 |
 |
|
Home |
Current Issue |
Past Issues |
In the Clinic |
ACP Journal Club |
CME |
Collections |
Audio/Video |
Mobile |
Subscribe |
Tools |
Help |
ACP Online
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 December 1995 | Volume 123 Issue 11 | Pages 823-827
Objective: To determine causes of death and mortality rates in patients with hemophilia over a period of 20 years, to assess changes in mortality, and to distinguish between hemophilia-related death and recent death induced by viral infections.
Design: Cohort study of 919 patients followed from January 1986 to June 1992. Results were compared with outcomes of previous follow-up from 1973 to 1986.
Setting: Consecutive national questionnaire surveys on hemophilia, using patient registries of the Netherlands Hemophilia Society and Dutch hemophilia centers.
Patients: 919 males with hemophilia A or B who participated in a national questionnaire survey on hemophilia in 1985. Median duration of follow-up was 6.4 years, which yielded 5753 person-years of follow-up. The mean age at study entry was 30 years (range, 1 to 85 years).
Measurements: Standardized mortality ratios, causes of death, median life expectancy, age-adjusted relative risks associated with the type or severity of hemophilia, presence of inhibitors, prophylaxis, and human immunodeficiency virus infection.
Results: 45 patients (5%) died between January 1986 and June 1992; 22.6 patients had been expected to die. Thus, the overall standardized mortality ratio was 2.0. The overall median life expectancy was 66 years for the cohort studied from 1973 to 1986 and 68 years for the cohort studied from 1986 to 1992. When deaths related to viral infection were excluded, the life expectancy almost equaled that of the general male population. Between 1986 and 1992, 1 patient died of ischemic heart disease compared with the 5.2 who were expected to die of this disease. Infection with HIV was the strongest independent predictor of death (relative risk, 27.5 [95% CI, 5.7 to 132.8]). After adjustment for HIV infection, no other hemophilia-related risk factors were associated with the risk for death.
Conclusions: The acquired immunodeficiency syndrome and hepatitis strongly influence mortality in patients with hemophilia. In the absence of viral infections, the life expectancy of patients with hemophilia would almost equal that of the general male population.
Author and Article Information
From Vrije Universiteit, Amsterdam, the Netherlands; University Hospital Leiden, Leiden, the Netherlands; and the Netherlands Hemophilia Society, Badhoevedorp, the Netherlands.
ARTICLE
Mortality in Patients with Hemophilia: Changes in a Dutch Population from 1986 to 1992 and 1973 to 1986
Acknowledgments: The authors thank the patients who participated in the ongoing national questionnaire survey on hemophilia, conducted for the fourth time in 1992; the Netherlands Hemophilia Society, the Dutch hemophilia treatment centers, and associated physicians for their help in compiling information on patients; Mrs. A.M.T. Borm and Mrs. W. Noteboom for clerical assistance in updating the patient registry and data entry; and Professor J.P. Vandenbroucke for his helpful comments on epidemiologic matters.
Grant Support: By grant 28-2139 from Het Praeventiefonds, the Netherlands.
Requests for Reprints: Frits R. Rosendaal, MD, Department of Clinical Epidemiology, University Hospital Leiden, Building 1, CO-P42, PO Box 9600, 2300 RC Leiden, the Netherlands.
Current Author Addresses: Drs. Triemstra and Van der Ploeg: Department of Medical Psychology, Vrije Universiteit Amsterdam, Van der Boechorststraat 7, 1081 BT Amsterdam, the Netherlands.
