Factor XIII deficiency: pathogenic mechanisms and clinical significance.

Abstract

Congenital factor XIII deficiency is a rare disease, but has provided valuable information on the physiological role of factor XIII and the benefit of factor XIII replacement therapy. It could be shown that not only homozygous patients but also heterozygotes are at risk for bleeding complications. Acquired factor XIII deficiency, however, is much more common, and preliminary studies suggest a lack of factor XIII to be an important feature of various diseases. In acute states and severe hemorrhages, replacement therapy with factor XIII concentrates is recommended. Recent progress in assay methods and future clinical studies should help to evaluate the therapeutic potential of factor XIII.

• Authors: R Egbring, A Kröniger, R Seitz
• Journal: Seminars in thrombosis and hemostasis (Semin Thromb Hemost) Vol. 22 Issue 5 Pg. 419-25 ( 1996) ISSN: 0094-6176 [Print] United States
• PMID: 8989826 (Publication Type: Journal Article, Review)

Chemical References

• Autoantibodies
• Epitopes
• Isoantibodies
• Fibrin
• Factor XIII
• Isoniazid

Topics

• Autoantibodies (immunology)
• Autoimmune Diseases (immunology)
• Epitopes (drug effects, immunology)
• Factor XIII (drug effects, immunology, physiology, therapeutic use)
• Factor XIII Deficiency (complications, congenital, genetics, therapy)
• Female
• Fibrin (metabolism)
• Fracture Healing (physiology)
• Hemorrhage (etiology)
• Humans
• Isoantibodies (biosynthesis)
• Isoniazid (adverse effects, pharmacology)
• Male
• Pregnancy
• Pregnancy Complications, Hematologic (etiology)
• Wound Healing (physiology)