Abstract
Since 1960 when Duckert and associates<sup>2</sup> observed a familial bleeding disorder caused by a deficiency of Factor XIII, 21 cases of Factor XIII deficiency, involving 10 families, have been reported. A 6-year-old boy with Factor XIII deficiency has been diagnosed and an assay technique with the patient's plasma as deficient substrate has been devised. In vivo survival studies, after transfusion of the patient with Factor XIII, indicate a half life of 5 to 7 days. Factor XIII assays in 50 infants and children from birth to 20 months of age reveal, a significantly lower level in the newborn infant and normal levels by 1 month of age. Factor XIII levels in the parents and siblings were significantly lower than in 20 normal individuals, suggesting an autosomal recessive inheritance of Factor XIII deficiency.
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