This article has been cited by other articles:
|
|
 |
|
  O. Salomon, D. M. Steinberg, N. Koren-Morag, D. Tanne, and U. Seligsohn Reduced incidence of ischemic stroke in patients with severe factor XI deficiency Blood, April 15, 2008; 111(8): 4113 - 4117. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Gailani and T. Renne Intrinsic Pathway of Coagulation and Arterial Thrombosis Arterioscler. Thromb. Vasc. Biol., December 1, 2007; 27(12): 2507 - 2513. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. C. Darby, S. W. Kan, R. J. Spooner, P. L. F. Giangrande, F. G. H. Hill, C. R. M. Hay, C. A. Lee, C. A. Ludlam, M. Williams, and for the UK Haemophilia Centre Doctors' Organisatio Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV Blood, August 1, 2007; 110(3): 815 - 825. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. M. Arnold, J. A. Julian, I. R. Walker, and for the Association of Hemophilia Clinic Directors Mortality rates and causes of death among all HIV-positive individuals with hemophilia in Canada over 21 years of follow-up Blood, July 15, 2006; 108(2): 460 - 464. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. K. MacCallum Markers of Hemostasis and Systemic Inflammation in Heart Disease and Atherosclerosis in Smokers Proceedings of the ATS, April 1, 2005; 2(1): 34 - 43. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Mascitelli and F. Pezzetta Cardiovascular Protection in Premenopausal Women Arch Intern Med, January 10, 2005; 165(1): 120 - 120. [Full Text] [PDF]
|
|
|
|
 |
|
 F. R. Rosendaal Venous Thrombosis: The Role of Genes, Environment, and Behavior Hematology, January 1, 2005; 2005(1): 1 - 12. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Plug, J. G. van der Bom, M. Peters, E. P. Mauser-Bunschoten, A. de Goede-Bolder, L. Heijnen, C. Smit, J. E. F. Zwart-van Rijkom, J. Willemse, and F. R. Rosendaal Thirty years of hemophilia treatment in the Netherlands, 1972-2001 Blood, December 1, 2004; 104(12): 3494 - 3500. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Mascitelli and F. Pezzetta Cardiovascular Protections in Severely Impaired Hemostasis Circulation, July 27, 2004; 110(4): e39 - e39. [Full Text] [PDF]
|
|
|
|
|
|
N. M. Ananyeva, D. V. Kouiavskaia, M. Shima, and E. L. Saenko Intrinsic pathway of blood coagulation contributes to thrombogenicity of atherosclerotic plaque Blood, May 29, 2002; 99(12): 4475 - 4485. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Rasche Haemostasis and thrombosis: an overview Eur. Heart J. Suppl., December 1, 2001; 3(suppl_Q): Q3 - Q7. [Abstract] [PDF]
|
|
|
|
|
|
A. Sramek, J.H.C. Reiber, W.B.J. Gerrits, and F.R. Rosendaal Decreased Coagulability Has No Clinically Relevant Effect on Atherogenesis: Observations in Individuals With a Hereditary Bleeding Tendency Circulation, August 14, 2001; 104(7): 762 - 767. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. W. Kamphuisen, J. C. J. Eikenboom, and R. M. Bertina Elevated Factor VIII Levels and the Risk of Thrombosis Arterioscler. Thromb. Vasc. Biol., May 1, 2001; 21(5): 731 - 738. [Full Text] [PDF]
|
|
|
|
|
|
J. M. Soucie, R. Nuss, B. Evatt, A. Abdelhak, L. Cowan, H. Hill, M. Kolakoski, N. Wilber, and the Hemophilia Surveillance System Project Investi Mortality among males with hemophilia: relations with source of medical care Blood, July 15, 2000; 96(2): 437 - 442. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Santagostino, M. Colombo, D. Cultraro, M. Muca-Perja, A. Gringeri, and P.M. Mannucci High Prevalence of Serum Cryoglobulins in Multitransfused Hemophilic Patients With Chronic Hepatitis C Blood, July 15, 1998; 92(2): 516 - 519. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Tradati, M. Colombo, P.M. Mannucci, M.G. Rumi, C. De Fazio, G. Gamba, N. Ciavarella, A. Rocino, M. Morfini, A. Scaraggi, et al. A Prospective Multicenter Study of Hepatocellular Carcinoma in Italian Hemophiliacs With Chronic Hepatitis C Blood, February 15, 1998; 91(4): 1173 - 1177. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. G. Rumi, E. Santagostino, M. Morfini, A. Gringeri, G. Tagariello, A. Chistolini, P. Pontisso, A. Tagger, M. Colombo, P.M. Mannucci, et al. A Multicenter Controlled, Randomized, Open Trial of Interferon alpha 2b Treatment of Anti-Human Immunodeficiency Virus-Negative Hemophilic Patients With Chronic Hepatitis C Blood, May 15, 1997; 89(10): 3529 - 3533. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 MORTALITY AMONG HEMOPHILIACS Journal Watch (General), December 19, 1995; 1995(1219): 2 - 2. [Full Text]
|
|
Article
