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Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Schönlein purpura. Japanese
H Fukui1, H Kamitsuji, T Nagao
1Dept. of Pediatrics, Nara Medical College, Japan.
Insights
Decreased coagulation factor XIII (F XIII) levels correlate with Henoch-Schönlein purpura (HSP) severity, especially abdominal symptoms. F XIII concentrate treatment improved symptoms in a controlled study, confirming its efficacy.
Area of Science:
- Pediatrics
- Hematology
- Immunology
Background:
- Henoch-Schönlein purpura (HSP) is a common vasculitis in children.
- Clinical manifestations include arthral, abdominal, and renal symptoms.
- The role of coagulation factor XIII (F XIII) in HSP pathogenesis is not fully understood.
Purpose of the Study:
- To investigate the correlation between coagulation factor XIII (F XIII) activity and the clinical severity of Henoch-Schönlein purpura (HSP).
- To evaluate the efficacy of F XIII concentrate in treating moderate HSP cases.
Main Methods:
- Fifty-six children with HSP had their clinical symptoms scored and F XIII activity measured.
- A controlled study compared F XIII concentrate treatment versus no treatment in 24 children with moderate HSP.
Main Results:
- A significant negative correlation was observed between F XIII levels and HSP symptom severity scores, particularly abdominal symptoms.
- Children treated with F XIII concentrate showed remarkable symptom improvement within three days compared to the non-treated group.
- Clinical symptom scoring proved useful in assessing F XIII concentrate efficacy.
Conclusions:
- Reduced F XIII activity is linked to increased clinical severity in Henoch-Schönlein purpura (HSP).
- F XIII concentrate administration is an effective therapeutic option for moderate HSP.
- Clinical scoring systems are valuable tools for monitoring HSP and treatment response.
Abstract:
Arthral, abdominal and renal symptoms in Henoch-Schönlein purpura (HSP) were scored. Coagulation factor XIII (F XIII) activity was determined in fifty-six children with HSP and the correlation with the severity score of the clinical symptoms was investigated. As a result, it was found that the decrease in F XIII level was correlated with the severity score of clinical symptoms, particularly abdominal symptoms. Based on the results, a controlled study was performed in 24 cases with moderate symptoms divided into a group treated with F XIII concentrate and a non-treated group to investigate clear-cut efficacy as a next study. In three days after the administration the symptoms were improved remarkably in accordance with the increase of F XIII level compared with non-treated group and scoring of clinical symptoms was confirmed to be useful for assessing the application of the F XIII concentrate to HSP.