Guidelines on the laboratory diagnosis of congenital bleeding disorders in Pakistan

Congenital bleeding disorders are found in all racial groups and are present worldwide. Among all of them haemophilia A, B and Von Willebrand's disease are the commonest and they are characterized by the low blood levels of factor VIII, IX and Von Willebrand's factor respectively. Severity...

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Bibliographic Details
Published in:Journal of the Pakistan Medical Association 2012-05, Vol.62 (5), p.477-486
Main Authors: Borhany, Munira, Shamsi, Tahir, Moiz, Bushra, Hasan, Khalid, Hashmi, Khalid Zafar, Ayyub, Muhammad, Ali, Nadir, Amanat, Nisar Ahmed Samina, Zafar, Tahira
Format: Article
Language:English
Subjects:
Blood Coagulation Disorders - congenital
Blood Coagulation Disorders - diagnosis
Blood coagulation factor VIII
Blood Coagulation Tests
Blood diseases
Diagnosis
Humans
Pakistan
Physiological aspects
Practice guidelines (Medicine)
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Summary:Congenital bleeding disorders are found in all racial groups and are present worldwide. Among all of them haemophilia A, B and Von Willebrand's disease are the commonest and they are characterized by the low blood levels of factor VIII, IX and Von Willebrand's factor respectively. Severity of bleeding is proportional to the severity of factor deficiency. The diagnosis of bleeding disorders can be complex, and no single diagnostic tests are suitable for all patients. The guideline was developed after reviewing relevant publications, summarizing current understanding of bleeding disorders and classification, and present a consensus diagnostic recommendation based on analysis of the literature and expert opinion. They also suggest an approach for clinical and laboratory evaluation of individuals with bleeding symptoms, history of bleeding or conditions associated with increased bleeding risk. The document summarizes needs for improvement in laboratory testing and quality which is very much needed in Pakistan to make a correct diagnosis, train master trainers, identify complications of bleeding disorders in local population, increase awareness among masses, involve government in haemophilia care, education of patients and their families and health care community. It further enhances the need for research in bleeding disorders, including clinical research to obtain more objective information about bleeding symptoms, advancements in diagnostic and therapeutic tools.
ISSN:0030-9982