Haemophilia A (Factor VIII Deficiency)
ثبت نشده
چکیده
This is a bleeding disorder caused by deficiency of clotting factor VIII. The vast majority of cases are inherited but acquired forms do exist, largely in older patients, due to autoantibodies directed against factor VIII or haematological malignancy. [2] Severity of disease depends upon levels of remaining factor activity, with normal range expressed as 50-200% (refer to local laboratory for reference range):
منابع مشابه
Combined Factor V and VIII Deficiency
This review summarizes current data on the pathomechanisms and new genetic findings of combined factor V and VIII deficiency (CF5F8D). Congenital haemorrhagic disorders characterized by deficiency of two clotting factors comprise an interesting group. Among dual coagulation disorders, CF5F8D is the most common type. For the first time combined factor V and VIII deficiency (F5F8D) was reported b...
متن کاملRare diseases treated by plasma proteins
When plasma is separated or recovered from a blood donation or when plasma is collected by plasmapheresis the end result is not just a fluid which can be used in the treatment of many medical conditions but a raw material which can be used for the production of specific life saving or life enhancing medications for a variety of conditions. These conditions include Haemophilia A and Haemophilia ...
متن کاملIdentification of carriers of haemophilia by polymerase chain reaction.
The gene for the coagulation protein factor VIII contains several common restriction fragment length polymorphisms which can be used to analyse the pattern of inheritance of factor VIII alleles within families. This can be exploited to identify carriers of haemophilia, an X-linked inherited disorder characterised by deficiency of factor VIII. In this study the polymerase chain reaction was used...
متن کاملUtilization of care in haemophilia: a resource-based method for cost analysis from the Haemophilia Utilization Group Study (HUGS).
The Haemophilia Utilization Group Study (HUGS) was created 10 years ago to examine the annual utilization and cost of haemophilia-related healthcare services. Retrospective chart reviews for 336 patients with haemophilia A receiving treatment in one of five comprehensive haemophilia treatment centres (HTCs) during 1995 were completed through interview of the provider. This method provided adequ...
متن کاملGenotyping of Intron 22 and Intron 1 Inversions of Factor VIII Gene Using an Inverse-Shifting PCR Method in an Iranian Family with Severe Haemophilia A
Abstract Background: Haemophilia A (HA) is an X-linked bleeding disorder caused by the absence or reduced activity of coagulation factor VIII (FVIII). Coagulation factors are a group of related proteins that are essential for the formation of blood clots. The aim of this study was to genotype the coagulation factor VIII gene mutations using Inverse Shifting PCR (IS-PCR) in an Iranian family ...
متن کاملLymphocyte subset ratios and factor VIII usage in haemophilia.
Type and quantity of replacement treatment, together with haematological and immunological parameters were determined in 37 boys with severe haemophilia A and 41 children with other bleeding disorders. The quantity of factor VIII concentrate given to boys with severe haemophilia A (mean U/year) showed a significant inverse correlation with total white cell counts, lymphocyte counts, platelet co...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2017