The Czech National Haemophilia Programme (CNHP) is a non-interventional multicentre prospective study which monitors inherited haemorrhagic disorders: haemophilia, von Willebrand's disease and other haemorrhagic disorders.
start of the project: January 2010
The CNHP registry – a registry of haemorrhagic conditions within the Czech National Haemophilia Programme (CNHP) – has been created in order to keep records on patients with inherited haemorrhagic conditions: haemophilia, von Willebrand's disese and other congenital haemorrhagic coagulopathies. The CNHP registry was established in May 2011 as a continuation of the HemIS registry (the primary aim of which was to monitor the numbers of haemophilia patients).
Haemophilia is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation. Like most recessive sex-linked, X chromosome disorders, haemophilia is more likely to occur in males than females. Females are almost exclusively asymptomatic carriers of the disorder.
Von Willebrand's disease is the most common hereditary coagulation abnormality. It arises from a deficiency or abnormal structure of von Willebrand factor (vWF). There are three types of hereditary vWD, (Type I, Type II Type III). Within the three inherited types of vWD, there are various subtypes. The condition affects both men and women.
Other congenital haemorrhagic conditions are very rare, usually arising from decreased activity of a coagulation factor or dysproteinaemia. These inherited conditions are mostly autosomally recessive. The bleeding severity depends on how much the coagulation activity of a factor has been affected; in less severe forms, unusual bleeding can be discovered during a surgical intervention or after an injury.
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