Changing causes of death in Pennsylvania's hemophiliacs 1976 to 1991: impact of liver disease and acquired immunodeficiency syndrome [letter]

Eyster, M. Elaine; Jh, Schaefer; Ragni, MV; Gorenc, TJ; Shapiro, S; Cutter, S; Mk, Kajani; Abrams, J; Barron, LE; Odenwelder, A

doi:10.1182/blood.v79.9.2494.2494

Cited by 19 publications

(6 citation statements)

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“…Moreover, it is unclear whether HCV infection by itself shortens the normal individual’s life [ 29]. Even so, continuous use of IP/VHP concentrates has been associated with increased risk of liver disease and liver failure among HCV+ patients [ 30]. We therefore estimated that 0%–5% of the HCV+ users of IP/VHP products experience liver failure, with a midrange figure of 2.5%.…”

Section: Resultsmentioning

confidence: 99%

Cost‐effectiveness analysis of alternative factor VIII products in treatment of haemophilia A

1999

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Manufactured factor VIII (FVIII) concentrates of varying purity are available for managing patients with haemophilia A. This study is a cost-effectiveness analysis of ultra-high purity and recombinant (UHP/R) FVIII products relative to intermediate and very-high purity (IP/VHP) preparations. Because the societal (including research and development) costs of FVIII products are unknown and product prices vary with market conditions, we conducted the analysis with treatment cost as a variable quantity. We estimated the largest price premium that could be paid for a UHP/R concentrate relative to an IP/VHP concentrate such that the UHP/R product is the more cost-effective preparation. In the analysis haemophilic patients were assumed to be seropositive for human immunodeficiency virus, seropositive for hepatitis C (HCV), or at risk for seroconversion of hepatitis A (HAV) or hepatitis B (HBV). The results showed that the maximum cost-effective UHP/R price premium is essentially zero if the patient is only at risk of HAV or HBV infection, positive but small for the base-case HCV+ patient, and positive and large for the base-case HIV+ patient having a short life expectancy. Thus UHP/R preparations are not uniformly more cost-effective than IP/VHP products across the spectrum of haemophilic patients' health problems, and the relative cost-effectiveness of the two classes of prepared FVIII products is sensitive to product prices. The methodology employed here can be used in other circumstances where multiple treatments exist for illnesses for which there are significant multiple comorbidities or health risks.

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Section: Resultsmentioning

confidence: 99%

Cost‐effectiveness analysis of alternative factor VIII products in treatment of haemophilia A

1999

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“…Chronic HCV and HIV remain major health concerns in those with haemophilia, especially in patients who received factor transfusions prior to available screening tests for HCV and HIV and widespread use of viral inactivated clotting factors [8–10]. Because of the increased bleeding risk associated with liver biopsy, despite a high prevalence of HCV, there are little data on the spectrum of liver histology of HCV and the impact of HIV coinfection in this population.…”

Section: Discussionmentioning

confidence: 99%

“…This was particularly true in those with haemophilia as patients who were treated with non‐viral‐inactivated clotting factors have a prevalence of HCV of 90% [3–5]. Because of the similar mode of transmission, human immunodeficiency virus (HIV) is also common in those with haemophilia [6,7] and coinfection with HIV/HCV is now a cause of significant morbidity and mortality in this population [8–10]. With improvements in anti‐HCV therapy, for both those with [11–13] and without HIV [14–16], accurate assessment of liver disease has become increasingly important.…”

Section: Introductionmentioning

confidence: 99%

Percutaneous liver biopsy in adult haemophiliacs with hepatitis C virus: safety of outpatient procedure and impact of human immunodeficiency virus coinfection on the spectrum of liver disease

