factor xiii deficiency/urea

BACKGROUND Factor XIII deficiency is a rarely encountered bleeding disorder traditionally identified by clot dissolution in 5 molar urea (urea solubility test). METHODS We report a patient with delayed post-surgical bleeding characteristic of factor XIII deficiency with a normal urea solubility

Clinical and hematological data of 9 cases with factor XIII deficiency is highlighted. The age at first bleed ranged from 3 days of life to 1 year. Seven of these 9 cases had bleeding from the umbilicus, 3 had recurrent subcutaneous and muscle hematomas, while 4 cases had CNS bleeds of which 3

Two patients with factor XIII deficiency, presenting with bleeding disorder since birth, are reported. The condition is rare and is likely to be missed unless clot solubility in 5 M urea is performed as a screening test in all patients with bleeding disorders. A correct diagnosis is essential as

Patients with undiagnosed haemostatic defects seen at The Aga Khan Hospital and Fatimid Blood Transfusion Centre during the period of 7 years (1985-1992) were screened with routine tests including bleeding time (BT), whole blood clotting time (CT), platelet count, activated partial thromboplastin

OBJECTIVE To determine the demographic features and clinical outcome of children with Factor XIII deficiency. METHODS Observational case series. METHODS The Aga Khan University Hospital, Karachi, from January 1996 to December 2006. METHODS Records of all hospitalized pediatric patients with

Severe homozygous factor XIII deficiency was first described in Switzerland, in 1961. At present 14 patients are known here. Nine are of Swiss origin, the others are immigrants from eastern Europe. A 27-year-old woman with many haemorrhages during childhood immigrated to Switzerland and went through

Background: Factor XIII (FXIII) deficiency is an extremely rare bleeding disorder that is commonly due to mutations in the FXIIIA subunit gene (F13A1), and it has been reported to have a prevalence of one per 2 million. We describe a new

Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between

We report the case of a three year old female child with combined occurrence of von Willebrand's disease and factor XIII deficiency, an extremely rare combination. The patient presented with prolonged bleeding following cuts and wounds. Clot solubility test using 5M urea was positive. Platelet

OBJECTIVE This is a retrospective analysis of the clinical and laboratory features of 17 cases of factor XIII deficiency 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

Congenital factor XIII (FXIII) deficiency is associated with a tendency for severe bleeding, a risk for spontaneous abortion, and a high rate of spontaneous intracranial hemorrhage. This phase 1 escalating-dose study was developed to evaluate the safety and pharmacokinetics of a single

BACKGROUND Deficiency of factor XIII (FXIII) is a rare bleeding disorder (RBD) affecting approximately 1 person per 2 million worldwide. Its life-threatening diatheses-umbilical cord bleeding, intracranial hemorrhage and central nervous system bleeding-present a significant challenge in both

Factor XIII is the last factor in the coagulation cascade with unique chemical properties and physiological functions. The history of discovery of factor XIII can be traced back to 1923 when Barkan and Gasper first demonstrated that fibrin clots formed in the presence of calcium ions (Ca2+) were

Deficiency of coagulation factor XIII (FXIII) belongs to the rare bleeding disorders and its incidence is higher in populations with consanguineous marriages. The aims of this study were to characterize patients and relatives from seven families with suspected FXIII deficiency from Pakistan and to

Acquired factor XIII (FXIII) deficiency is a rare and life-threatening condition that is often misdiagnosed or missed completely. A 72-year-old woman presented with symptoms of major unprovoked bleeding but routine coagulation screening tests and platelet count were normal. Low activated FXIII