0
selected
-
1.
Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan
Amano S, Oka K, Sato Y, Sano C, Ohta R
Journal of clinical medicine. 2022;11(6)
Abstract
Factor XIII (FXIII) deficiency is a rare but serious coagulopathy. FXIII is critical in blood coagulation, and FXIII deficiencies can lead to uncontrolled or spontaneous bleeding. FXIII deficiencies can be congenital or acquired; acquired FXIII deficiency can be categorized as autoimmune and non-autoimmune. Immunological tests to measure FXIII inhibitors are required to diagnose acquired FXIII deficiency; however, appropriate test facilities are limited, which increases the turnaround time of these tests. In the case of critical bleeding, delayed test results may worsen prognosis due to delayed treatment. Here, we report a case of acquired FXIII deficiency, followed by a review of FXIII deficiency cases in Japan. We performed a systematic review to investigate the present conditions of the diagnosis and treatment of FXIII deficiency, including the measurement of FXIII inhibitors in Japan. FXIII inhibitor testing was only performed in 29.7 of acquired FXIII deficiency cases. Clinical departments other than internal medicine and pediatrics were often involved in medical treatment at the time of onset. Therefore, it is important for doctors in clinical departments other than internal medicine and pediatrics to consider FXIII deficiency and perform FXIII inhibitor testing when examining patients with prolonged bleeding of unknown cause or persistent bleeding after trauma.
-
2.
Effect of early administration of coagulation factor XIII on fistula after pancreatic surgery: the FIPS randomized controlled trial
Takeda Y, Mise Y, Ishizuka N, Harada S, Hayama B, Inoue Y, Ishizawa T, Ito H, Takahashi Y, Saiura A
Langenbeck's archives of surgery. 2018
Abstract
PURPOSE The administration of exogenous factor XIII (FXIII) is reportedly effective for fistula closure in patients with a low plasma FXIII level. This study was performed to analyze the effect of early administration of exogenous FXIII on postoperative pancreatic fistula (POPF). METHODS A single-center randomized controlled, open-label, parallel group, superiority trial was conducted from October 2015 to August 2016 in Japan. Patients with POPF and a plasma FXIII level of ≤ 70% on postoperative day 7 were randomly assigned to an early replacement (ER) group or control group in a 1:1 ratio by an independent coordinator using a computer-generated random number table. The ER group received FXIII concentrate the day after randomization, and the control group received no FXIII concentrate within 2 weeks. The primary endpoint was the duration of drain placement from randomization (DDPR). RESULTS Fifty patients were randomized (ER group, 24; control group, 26), and all were analyzed with an intention-to-treat approach. There was no significant difference in the DDPR between the two groups (18 vs. 16 days; hazard ratio, 1.45; 95% confidence interval, 0.813-2.583). No serious harm was reported in either group. CONCLUSION Early administration of exogenous FXIII does not facilitate the healing of POPF. TRIAL REGISTRATION University Hospital Medical Information Network (UMIN) Center (UMIN000019480, http://www.umin.ac.jp ).
-
3.
Efficacy and safety of prophylactic treatment with plasma-derived factor XIII concentrate (human) in patients with congenital factor XIII deficiency
Ashley C, Chang E, Davis J, Mangione A, Frame V, Nugent DJ
Haemophilia. 2015;21((1):):102-8.
Abstract
UNLABELLED Congenital factor XIII (FXIII) deficiency is an extremely rare, potentially life-threatening bleeding disorder. Routine prophylactic management is recommended for individuals with clinically relevant FXIII deficiency. This prospective, multicentre, open-label study evaluated the long-term efficacy and safety of prophylactic infusions of FXIII concentrate (human) 40 IU kg(-1) in patients with congenital FXIII deficiency. FXIII concentrate (human) was administered every 4 weeks for 12 months. Dosing was adjusted to maintain trough FXIII activity levels of 5-20%. Logistical and ethical constraints precluded use of a placebo control group. Annualized incidence of spontaneous bleeding was compared with historical rates; safety was assessed as a secondary objective. Forty-one patients were enrolled and completed the study. The annualized rate for spontaneous bleeding episodes requiring FXIII treatment was 0.000 episodes per patient-year (95% CI: 0.000; 0.097). The study met its primary endpoint: the upper limit of the 95% CI was substantially below the historical rate of 2.5 bleeding episodes per patient-year. Five spontaneous bleeding episodes (involving three patients; none requiring FXIII treatment) and eight trauma-related bleeding episodes (two requiring FXIII treatment) occurred. Five patients had surgery during the study, only one of whom required FXIII treatment for post-surgical bleeding. Most patients (>85%) had trough FXIII activity levels >10%. No patient discontinued treatment due to an adverse event. No adverse events related to thromboembolism or viral transmission were reported. Prophylactic treatment with FXIII concentrate (human) was well tolerated and prevented spontaneous bleeding episodes that were serious enough to require treatment with FXIII-containing product. CLINICAL TRIAL REGISTRATION www.clinicaltrials.gov/ct2/show/NCT00885742. 2014 The Authors. Haemophilia Published by John Wiley & Sons Ltd.