Sterling¹,

Lyons²,

Stravitz³

et al. 2007

Haemophilia

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Both HCV and HIV are common in haemophiliacs previously treated with non-viral-inactivated clotting factor concentrates. Because of increased bleeding risks, little data are available on the safety of percutaneous outpatient liver biopsy (LBx) and impact of HIV coinfection in this population. This study aims at reporting our experience with percutaneous LBx in a cohort of haemophiliacs infected with HCV and describe the spectrum of disease and impact of HIV coinfection. A retrospective review of consecutive patients with haemophilia and HCV who underwent percutaneous LBx was performed. All patients were positive for HCV RNA by commercial assay and received factor concentrate prior to biopsy. A total of 29 male patients (mean age 36, 24 haemophilia A, five haemophilia B, and 44% coinfected with HIV) underwent successful outpatient percutaneous LBx without bleeding complication. Histologic activity index was 6.44 with advanced fibrosis (bridging fibrosis/cirrhosis) in 31%. When patients were stratified by HIV positive (n = 13) vs. HIV negative (n = 16), coinfected patients had higher fibrosis scores and higher proportion advanced fibrosis (54% vs. 12%; P = 0.0167) with no differences in age, demographic or other laboratory parameters. Multivariate logistic regression found that HIV positivity was independently associated with advanced fibrosis (OR = 3.7; 95% CI: 1.17-11.8; P = 0.026). Outpatient percutaneous LBx can be safely performed in patients with haemophilia. Despite similar age, HIV coinfection was an independent predictor of advanced fibrosis. These data support the hypothesis that HIV accelerates fibrosis progression in those coinfected with HCV and highlights the importance of liver histology in this population.

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“…2 Unfortunately, exposure to human immunodeficiency virus (HIV) and hepatitis C virus (HCV) through contaminated blood products in the 1980s led to a sharp increase in mortality. [4][5][6][7][8][9][10] Through the use of viral inactivation techniques, as well as the introduction of recombinant factor replacement products, the transmission of both HIV and HCV has been halted since 1992. 2 From 1999 onward, hemophilia treatment in the Netherlands was gradually centralized and quality criteria were introduced in comprehensive hemophilia treatment centers.…”

Section: Introductionmentioning

confidence: 99%

Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001–2018

Hassan

Monahan

Mauser‐Bunschoten

et al. 2021

Journal of Thrombosis and Haemostasis

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Background Treatment of patients with hemophilia has advanced over the past decades, but it is unknown whether this has resulted in a normal life expectancy in the Netherlands. Objective This observational cohort study aimed to assess all‐cause and cause‐specific mortality in patients with hemophilia in the Netherlands between 2001 and 2018 and to compare mortality and life expectancy with previous survival assessments from 1973 onward. Patients/methods All 1066 patients with hemophilia who participated in a nationwide survey in 2001 were followed until July 2018. Results Information on 1031 individuals (97%) was available, of whom 142 (14%) deceased during follow‐up. Compared with the general Dutch male population, mortality of patients with hemophilia was still increased (standardized mortality ratio: 1.4, 95% confidence interval: 1.2–1.7). Intracranial bleeding and malignancies were the most common causes of death. Estimated median life expectancy of patients with hemophilia was 77 years, 6 years lower than the median life expectancy of the general Dutch male population (83 years). Over the past 45 years, death rates of patients with hemophilia have consistently decreased, approaching the survival experience of the general population. Over the past decades, mortality due to human immunodeficiency virus and hepatitis C virus infections has decreased, death due to intracranial hemorrhages has increased, and death due to ischemic heart disease has remained consistently low over time. Conclusions Survival in patients with hemophilia in the Netherlands has improved over time but is still lower than that of the general population.

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Changing causes of death in Pennsylvania's hemophiliacs 1976 to 1991: impact of liver disease and acquired immunodeficiency syndrome [letter]

Cited by 19 publications

References 0 publications

Cost‐effectiveness analysis of alternative factor VIII products in treatment of haemophilia A

Cost‐effectiveness analysis of alternative factor VIII products in treatment of haemophilia A

Percutaneous liver biopsy in adult haemophiliacs with hepatitis C virus: safety of outpatient procedure and impact of human immunodeficiency virus coinfection on the spectrum of liver disease

Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001–2018

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