1459906 |
Middle Eastern |
0/21677 |
1/360 |
0/21677 |
0/21677 |
|
|
Native American |
1/16529 |
1/176 |
0/16529 |
0/16529 |
|
|
White |
1/158127 |
1/625 |
1/553447 |
0/1106895 |
|
|
(a) Source: Lorey et al (1996)
(b) HbSS includes HbS/beta 0-thalassemia
TABLE 2: Average annual state-specific mortality rates (age-adjusted) for sickle cell disease per 1,000,000 individuals during 1979-1995
Alabama |
5.5 |
6.3 |
4.7 |
22.16 |
0.04 |
Alaska |
0.5 |
0.2 |
0.9 |
14.33 |
. |
Arizona |
0.8 |
0.9 |
0.6 |
27.13 |
0.02 |
Arkansas |
2.8 |
3.2 |
2.4 |
18.39 |
. |
California |
1.5 |
1.7 |
1.4 |
20.89 |
0.04 |
Colorado |
0.7 |
0.7 |
0.7 |
17.8 |
. |
Connecticut |
1.4 |
1.5 |
1.4 |
17.74 |
0.02 |
Delaware |
2.8 |
3.3 |
2.4 |
16.92 |
. |
D.C. |
14.6 |
15.2 |
14.1 |
23.2 |
. |
Florida |
3.5 |
3.8 |
3.2 |
24.69 |
0.08 |
Georgia |
5.3 |
5.9 |
4.7 |
19.85 |
0.04 |
Hawaii |
0.3 |
0.2 |
0.3 |
7.73 |
0.06 |
Idaho |
. |
. |
. |
. |
. |
Illinois |
2.6 |
2.9 |
2.4 |
17.55 |
0.06 |
Indiana |
1.7 |
1.8 |
1.6 |
21.54 |
0.02 |
Iowa |
0.3 |
0.4 |
0.3 |
18.46 |
0.02 |
Kansas |
1.3 |
1.3 |
1.3 |
22.88 |
0.03 |
Kentucky |
1.3 |
1.3 |
1.2 |
17.68 |
0.04 |
Louisiana |
5.3 |
6.4 |
4.2 |
17.49 |
0.06 |
Maine |
0.1 |
0.1 |
. |
8.76 |
. |
Maryland |
2.7 |
3.0 |
2.5 |
10.89 |
0.02 |
Massachusetts |
0.7 |
0.6 |
0.7 |
13.03 |
0.05 |
Michigan |
2.5 |
2.7 |
2.4 |
18.49 |
0.02 |
Minnesota |
0.3 |
0.4 |
0.3 |
17.66 |
0.01 |
Mississippi |
6.6 |
7.9 |
5.4 |
19.23 |
. |
Missouri |
2.1 |
2.6 |
1.7 |
19.44 |
0.05 |
Montana |
. |
. |
. |
. |
. |
Nebraska |
0.4 |
0.4 |
0.4 |
10.68 |
0.04 |
Nevada |
1.3 |
1.7 |
1.0 |
20.0 |
0.05 |
New Hampshire |
0 |
0.1 |
. |
9.01 |
. |
New Jersey |
2.4 |
2.4 |
2.4 |
17.88 |
0.1 |
New Mexico |
0.4 |
0.4 |
0.5 |
18.55 |
0.08 |
New York |
2.9 |
3.0 |
2.8 |
18.39 |
0.25 |
North Carolina |
4.2 |
4.9 |
3.6 |
19.35 |
0.07 |
North Dakota |
0.1 |
. |
0.2 |
16.19 |
. |
Ohio |
1.9 |
2.2 |
1.7 |
17.61 |
0.07 |
Oklahoma |
1.5 |
1.9 |
1.1 |
19.32 |
0.04 |
Oregon |
0.3 |
0.4 |
0.2 |
14.29 |
0.04 |
Pennsylvania |
1.8 |
1.9 |
1.7 |
19.01 |
0.05 |
Rhode Island |
0.8 |
0.4 |
1.1 |
20.72 |
0.05 |
South Carolina |
6.3 |
6.9 |
5.7 |
21.69 |
0.03 |
South Dakota |
0.2 |
0.2 |
0.2 |
65.78 |
. |
Tennessee |
3.3 |
3.6 |
3.0 |
20.50 |
0.06 |
Texas |
2.1 |
2.2 |
2.0 |
17.78 |
0.04 |
Utah |
0.2 |
0.2 |
0.1 |
27.05 |
. |
Vermont |
. |
. |
. |
. |
. |
Virginia |
2.2 |
2.3 |
2.0 |
11.59 |
0.06 |
Wshington |
0.5 |
0.5 |
0.5 |
17.54 |
0.03 |
West Virginia |
0.7 |
1.0 |
0.5 |
22.95 |
. |
Wisconsin |
1.0 |
1.5 |
0.6 |
20.83 |
0.01 |
Wyoming |
0.6 |
0.5 |
0.8 |
76.03 |
0.16 |
TABLE 3: Incidence of sickle cell complications in the US , based on genotype
Acute Chest Syndrome |
12.8 |
5.2 |
9.4 |
3.9 |
All |
12 |
Acute Chest Syndrome |
26.8 |
10.6 |
|
|
1 year |
11 |
Bacteremia |
6.5 |
5.8 |
|
|
1 year |
11 |
Cerebrovascular Accidents |
0.3 |
0 |
|
|
1 year |
11 |
Cerebrovascular Accidents |
0.61 |
0.17 |
0.10 |
0.11 |
All |
13 |
Hand/Foot Syndrome |
20.0 |
3.2 |
|
|
1 year |
11 |
Pain crisis |
24.0 |
8.5 |
|
|
1 year |
11 |
Splenic sequestration |
6.2 |
0.5 |
|
|
1 year |
11 |
TABLE 4: Median survival of individuals of all ages with sickle cell disease based on genotype and sex (10).
Males with Hb SS |
42 years |
Females with Hb SS |
48 years |
Males with Hb SC |
60 years |
Females with Hb SC |
68 years |
TABLE 5: Sensitivity and specificity of tests to detect sickle cell disease
Cellulose Acetate/Citrate Agar Electrophoresis |
93.1 % |
99.9 % |
2 |
Isoelectric Focusing |
100 % |
100 % |
2 |
High-Performance Liquid Chromatography |
100 % |
100 % |
18 |
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