-
4.
Congenital factor XIII deficiency in women: a systematic review of literature
Sharief LA, Kadir RA
Haemophilia. 2013;19((6):):e349-57.
Abstract
Factor XIII (FXIII) deficiency is a rare congenital bleeding disorder. There is a paucity of data in the literature about obstetrics and gynaecological problems in women affected by FXIII deficiency. The aim of this study was to examine gynaecological problems and obstetric complications and outcome in women with congenital FXIII deficiency. An electronic search was performed to identify the published literature on PUBMED, MEDLINE, EMBASE, Journals @OVID and CINAHL Plus databases using the following keywords: 'congenital factor XIII deficiency' AND 'women OR Pregnancy'. A total of 39 relevant articles were found and included in this systematic review; 27 case reports and 12 case series dating from 1964 to 2012. A total of 121 women were identified. Menorrhagia (26%) was the second most common bleeding reported after umbilical bleeding. Ovulation bleeding reported in 8% of women. Among 63 women, 192 pregnancies were reported; of these, 127 (66%) resulted in a miscarriage and 65 (34%) reached viability stage. In 136 pregnancies without prophylactic therapy, 124 (91%) resulted in a miscarriage and 12(9%) progressed to viability stage. Antepartum haemorrhage occurred in 5/65 (8%) pregnancies reaching viability stage while postpartum haemorrhage (PPH) seen in 16 (25%) cases. Women with congenital FXIII deficiency suffer significant bleeding complications. Menorrhagia and ovulation bleeding are common gynaecological problems and more prevalent than reported. Pregnancies in women with FXIII deficiency have a significant risk of miscarriage, placental abruption and PPH if not on prophylaxis treatment. 2013 John Wiley & Sons Ltd.
-
5.
Factor XIII substitution in surgical cancer patients at high risk for intraoperative bleeding
Korte WC, Szadkowski C, Gähler A, Gabi K, Kownacki E, Eder M, Degiacomi P, Zoller N, Devay J, Lange J, et al
Anesthesiology. 2009;110((2):):239-45.
Abstract
BACKGROUND Excessive intraoperative bleeding is associated with significant morbidity and mortality. The authors and others have shown that fibrin monomer allows preoperative risk stratification for intraoperative blood loss, likely due to an imbalance between available factor XIII and prothrombin conversion. The authors hypothesized that the use of factor XIII would delay the decrease of clot firmness in high-risk patients. METHODS The concept was tested in a prospective, randomized, double-blind, placebo-controlled trial in elective gastrointestinal cancer surgery. Patients were randomized to receive factor XIII (30 U/kg) or placebo in addition to controlled standard therapy. RESULTS Twenty-two patients were evaluable for a planned interim analysis. For the primary outcome parameter maximum clot firmness, patients receiving factor XIII showed a nonsignificant 8% decrease, and patients receiving placebo lost 38%, a highly significantly difference between the two groups (P = 0. 004). A reduction in the nonprimary outcome parameters fibrinogen consumption (-28%, P = 0. 01) and blood loss (-29%, P = 0. 041) was also observed in the factor XIII group. Three patients experienced adverse events that seemed unrelated to factor XIII substitution. The trial was stopped early after a planned interim analysis with the primary endpoint reached. CONCLUSIONS This proof of concept study confirms the hypothesis that patients at high risk for intraoperative blood loss show reduced loss of clot firmness when factor XIII is administered early during surgery. Further clinical trials are needed to assess relevant clinical endpoints such as blood loss, loss of other coagulation factors, and use of blood products.
