و قسمت مختلف حلاليت لخته در استان سيستان و بلوچستان XIII شيوع كمبود فاكتور

Prevalence of factor XIII deficiency and abnormal clot sohubility test in Sistan and Baluchestan province, Iran

Introduction: Factor XIII deficiency is a very rare bleeding disorder. It ʹs prevalence is estimated to be I in 1-2 million. This study was carried out to measure the prevalence of deficiency and it ʹs clinical picture in Sistan and Baluchestan province with a population of 1.5 million and a very high rate of consanguinity. Methods: During 1997-2002 all patients admitted in or referred to our hospital (which is the only center• in the province with sub-specialty for bleeding disorders) with bleeding complaints, delayed wound healing, delayed umbilical bleeding or recurrent abortions and had normal routine coagulation tests, were screened by clot solubility test (1% monochloroacetic acid). Since 2001 the diagnosis has confirmed by measurement of plasma factor XIII levels. Results: We were able to detect 44 patients with abnormal clot solubility tests; among them, we performed factor XIII level measurement in 13 patients, which all had low factor XIII levels. Most common symptoms were delayed umbilical bleeding (84%) echymosis and hematoma (69%) and delayed wound healing (66%). Intracranial bleeding occurred in 4 patients with abnormal clot solubility, resulted in 1 case of death and another case of hemiplegia. There was no patient referred for recurrent abortions. Conclusion: The prevalence of factor XIII deficiency is very high in this area and is the second most common congenital bleeding disorder related to coagulation factor deficiencies in this province (17%). DNA analysis studies are needed to explain this, finding.