Acute coronary syndrome management in hemophiliacs: How to maintain balance?: A review

Chen, Hao; Sha-ning, Yang

doi:10.1097/md.0000000000033298

Cited by 2 publications

(2 citation statements)

References 57 publications

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“…Elderly hemophiliacs can undergo diagnostic invasive procedures and drugs provided that tailored prophylaxis has been implemented [ 25 , 26 ]. The need of correcting the hemostatic defect using adequate replacement therapy before any anticoagulant therapy and/or invasive procedures is strongly recommended in acute coronary syndrome management [ 27 ].…”

Section: Replacement Therapy With Fviii/ix Concentrates On People Wit...mentioning

confidence: 99%

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Morfini,

Agnelli Giacchiello,

Baldacci

et al. 2023

Hematology Reports

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The Medical Directors of nine Italian Hemophilia Centers reviewed and discussed the key issues concerning the replacement therapy of hemophilia patients during a one-day consensus conference held in Rome one year ago. Particular attention was paid to the replacement therapy needed for surgery using continuous infusion (CI) versus bolus injection (BI) of standard and extended half-life Factor VIII (FVIII) concentrates in severe hemophilia A patients. Among the side effects, the risk of development of neutralizing antibodies (inhibitors) and thromboembolic complications was addressed. The specific needs of mild hemophilia A patients were described, as well as the usage of bypassing agents to treat patients with high-responding inhibitors. Young hemophilia A patients may take significant advantages from primary prophylaxis three times or twice weekly, even with standard half-life (SHL) rFVIII concentrates. Patients affected by severe hemophilia B probably have a less severe clinical phenotype than severe hemophilia A patients, and in about 30% of cases may undergo weekly prophylaxis with an rFIX SHL concentrate. The prevalence of missense mutations in 55% of severe hemophilia B patients allows the synthesis of a partially changed FIX molecule that can play some hemostatic role at the level of endothelial cells or the subendothelial matrix. The flow back of infused rFIX from the extravascular to the plasma compartment allows a very long half-life of about 30 h in some hemophilia B patients. Once weekly, prophylaxis can assure a superior quality of life in a large severe or moderate hemophilia B population. According to the Italian registry of surgery, hemophilia B patients undergo joint replacement by arthroplasty less frequently than hemophilia A patients. Finally, the relationships between FVIII/IX genotypes and the pharmacokinetics of clotting factor concentrates have been investigated.

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Section: Replacement Therapy With Fviii/ix Concentrates On People Wit...mentioning

confidence: 99%

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Morfini,

Agnelli Giacchiello,

Baldacci

et al. 2023

Hematology Reports

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“…3 The primary challenge in averting and managing cardiovascular and thrombotic diseases in people with haemophilia is the restricted utilization of anticoagulants and antiplatelet medications owing to the inherent bleeding risk. 4 While these pharmacological agents are routinely used to prevent and treat thrombotic and cardiovascular diseases, their dosage and administration require re-evaluation in people with haemophilia owing to the heightened risk of bleeding. The ADVANCE group published a modified Delphi consensus statement that provides detailed guidance for selecting antiplatelet drugs and determining trough levels of clotting factor concentrates.…”

Section: Introductionmentioning

confidence: 99%

Haemophilia and cardiovascular disease in Japan: Low incidence rates from ADVANCE Japan baseline data

Nagao,

Chikasawa,

Sawada

et al. 2023

Haemophilia

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IntroductionWith the increasing life expectancy of people with haemophilia, the risk of cardiovascular disease (CVD) and thrombotic events has become a growing concern. Longitudinal studies on the incidence and risk factors of CVD in this population are limited, and optimal prevention and treatment strategies are yet to be established.AimThis study aimed to present the baseline data of a prospective longitudinal study focusing on a subset of Japanese patients with haemophilia, specifically investigated the incidence, risk factors and treatment modalities for CVD and thrombotic diseases in people aged 40 years in Japan over 10 years through the ADVANCE Japan study.MethodsThe ADVANCE Japan study is a prospective multicentre cohort study involving 600 adult individuals with haemophilia A/B aged 40 years in Japan. The primary endpoint was the incidence of CVD, with secondary endpoints encompassing anticoagulant use, mortality rates, and comparison with the general population.ResultsBaseline data from the 600 participants revealed that thrombotic events occurred in 13 individuals (2.2%), mostly in those with haemophilia A. Atrial fibrillation was observed in 11 participants (1.8%). Hypertension and dyslipidaemia were identified as the prevalent risk factors. Various prophylactic treatments were employed, and no severe bleeding events were observed during the study period.ConclusionThis study provides vital baseline data for a 10‐year prospective investigation of CVD and thrombotic disease risk in people with haemophilia. These findings will contribute to refining prevention and treatment approaches and improving patients’ quality of life.

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Acute coronary syndrome management in hemophiliacs: How to maintain balance?: A review

Cited by 2 publications

References 57 publications

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Haemophilia and cardiovascular disease in Japan: Low incidence rates from ADVANCE Japan baseline data

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