SynthesisJournal of thrombosis and haemostasis : JTH2021
Mortality in congenital hemophilia A - a systematic literature review.
Synthesis in Journal of thrombosis and haemostasis : JTH, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
30 citing papers in PubMed, 1 synthesis or guideline pooled it, 62 citations in OpenAlex.
- Mortality in congenital hemophilia A - a systematic literature review.Journal of thrombosis and haemostasis : JTH · 2021Pooled it
- Treatment of Bleeding Episodes With Efanesoctocog Alfa in Previously Treated Patients With Severe Hemophilia A in the Phase 3 XTEND-1 Study.American journal of hematology · 2025Trial
- Cardiovascular risk scores estimate arterial thrombotic risk in aging adults with hemophilia in the ADVANCE Japan cohort.Blood advances · 2026Article
- Ageing with Haemophilia: Comorbidities in Focus.Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie · 2026Review
- Genomic medicine in hepatology: mechanisms and liver treatment strategies.Molecular medicine (Cambridge, Mass.) · 2025Review
- [Clinical efficacy and safety of Octocog alfa in Chinese patients with hemophilia A: One-year follow-up results from the Antihemophilic Factor Hemophilia A Outcome Database (AHEAD) study].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2025Article
- Comorbidity and Mortality in Men and Women With Haemophilia in Three Nordic Countries-Comparisons to Matched Controls.Haemophilia : the official journal of the World Federation of Hemophilia · 2025Observational
- Epigenetic Aging Signatures in People with Hemophilia.TH open : companion journal to thrombosis and haemostasis · 2025Article
- Predictors of liver disease outcomes in individuals with hemophilia and HCV infection.Blood advances · 2024Article
- Optimizing liver health before and after gene therapy for hemophilia A.Blood advances · 2024Review
- Evaluating Factor VIII Concentrates Using Clot Waveform Analysis.Journal of clinical medicine · 2024Article
- Emicizumab is efficacious in people with hemophilia A with comorbidities aged ≥50 years: analysis of 4 phase III trials.Research and practice in thrombosis and haemostasis · 2024Article
- Personalized Prophylaxis with myPKFiTMedicina (Kaunas, Lithuania) · 2023Observational
- Emicizumab prophylaxis for people with hemophilia A: Waste estimation and the Brazilian perspective.Saudi pharmaceutical journal : SPJ : the official publication of the Saudi Pharmaceutical Society · 2023Article
- Racial and ethnic differences in reported haemophilia death rates in the United States.Haemophilia : the official journal of the World Federation of Hemophilia · 2023Review
- Residual burden of liver disease after HCV clearance in hemophilia: a word of caution in the era of gene therapy.Blood advances · 2023Article
- A Qualitative Study Exploring the Experiences and Perceptions of Patients with Hemophilia Regarding Their Health-Related Well-Being, in Salamanca.Journal of clinical medicine · 2023Article
- Complication Analysis in Korean Patients With Hemophilia A From 2007 to 2019: A Nationwide Study by the Health Insurance Review and Assessment Service Database.Journal of Korean medical science · 2023Article
- A polymer-based systemic hemostat for managing uncontrolled bleeding.Bioengineering & translational medicine · 2023Article
- Article
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Against a background of a rapidly evolving treatment landscape, a contemporary, evidence-based consolidated understanding of mortality in people with congenital hemophilia A (PwcHA) is lacking. This systematic literature review examines the available data on mortality and causes of death in PwcHA to enable a better understanding of fatalities in PwcHA and evaluate the impact of new treatment paradigms on mortality. A systematic literature review of observational studies was conducted by searching Medline, Embase, and clinical trials registries for articles published from January 2010 to March 2020, using the search terms: hemophilia A (HA), mortality, cause of death. Interventional studies, studies not reporting fatalities, and those reporting only on hemophilia B, acquired HA, or mixed other coagulopathies were excluded. Overall, 7818 unique records were identified and 17 were analyzed. Of these, six reported mortality rates and five reported mortality ratios. Mortality generally decreased over time, despite a spike associated with human immunodeficiency virus (HIV)/hepatitis C virus (HCV) infection in the 1980s and 1990s. Mortality was strongly correlated with age and hemophilia severity. People with hemophilia had a raised mortality risk compared with the general population, particularly in severe hemophilia, and when infected with HIV or HCV. Causes of death varied across populations, countries, and time in 15 identified studies; however, incomplete and heterogeneous reporting limits evidence. Hemorrhage, HIV, HCV, and hepatic disease were the leading causes of death. A unified approach to reporting mortality and cause of death is needed to understand mortality in PwcHA as treatments continue to advance.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.