-
6.
Coagulation factor XIII reduces postoperative bleeding after coronary surgery with extracorporeal circulation
Gödje O, Gallmeier U, Schelian M, Grünewald M, Mair H
The Thoracic and Cardiovascular Surgeon. 2006;54((1):):26-33.
Abstract
BACKGROUND One cause of diffuse bleeding after cardiac operations may be a low plasma concentration of coagulation Factor XIII, which is essential for coagulation but is not covered by standard coagulation monitoring. PATIENTS AND METHODS In a prospective, randomized, double blinded study, 2500 units, 1250 units, and a placebo were administered in groups of 25 patients each, immediately after administration of protamine. Postoperative amount of blood loss and blood transfusion was recorded. RESULTS Patients were not statistically different with respect to the course of plasma levels of Factor XIII until administration of the study drug. In all groups Factor XIII fell from preoperative normal values to subnormal values after extracorporeal circulation. After administration of the study drug, Factor XIII increased to 71 %, 85 %, 103 % in the placebo, 1250 units, and 2500 units group, respectively, and these differences were statistically significant ( p < 0. 05). Postoperative blood loss was lowest in the 2500 units group and highest in the placebo group, however this was not significantly different. There was also no significant difference in the amount of blood transfusion. After differentiating all patients according to their post medication Factor XIII level into two groups with levels of < 70 % and > or = 70 %, postoperative blood loss was found to be significantly higher in the < 70 % group as was the amount of blood transfusions. CONCLUSIONS Factor XIII administration reduces postoperative blood loss and the extent of blood transfusion after coronary surgery, however administration is only helpful if plasma levels are below the normal value. Measurement of plasma levels is recommended before Factor XIII substitution.
-
7.
Factor XIII-mediated inhibition of fibrinolysis and venous leg ulcers
Herouy Y, Hellstern MO, Vanscheidt W, Schopf E, Norgauer J
Lancet. 2000;355((9219):):1970-1.
Abstract
Densitometric analysis shows an accelerated healing rate and a significantly diminished lesional fibrinolytic activity in patients with venous leg ulcers treated topically with the fibrin-stabilising factor XIII compared with controls.
-
8.
Blood coagulation findings and the efficacy of factor XIII concentrate in premature infants with intracranial hemorrhages
Shirahata A, Nakamura T, Shimono M, Kaneko M, Tanaka S
Thrombosis Research. 1990;57((5):):755-63.
Abstract
Coagulation findings were examined in 55 cases of neonatal intracranial hemorrhages (ICH). Marked decreases of the platelet count, fibrinogen, factor XIII (F XIII) activity, were observed in these cases. However, the relatively mild increases in the alpha 2-plasmin inhibitor activity, alpha 2-plasmin inhibitor.plasmin complex and fibrin/fibrinogen degradation products level were observed and only about one third of the cases showed abnormal values. In consideration of these coagulation findings, fifty-eight cases of premature infants were randomly divided into a treated group with a F XIII concentrate and a non-treated group. Thirty cases were administered within 6 hours after delivery to investigate the preventive effects against intraventricular hemorrhages (IVH). Compared in frequencies between both groups limited to the cases with a high risk of IVH, the frequency in the treated group was two out of 13 (15.4%), significantly low (p less than 0.05), while it was six out of eight cases (75.0%) in the non-treated group. From these results it was concluded that a F XIII concentrate is effective in the prophylaxis of IVH in premature infants.
-
9.
Blood clotting factor XIII substitution in acute leukaemia: result of a randomized and controlled study . German
Rasche H, Haghou F, Gaus W, Dietrich M, Hoelzer D, Pflieger H, Kurrle E, Pindur G, Seifried E, Heimpel H
Deutsche Medizinische Wochenschrift. 1982;107((49):):1882-6.
Abstract
A randomized and controlled study was undertaken to test whether substitution with factor XIII concentrates influences the clinical course in patients with acute leukaemia and acquired factor XIII deficiency (less than or equal to 60%). A control group of 31 patients was compared with a factor XIII-treated group of 29 patients. Partial factor XIII deficiency was successfully corrected by substitution. On the other hand, there was no statistically significant difference between the two patient groups in the frequency and severity of bleeding complications, transfusion requirements, and the number of remissions. Undesirable side-effects as a result of substitution treatment were not observed.