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Activity of blood coagulation factor XIII as a prognostic indicator in patients with Henoch-Schönlein purpura. Efficacy of factor XIII substitution. 1987

H Kamitsuji, and K Tani, and M Yasui, and A Taniguchi, and K Taira, and S Tsukada, and Y Iida, and H Kanki, and H Fukui
Department of Pediatrics, Nara Medical University, Japan.

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.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D011695 IgA Vasculitis A systemic non-thrombocytopenic purpura caused by HYPERSENSITIVITY VASCULITIS and deposition of IGA-containing IMMUNE COMPLEXES within the blood vessels throughout the body, including those in the kidney (KIDNEY GLOMERULUS). Clinical symptoms include URTICARIA; ERYTHEMA; ARTHRITIS; GASTROINTESTINAL HEMORRHAGE; and renal involvement. Most cases are seen in children after acute upper respiratory infections. Allergic Purpura,Anaphylactoid Purpura,Henoch Purpura,Henoch-Schoenlein Purpura,Purpura Hemorrhagica,Purpura, Nonthrombocytopenic,Purpura, Schoenlein-Henoch,Rheumatoid Purpura,Schoenlein-Henoch Purpura,Vasculitis, Hemorrhagic,Henoch Schonlein Purpura,Henoch-Schonlein Purpura,Purpura, Nonthrombopenic,Purpura, Schonlein-Henoch,Hemorrhagic Vasculitis,Hemorrhagica, Purpura,Henoch Schoenlein Purpura,Henoch Schonlein Purpuras,Henoch-Schonlein Purpuras,Nonthrombocytopenic Purpura,Nonthrombopenic Purpura,Nonthrombopenic Purpuras,Purpura, Allergic,Purpura, Anaphylactoid,Purpura, Henoch,Purpura, Henoch Schonlein,Purpura, Henoch-Schoenlein,Purpura, Henoch-Schonlein,Purpura, Rheumatoid,Purpura, Schoenlein Henoch,Purpura, Schonlein Henoch,Purpuras, Henoch Schonlein,Purpuras, Henoch-Schonlein,Purpuras, Nonthrombopenic,Purpuras, Schonlein-Henoch,Schoenlein Henoch Purpura,Schonlein Purpura, Henoch,Schonlein Purpuras, Henoch,Schonlein-Henoch Purpura,Schonlein-Henoch Purpuras,Vasculitis, IgA
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005176 Factor XIII A fibrin-stabilizing plasma enzyme (TRANSGLUTAMINASES) that is activated by THROMBIN and CALCIUM to form FACTOR XIIIA. It is important for stabilizing the formation of the fibrin polymer (clot) which culminates the coagulation cascade. Coagulation Factor XIII,Factor XIII Transamidase,Fibrin Stabilizing Factor,Fibrinase,Laki-Lorand Factor,Blood Coagulation Factor XIII,Factor 13,Factor Thirteen,Laki Lorand Factor,Factor XIII, Coagulation,Stabilizing Factor, Fibrin,Transamidase, Factor XIII,XIII, Coagulation Factor
D005177 Factor XIII Deficiency A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis. Deficiency, Factor 13,Deficiency, Factor Thirteen,Deficiency, Factor XIII,Factor 13 Deficiency,Factor Thirteen Deficiency,Deficiencies, Factor 13,Deficiencies, Factor Thirteen,Deficiencies, Factor XIII,Factor 13 Deficiencies,Factor Thirteen Deficiencies,Factor XIII Deficiencies
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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