OBJECTIVE: This is a retrospective analysis of the clinical and laboratory features of 17 cases of @entity19434 that were followed in tertiary care hospitals in Riyadh, Kingdom of Saudi Arabia, over 20 years. Cases were referred to these hospitals from other health care centers in the country. METHODS: We performed a retrospective analysis of 17 cases of @entity19434 comprising 11 males and 6 females, who were seen over a period of 20 years (1978-1998) in Riyadh, Kingdom of Saudi Arabia. Data variables including age, sex, origin, clinical presentation, @entity548 time, prothrombin time, partial thromboplastin time, factor XIII screening and assay, hemoglobin, and platelet count were collected and analyzed. The diagnosis of @entity19434 was made by @entity1133 clot lysis test alone in one @entity1 and @entity1133 clot lysis test in combination with factor XIII quantitative assay in 16 @entity1 . RESULTS: Eleven @entity1 were males and 6 were females. Median age at the time of diagnosis was 9 years (3-29 years). Ten @entity1 (59%) had a family history of excessive @entity548 . Presenting symptoms included ecchymosis and recurrent @entity1410 in 12 @entity1 (71%), @entity548 after circumcision in 6 male @entity1 (55%), umbilical stump @entity548 in 7 (41%), poor wound healing and keloids in 3 @entity1 (18%), and @entity510 in 3 @entity1 (18%). Other manifestations included cephalohematoma, abortion, @entity5869 , and intraperitoneal @entity548 (one @entity1 each). Laboratory evaluation revealed a normal prothrombin time, partial thromboplastin time, @entity548 time and platelet count in all @entity1 . Factor XIII screening test was positive in all 17 @entity1 tested and assay for factors XIII was <0.06 U/ml in 16 @entity1 tested. CONCLUSION: Our data confirms that @entity19434 is a @entity3459 characterized by variable @entity548 manifestations but consistent laboratory findings. The occurrence of keloid in our @entity1 group may reflect the poor quality of the clotting, associated with loss of tensile strength of fibrin polymers, caused by @entity19434 and leading to abnormally large scar formation.

Clinical and laboratory features of XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity548 :: ('MESH:D006470', 'Disease') :: ['bleeding'] @entity1133 :: ('MESH:D014508', 'Chemical') :: ['urea'] @entity510 :: ('MESH:D013345', 'Disease') :: ['intracranial bleeding'] @entity5869 :: ('MESH:D000037', 'Disease') :: ['abruptio placenta'] @entity3459 :: ('MESH:D025861', 'Disease') :: ['rare bleeding disorder'] @entity1410 :: ('MESH:D006406', 'Disease') :: ['hematomas'] @entity19434 :: ('MESH:D005177', 'Disease') :: ['factor XIII deficiency']
@entity19434 :: (MESH:D005177,Disease) :: ['congenital factor XIII deficiency']
