Preview

Transbaikalian Medical Bulletin

Advanced search

COAGULATION FACTOR XIII DEFICIENCY – LAKI-LORAND DISEASE: SCIENTIFIC REVIEW AND A CASE REPORT

https://doi.org/10.52485/19986173_2022_3_105

Abstract

The proposed article presents a modern review of the literature covering the mechanism of development, clinical, laboratory criteria and tactics conducting of children with rare coagulopathy – deficiency of factor XIII of blood coagulation (Laki-Lorand disease). The review is illustrated by a spectacular clinical example of an early-age girl with the described pathology, manifested by repeated recurrent bleedings from the newborn period, confirmed by a specific molecular genetic testing.

About the Authors

O. G. Maksimova
Chita State Medical Academy
Russian Federation

39А Gorky str., Chita, 672000



E. P. Bataeva
Chita State Medical Academy
Russian Federation

39А Gorky str., Chita, 672000



A. G. Rakhmaturov
Regional children's clinical hospital
Russian Federation

20 Novobulvarnaya str., Chita, 672027



A. I. Kudryavtseva
Regional children's clinical hospital
Russian Federation

20 Novobulvarnaya str., Chita, 672027



I. I. Petrukhina
Chita State Medical Academy
Russian Federation

39А Gorky str., Chita, 672000



O. I. Kryazheva
GUZ "Zabaikalsky regional oncological dispensary"
Russian Federation

104 Leningradskaya str., Chita, 672027



V. A. Sizenko
GUZ "Zabaikalsky regional oncological dispensary"
Russian Federation

104 Leningradskaya str., Chita, 672027



References

1. Lorand L. Factor XIII and the clotting of fibrinogen: From basic research to medicine. Journal of Thrombosis and Haemostasis. 2005. 3(7). 1337-1348. DOI: 10.1111/j.1538-7836.2005.01213.x.

2. Kuznik B.I., Sturov V.G., Levshin N.Yu., Maksimova O.G., Kudlay D.A. Hemorrhagic and thrombotic diseases and syndromes in children and adolescents. Novosibirsk. The science. 2018. ISBN 978-5-02-038708-9. in Russian.

3. Florinsky D.B., Zharkov P.A. Rare coagulopathies. Russian Journal of Pediatric Hematology and Oncology. 2020. 1(7). pp. 54-63. DOI: 10.21682/2311-1267-2020-7-3-54-63.

4. Dorgalaleh A., Rashidpanah J. Blood coagulation factor XIII and factor XIII deficiency. Blood Rev. 2016. 30(6). 461-475. DOI: 10.1016/j.blre.2016.06.002.

5. Ogawa Y. Acquired autoimmune coagulation factor XIII/13 deficiency. Rinsho Ketsueki. 2020. 61(7). 799-808. DOI: 10.11406/rinketsu.61.799.

6. Ichinose A. Diagnosis and treatment of acquired factor XIII/13 deficiencies: for all doctors treating the MHLW's designated intractable diseases. Rinsho Ketsueki. 2015. Oct. 56(10). 2110-2122. DOI: 10.11406/rinketsu.56.2110.

7. Osaki T., Sugiyama D., Magari Y., Souri M., Ichinose A. Rapid immunochromatographic test for detection of anti-factor XIII A subunit antibodies can diagnose 90 % of cases with autoimmune haemorrhaphilia XIII/13. Thromb Haemost. 2015. Jun. 113(6). 1347-1356. DOI: 10.1160/TH14-09-0745.

8. Souri M., Osaki T., Ichinose A.J. Anti-factor XIII A subunit (FXIII-A) autoantibodies block FXIII-A2 B2 assembly and steal FXIII-A from native FXIII-A2 B2. Thromb Haemost. 2015. May. 13(5). 802-814. DOI: 10.1111/jth.12877.

9. Biswas A., Ivaskevicius V., Thomas A., Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hamostaseologie. 2014. 34(2). 160-166. DOI: 10.5482/HAMO-13-08-0046.

10. Tahlan A., Ahluwalia J. Factor XIII: congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit. Arch Pathol Lab Med. 2014. Feb. 138(2). 278-281. DOI: 10.5858/arpa.2012-0639-RS.

11. Kohler H., Ichinose A., Seitz R., Ariens R.A.S, Muszbek L. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011. 9(7). 1404-1406. DOI: 10.1111/j.1538-7836.2011.04315.


Review

For citations:


Maksimova O.G., Bataeva E.P., Rakhmaturov A.G., Kudryavtseva A.I., Petrukhina I.I., Kryazheva O.I., Sizenko V.A. COAGULATION FACTOR XIII DEFICIENCY – LAKI-LORAND DISEASE: SCIENTIFIC REVIEW AND A CASE REPORT. Transbaikalian Medical Bulletin. 2022;(3):105-110. (In Russ.) https://doi.org/10.52485/19986173_2022_3_105

Views: 202


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 1998-6173 (Online)