Endogenous heparin-like syndrome in a patient after intrauterine spina bifida repair

DOI

https://dx.doi.org/10.18565/aig.2024.104

Beznoshchenko O.S., Ostrik K.A., Silaev B.V., Shmakov R.G., Pyregov A.V.
1) Academician V.I. Kulakov National Medical Research Centre for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia, Moscow, Russia; 2) Academician V.I. Krasnopolsky Moscow Regional Research Institute of Obstetrics and Gynecology, Moscow, Russia; 3) Moscow Regional Perinatal Center, Balashikha, Russia

Background: Endogenous heparin-like syndrome (HLS) in obstetrics is a rare complication most practicing physicians are unfamiliar with. Glycosaminoglycans (GAGs) circulating in the bloodstream, mostly of endothelial origin, play the role of endogenous heparins. Liver disease, systemic inflammatory response, and infectious diseases are associated with the HLS development. The incidence of HLS varies according to pathology; for instance, it is 5% in trauma, and in liver transplantation it can be as high as 100%. The incidence of HLS in obstetrics remains unclear. The difficulty in the correction of the hemorrhagic syndrome associated with HLS is the lack of a uniform approach to therapy.
Case report: This article presents the first clinical observation of the HLS development in a pregnant woman after intrauterine correction of fetal spina bifida by open fetal surgery. Severe HLS was detected by thromboelastometry (CTINTEM /CTHEPTEM ratio >2); Laboratory parameters (activated partial thromboplastin time, thrombin time) showed hypocoagulation due to the presence of GAGs in the blood.
Conclusion: Monitoring of the hemostasis system in the perioperative period in obstetric patients can help timely recognize HLS and reduce the incidence of bleeding caused by the presence of endogenous GAGs. Further study is required to better understand how to diagnose and treat HLS effectively.

Authors’ contributions: Shmakov R.G., Pyregov A.V., Silaev B.V. – review of the content, approval of the manuscript for publication; Ostrik K.A. – collecting clinical material and writing the manuscript; Beznoshchenko O.S. – performing laboratory tests, reviewing publications on the subject of the article, analyzing and interpreting data, writing the manuscript.
Conflicts of interest: Authors declare lack of the possible conflicts of interest.
Funding: The study was conducted without sponsorship.
Patient Consent for Publication: The patient provided an informed consent for the publication of her data related to the clinical research.
For citation: Beznoshchenko O.S., Ostrik K.A., Silaev B.V., Shmakov R.G., Pyregov A.V.
Endogenous heparin-like syndrome in a patient after intrauterine spina bifida repair.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2024; (10): 184-190 (in Russian)
https://dx.doi.org/10.18565/aig.2024.104

glycosaminoglycans

endogenous heparin-like syndrome

hemorrhagic syndrome

thromboelastometry

heparanase

spina bifida

Full text (in Russian)

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Received 24.04.2024

Accepted 30.09.2024

Olga S. Beznoshchenko, PhD, Researcher at the Institute of Anesthesiology, Reanimatology and Transfusiology, clinical laboratory diagnostics doctor at the Department of Anesthesiology and Intensive Care, Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia, 177997, Russia, Moscow, Ac. Oparin str., 4, o_beznoshchenko@oparina4.ru, https://orcid.org/0000-0003-4645-8976
Kirill A. Ostrik, anesthesiologist-resuscitator at the Department of Anesthesiology and Intensive Care, Academician V.I. Kulakov National Medical Research
Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia, 177997, Russia, Moscow, Ac. Oparin str., 4, k_ostrik@oparina4.ru,
https://orcid.org/0009-0005-6064-665С
Borislav V. Silaev, PhD, Head of the Department of Anesthesiology-Intensive Care, Director of the Institute of Anesthesiology-Intensive Care and Transfusiology, Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia, 177997, Russia, Moscow, Ac. Oparin, str. 4; Associate Professor at the Department of Anesthesiology and Intensive Care, I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia,
b_silaev@oparina4.ru, https://orcid.org/0000-0002-9698-3915
Roman G. Shmakov, Dr. Med. Sci., Professor of the Russian Academy of Sciences, Director, Academician V.I. Krasnopolsky Moscow Regional Research Institute of Obstetrics and Gynecology, 101000, Russia, Moscow, Pokrovka str., 22а; Professor at the Department of Obstetrics and Gynecology named after Academician G.M. Savelyeva of Pediatric Faculty, Pirogov Russian National Research Medical University, Ministry of Health of Russia; Chief Freelance Specialist in Obstetrics of the Ministry of Health of Russia, mdshmakov@mail.ru, https://orcid.org/0000-0002-2206-1002
Alexey V. Pyregov, Dr. Med. Sci., Professor, Deputy Chief Physician for Anesthesiology and Resuscitation, Moscow Regional Perinatal Center, 143900, Russia, Moscow Region, Balashikha, Shosse Entuziastov, 12; Chief Freelance Specialist in Anesthesiology and Resuscitation in Obstetrics of the Ministry of Health of the Moscow Region,
pyregov@mail.ru, https://orcid.org/0000-0001-8382-9671

Endogenous heparin-like syndrome in a patient after intrauterine spina bifida repair

Beznoshchenko O.S., Ostrik K.A., Silaev B.V., Shmakov R.G., Pyregov A.V.

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