Trend with calendar period in mortality rate for deaths involving bleeding or intracranial hemorrhage in males in the United Kingdom with hemophilia A or B who were not infected with HIV, 1977 to 1999
| Calendar period . | Severe . | Moderate/mild . | ||||||
|---|---|---|---|---|---|---|---|---|
| All patients . | Excluding inhibitors* . | All patients . | Excluding inhibitors* . | |||||
| No. of deaths . | Rate per 1000 y† (95% CI) . | No. of deaths . | Rate per 1000 y† (95% CI) . | No. of deaths . | Rate per 1000 y† (95% CI) . | No. of deaths . | Rate per 1000 y† (95% CI) . | |
| Deaths involving bleeding of any type, all ages | ||||||||
| 1977-1984 | 72 | 8.9 (6.6-11.2) | 44 | 5.5 (3.8-7.2) | 46 | 2.5 (1.8-3.3) | 43 | 2.0 (1.4-2.6) |
| 1985-1992 | 41 | 8.3 (5.7-11.0) | 24 | 4.9 (2.9-6.8) | 55 | 2.0 (1.4-2.5) | 52 | 1.6 (1.2-2.1) |
| 1993-1999 | 32 | 5.6 (3.6-7.6) | 29 | 5.6 (3.5-7.7) | 59 | 1.8 (1.3-2.3) | 55 | 1.6 (1.1-2.0) |
| Total deaths/person-years | 145/27 099 | — | 97/23 833 | — | 160/71 651 | — | 150/70 657 | — |
| P for trend | — | .05 | — | .90 | — | .08 | — | .16 |
| Deaths involving intracranial hemorrhage, all ages | ||||||||
| 1977-1984 | 39 | 4.8 (3.1-6.5) | 25 | 3.1 (1.8-4.3) | 26 | 1.4 (0.8-1.9) | 24 | 1.1 (0.7-1.5) |
| 1985-1992 | 24 | 4.8 (2.8-6.8) | 17 | 3.5 (1.8-5.1) | 34 | 1.2 (0.8-1.6) | 33 | 1.1 (0.7-1.4) |
| 1993-1999 | 21 | 3.4 (1.9-4.9) | 19 | 3.5 (1.9-5.1) | 24 | 0.7 (0.4-1.0) | 23 | 0.7 (0.4-0.9) |
| Total deaths/person-years | 84/27 099 | — | 61/23 833 | — | 84/71 651 | — | 80/70 657 | — |
| P for trend | — | .40 | — | .58 | — | .02 | — | .07 |
| Deaths involving intracranial hemorrhage, younger than 5 y‡ | ||||||||
| 1977-1984 | 5§ | 5.5 (2.3-13.1) | 4 | 4.5 (1.7-12.1) | 0 | 0.0 | 0 | 0.0 |
| 1985-1992 | 2‖ | 2.1 (0.5-8.3) | 1 | 1.1 (0.2-7.8) | 2** | 1.4 (0.4-5.8) | 2 | 1.4 (0.4-5.8) |
| 1993-1999 | 4¶ | 5.9 (2.2-15.8) | 4 | 6.7 (2.5-17.9) | 1†† | 0.6 (0.1-4.6) | 1 | 0.7 (0.1-4.7) |
| Total deaths/person-years | 11/2554 | — | 9/2376 | — | 3/3896 | — | 3/3838 | — |
| P for trend | — | .99 | — | .65 | — | .68 | — | .67 |
| Calendar period . | Severe . | Moderate/mild . | ||||||
|---|---|---|---|---|---|---|---|---|
| All patients . | Excluding inhibitors* . | All patients . | Excluding inhibitors* . | |||||
| No. of deaths . | Rate per 1000 y† (95% CI) . | No. of deaths . | Rate per 1000 y† (95% CI) . | No. of deaths . | Rate per 1000 y† (95% CI) . | No. of deaths . | Rate per 1000 y† (95% CI) . | |
| Deaths involving bleeding of any type, all ages | ||||||||
| 1977-1984 | 72 | 8.9 (6.6-11.2) | 44 | 5.5 (3.8-7.2) | 46 | 2.5 (1.8-3.3) | 43 | 2.0 (1.4-2.6) |
| 1985-1992 | 41 | 8.3 (5.7-11.0) | 24 | 4.9 (2.9-6.8) | 55 | 2.0 (1.4-2.5) | 52 | 1.6 (1.2-2.1) |
| 1993-1999 | 32 | 5.6 (3.6-7.6) | 29 | 5.6 (3.5-7.7) | 59 | 1.8 (1.3-2.3) | 55 | 1.6 (1.1-2.0) |
| Total deaths/person-years | 145/27 099 | — | 97/23 833 | — | 160/71 651 | — | 150/70 657 | — |
| P for trend | — | .05 | — | .90 | — | .08 | — | .16 |
| Deaths involving intracranial hemorrhage, all ages | ||||||||
| 1977-1984 | 39 | 4.8 (3.1-6.5) | 25 | 3.1 (1.8-4.3) | 26 | 1.4 (0.8-1.9) | 24 | 1.1 (0.7-1.5) |
| 1985-1992 | 24 | 4.8 (2.8-6.8) | 17 | 3.5 (1.8-5.1) | 34 | 1.2 (0.8-1.6) | 33 | 1.1 (0.7-1.4) |
| 1993-1999 | 21 | 3.4 (1.9-4.9) | 19 | 3.5 (1.9-5.1) | 24 | 0.7 (0.4-1.0) | 23 | 0.7 (0.4-0.9) |
| Total deaths/person-years | 84/27 099 | — | 61/23 833 | — | 84/71 651 | — | 80/70 657 | — |
| P for trend | — | .40 | — | .58 | — | .02 | — | .07 |
| Deaths involving intracranial hemorrhage, younger than 5 y‡ | ||||||||
| 1977-1984 | 5§ | 5.5 (2.3-13.1) | 4 | 4.5 (1.7-12.1) | 0 | 0.0 | 0 | 0.0 |
| 1985-1992 | 2‖ | 2.1 (0.5-8.3) | 1 | 1.1 (0.2-7.8) | 2** | 1.4 (0.4-5.8) | 2 | 1.4 (0.4-5.8) |
| 1993-1999 | 4¶ | 5.9 (2.2-15.8) | 4 | 6.7 (2.5-17.9) | 1†† | 0.6 (0.1-4.6) | 1 | 0.7 (0.1-4.7) |
| Total deaths/person-years | 11/2554 | — | 9/2376 | — | 3/3896 | — | 3/3838 | — |
| P for trend | — | .99 | — | .65 | — | .68 | — | .67 |
Includes all deaths with bleeding/intracranial hemorrhage mentioned on the death certificate or notified by the hemophilia center. HIV-positive patients were excluded from 1985 or date of seroconversion, if later.
— indicates not applicable.
Excluding both deaths and person-years subsequent to the development of inhibitors.
Death rates directly standardized for age to the age distribution of all patients.
All the deaths involving bleeding in patients younger than 5 years involved intracranial hemorrhage.
Hemophilia A at ages: younger than 1 month, 10 months, 2.9 years, 3.9 years (with inhibitor), and 4.7 years.
Hemophilia A at ages: younger than 1 month and 1.7 years (with inhibitor).
Hemophilia A at ages: younger than 1 month (2 children) and 1.0 year. Hemophilia B at younger than 1 month.
Hemophilia A at ages 1.4 and 2 years.
Hemophilia A at younger than 1 month.