Table 2.
Prevalence of Congenital Hemolytic Disorders in Denmark in 2000, 2007 and 2015
| Prevalence per 100,000 Persons [95% CI] | ||||
|---|---|---|---|---|
| 2000 | 2007 | 2015 | ||
| Alpha Thalassemia | ||||
| Alpha Thalassemia Trait | ||||
| All | 0.47 [0.30; 0.69] | 6.76 [6.08; 7.48] | 19.22 [18.10; 20.40] | |
| Age <20 | 0.55 [0.22; 1.14] | 7.85 [6.42; 9.51] | 22.58 [20.08; 25.29] | |
| Age 20–50 | 0.76 [0.45; 1.21] | 10.84 [9.53; 12.29] | 31.31 [29.06; 33.69] | |
| Age >50 | 0.00 [0.00; 0.22] | 0.97 [0.58; 1.54] | 3.60 [2.83; 4.52] | |
| HbH Disease | ||||
| All | 0.06 [0.01; 0.16] | 0.28 [0.15; 0.45] | 0.74 [0.53; 1.00] | |
| Age <20 | 0.00 [0.00; 0.29] | 0.30 [0.08; 0.77] | 1.06 [0.58; 1.79] | |
| Age 20–50 | 0.13 [0.03; 0.37] | 0.44 [0.21; 0.81] | 1.09 [0.71; 1.61] | |
| Age >50 | 0.00 [0.00; 0.22] | 0.05 [0.00; 0.30] | 0.15 [0.03; 0.43] | |
| Alpha Thalassemia NOS | ||||
| All | 0.21 [0.10; 0.37] | 0.42 [0.27; 0.63] | 0.90 [0.67; 1.18] | |
| Age < 20 | 0.32 [0.09; 0.81] | 1.05 [0.57; 1.76] | 2.51 [1.73; 3.52] | |
| Age 20–50 | 0.21 [0.07; 0.50] | 0.27 [0.10; 0.58] | 0.70 [0.40; 1.13] | |
| Age > 50 | 0.12 [0.01; 0.42] | 0.16 [0.03; 0.47] | 0.10 [0.01; 0.35] | |
| Beta Thalassemia | ||||
| Beta Thalassemia Minor | ||||
| All | 4.45 [3.90; 5.05] | 15.44 [14.41; 16.52] | 34.91 [33.39; 36.49] | |
| Age <20 | 7.13 [5.73; 8.76] | 24.91 [22.30; 27.73] | 51.77 [47.95; 55.80] | |
| Age 20–50 | 5.44 [4.53; 6.46] | 19.12 [17.36; 21.01] | 47.65 [44.86; 50.56] | |
| Age >50 | 1.11 [0.67; 1.73] | 4.11 [3.24; 5.14] | 9.93 [8.61; 11.39] | |
| Beta Thalassemia Intermedia | ||||
| All | 0.02 [0.00; 0.10] | 0.09 [0.03; 0.21] | 0.44 [0.29; 0.65] | |
| Age <20 | 0.08 [0.00; 0.44] | 0.37 [0.12; 0.87] | 1.06 [0.58; 1.79] | |
| Age 20–50 | 0.00 [0.00; 0.16] | 0.00 [0.00; 0.16] | 0.44 [0.21; 0.80] | |
| Age >50 | 0.00 [0.00; 0.22] | 0.00 [0.00; 0.20] | 0.05 [0.00; 0.27] | |
| Beta Thalassemia Major | ||||
| All | 0.02 [0.00; 0.10] | 0.31 [0.18; 0.50] | 0.69 [0.49; 0.94] | |
| Age <20 | 0.08 [0.00; 0.44] | 0.97 [0.52; 1.66] | 1.90 [1.23; 2.81] | |
| Age 20–50 | 0.00 [0.00; 0.16] | 0.18 [0.05; 0.45] | 0.57 [0.30; 0.97] | |
| Age >50 | 0.00 [0.00; 0.22] | 0.00 [0.00; 0.20] | 0.05 [0.00; 0.27] | |
| Thalassemia NOS | ||||
| All | 1.56 [1.24; 1.93] | 2.44 [2.04; 2.89] | 3.29 [2.83; 3.79] | |
| Age <20 | 3.41 [2.46; 4.59] | 5.39 [4.21; 6.78] | 7.75 [6.32; 9.41] | |
| Age 20–50 | 1.32 [0.89; 1.87] | 2.12 [1.57; 2.82] | 2.84 [2.19; 3.62] | |
| Age >50 | 0.53 [0.24; 1.00] | 0.70 [0.37; 1.20] | 0.92 [0.56; 1.44] | |
| Other Defined Congenital | ||||
| Sickle Cell Trait | ||||
| All | 0.71 [0.50; 0.98] | 2.13 [1.76; 2.55] | 8.11 [7.38; 8.89] | |
| Age <20 | 0.79 [0.38; 1.46] | 2.24 [1.51; 3.20] | 6.92 [5.57; 8.49] | |
| Age 20–50 | 1.19 [0.79; 1.72] | 3.32 [2.61; 4.16] | 13.67 [12.20; 15.27] | |
| Age >50 | 0.00 [0.00; 0.22] | 0.59 [0.30; 1.06] | 2.68 [2.02; 3.48] | |
| Sickle Cell Disease | ||||
| All | 0.53 [0.35; 0.76] | 1.16 [0.89; 1.48] | 2.70 [2.29; 3.17] | |
| Age <20 | 0.95 [0.49; 1.66] | 2.24 [1.51; 3.20] | 7.07 [5.71; 8.66] | |
| Age 20–50 | 0.42 [0.20; 0.78] | 1.24 [0.82; 1.79] | 2.18 [1.62; 2.88] | |
| Age >50 | 0.35 [0.13; 0.76] | 0.27 [0.09; 0.63] | 0.49 [0.23; 0.90] | |
| Hereditary Spherocytosis | ||||
| All | 10.24 [9.40; 11.14] | 13.77 [12.80; 14.79] | 17.72 [16.64; 18.85] | |
| Age <20 | 30.57 [27.60; 33.78] | 39.19 [35.91; 42.70] | 55.03 [51.10; 59.19] | |
| Age 20–50 | 4.71 [3.88; 5.68] | 7.12 [6.07; 8.31] | 9.04 [7.85; 10.36] | |
| Age >50 | 2.86 [2.12; 3.78] | 3.51 [2.71; 4.48] | 3.50 [2.74; 4.41] | |
Notes: Prevalences estimated as the number of living persons assigned the diagnosis at the latest on 1 January each of the years 2000, 2007 and 2015 stratified by age and sex with population denominators derived from census data.
Abbreviations: CI, confidence interval; HbH, hemoglobin H; NOS, not otherwise specified.