Activity of blood coagulation factor XIII as a prognostic indicator in patients with Henoch-Schönlein purpura. Efficacy of factor XIII substitution.
Determination of coagulation Factor XIII (F XIII)-related parameters in 21 patients with Henoch-Schönlein purpura documented a significant decrease of F XIII activity as well as of the F XIII-related antigenic determinants. Subgroup analysis with regard to the clinical symptoms showed an even further decrease of these parameters in patients with gastrointestinal complications. Stimulated by these findings a substitution therapy with a F XIII concentrate was initiated in those patients whose F XIII activity in plasma remained low and who developed severe abdominal pain accompanied by persisting gastrointestinal bleeding. This therapeutic approach not only corrected the laboratory data, but more important led to a cessation of pain and bleeding. A rapid decrease of F XIII levels after transfusion below 40 U/ml was indicative of relapse of abdominal symptoms, while increasing values were associated with the recovery of the patients.


CONCLUSIONS
F XIII activity determinations appear to have a predictive value in patients with Henoch-Schönlein purpura, and the administration of F XIII concentrates may contribute to the improvement of gastrointestinal complications.
Kamitsuji
H
H
Department of Pediatrics, Nara Medical University, Japan.
Tani
K
K
Yasui
M
M
Taniguchi
A
A
Taira
K
K
Tsukada
S
S
Iida
Y
Y
Kanki
H
H
Fukui
H
H
D002648:Child; D002675:Child, Preschool; D005176:Factor XIII / Q000378:metabolism / Q000627:therapeutic use*; D005177:Factor XIII Deficiency / Q000097:blood / Q000628:therapy*; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood / Q000628:therapy*; D007223:Infant; D011379:Prognosis
D005176:Factor XIII