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Blood Transfusion
Other literature type . 2019
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Blood Transfusion
Article . 2016
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Haemophilia B is clinically less severe than haemophilia A: further evidence.

Authors: Franchini, Massimo; Mannucci, Pier Mannuccio;

Haemophilia B is clinically less severe than haemophilia A: further evidence.

Abstract

Preview Haemophilia A and B are rare bleeding disorders caused by mutations in the genes encoding coagulation factor VIII (FVIII) and factor IX (FIX)1. The prevalence of haemophilia A is 1 in 5,000 males in the general population, while that of haemophilia B is 1 in 40,0001,2. Patients with plasma factor levels 5 IU/dL as having moderate or mild haemophilia, respectively2. Patients with mild haemophilia bleed excessively only after surgery, tooth extractions or major injuries, whereas patients with moderate haemophilia bleed even after relatively minor trauma and those with severe haemophilia bleed spontaneously or after trivial trauma. Severe haemophilia is characterised by limb- or life-threatening symptoms such as haemarthrosis, soft-tissue haematoma, retroperitoneal and intracerebral haemorrhage and post-surgical bleeds. Complications from recurrent joint bleeding and soft-tissue haematomas include severe arthropathy, muscle contractures and pseudotumours, leading to chronic pain and disability that often warrant the intervention of the orthopaedic surgeon3-5. All these complications can be effectively prevented by the regular intravenous infusion of plasma-derived or recombinant products containing the deficient coagulation factor, and this prophylactic regimen is the mainstay of modern haemophilia care. [...]

Keywords

Factor IX, Factor VIII, Mutation, Prevalence, Humans, Hemophilia A, Hemophilia B, Severity of Illness Index

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
14
Top 10%
Average
Average
gold