Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan
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-aut...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-03-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/6/1699 |
| _version_ | 1797470442775117824 |
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| author | Shiho Amano Kohei Oka Yutaka Sato Chiaki Sano Ryuichi Ohta |
| author_facet | Shiho Amano Kohei Oka Yutaka Sato Chiaki Sano Ryuichi Ohta |
| author_sort | Shiho Amano |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T19:36:24Z |
| format | Article |
| id | doaj.art-38948ddebefb4f1d9ba3f9f8e84e1ba6 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T19:36:24Z |
| publishDate | 2022-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-38948ddebefb4f1d9ba3f9f8e84e1ba62023-11-24T01:51:06ZengMDPI AGJournal of Clinical Medicine2077-03832022-03-01116169910.3390/jcm11061699Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in JapanShiho Amano0Kohei Oka1Yutaka Sato2Chiaki Sano3Ryuichi Ohta4Community Care, Unnan City Hospital, Unnan 699-1221, JapanMedical Department, Shimane University, 89-1 Enya cho, Izumo 693-8501, JapanMedical Department, Shimane University, 89-1 Enya cho, Izumo 693-8501, JapanDepartment of Community Medicine Management, Faculty of Medicine, Shimane University, Izumo 693-8501, JapanCommunity Care, Unnan City Hospital, Unnan 699-1221, JapanFactor 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.https://www.mdpi.com/2077-0383/11/6/1699factor XIII deficiencybleeding disorderfactor XIII inhibitorsacquired factor XIII deficiency |
| spellingShingle | Shiho Amano Kohei Oka Yutaka Sato Chiaki Sano Ryuichi Ohta Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan Journal of Clinical Medicine factor XIII deficiency bleeding disorder factor XIII inhibitors acquired factor XIII deficiency |
| title | Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan |
| title_full | Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan |
| title_fullStr | Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan |
| title_full_unstemmed | Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan |
| title_short | Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan |
| title_sort | measuring factor xiii inhibitors in patients with factor xiii deficiency a case report and systematic review of current practices in japan |
| topic | factor XIII deficiency bleeding disorder factor XIII inhibitors acquired factor XIII deficiency |
| url | https://www.mdpi.com/2077-0383/11/6/1699 |
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