[Longtime therapy of congenital factor XIII deficiency using factor XIII concentrate].
Factor XIII was determined by enzymatic and immunochemical methods in 3 patients with congenital factor XIII deficiency. Factor XIII activity measured by trans-glutaminase assay was below 1% of normal value in each of these cases. Immunelectrophoresis determination revealed the absence of the functionally active subunit A, whereas subunit S was only slightly diminished (30 to 50% of the normal value). Substitution with factor XIII concentrate caused a parallel increase of factor XIII activity and subunit A concentration. No uptake of factor XIII activity or of subunit. A by platelets could be demonstrated. Despite discontinuous substitution over a period of six years no antibody against factor XIII activity could be demonstrated in one patient with congenital factor XIII deficiency.
D000906:Antibodies / Q000032:analysis; D002648:Child; D005176:Factor XIII / Q000008:administration & dosage / Q000037:antagonists & inhibitors / Q000627:therapeutic use*; D005177:Factor XIII Deficiency / Q000151:congenital / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008297:Male; D013997:Time Factors
D000906:Antibodies; D005176:Factor XIII
Erfahrungen bei der Langzeitbehandlung des angeborenen Faktor XIII-mangels mit Faktor XIII-Konzentrat