A Review on Hemophilia in Children

Introduction:
The hemophilias are the commonest inherited bleeding
disorder which can lead to chronic disorder and life
long disabilities if not properly managed1. Although
medical literature supports the existence of life long
bleeding disorders along with their familial occurrence
since tenth century, physicians were the helpless
witness of the exsanguinating bleeders of the
hemophiliacs2. Understanding of the patho-physiology
has long been delayed due to complexity of clotting
mechanism. Due to advancement in protein chemistry
and recombinant DNA technology a comprehensive
account of normal coagulation and molecular genetics
of hemophilia have been explored. Application of
restriction fragment length polymorphism (RFLP) for
carrier detection has revolutionized lab process 3. In
the past decades, hemophilia has moved from the
status and fatal heredity disorder to that of a defined
group of disorder in a molecular basis for which safe
and effective treatment is available 4. And hemophilia
is likely to be first common severe genetic disorder to
be cured by gene therapy until then main challenge
remains to overcome the development of inhibitor and
managing patient with inhibitors and in fact it is also a
great challenge for the society to provide proper
management to four fifth of hemophilia, who are living
in developing countries 4.

 

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