Congenital combined vitamin K-dependent clotting factors deficiency: case reports

D. B. Florinskiy; Флоринский Д. Б.; P. А. Zharkov; Жарков П. А.; A. V. Pshonkin; Пшонкин А. В.; A. V. Poletaev; Полетаев А. В.; D. V. Fedorova; Федорова Д. В.; E. A. Seregina; Серегина Е. А.

doi:10.24287/1726-1708-2022-21-1-104-109

Congenital combined vitamin K-dependent clotting factors deficiency: case reports

Authors: Florinskiy D.B.¹, Zharkov P.А.¹, Pshonkin A.V.¹, Poletaev A.V.¹, Fedorova D.V.¹, Seregina E.A.¹^,2
Affiliations:
1. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
2. Center for Theoretical Problems of Physicochemical Pharmacology, Russian Academy of Sciences
Issue: Vol 21, No 1 (2022)
Pages: 104-109
Section: CLINICAL OBSERVATIONS
Submitted: 29.03.2022
Accepted: 29.03.2022
Published: 29.03.2022
URL: https://hemoncim.com/jour/article/view/596
DOI: https://doi.org/10.24287/1726-1708-2022-21-1-104-109
ID: 596

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Abstract

Congenital combined vitamin K-dependent clotting factors deficiency is an extremely rare bleeding disorder, a few cases have been described worldwide. This coagulopathy requires a differential diagnosis with vitamin K deficiency bleeding of the newborn and coagulopathy, associated with different liver disease. The world practice of treating this hemorrhagic condition is the use of vitamin K 1 (not registered in the Russian Federation), as well as a prothrombin complex concentrate. This article describes the clinical manifestations, diagnosis and specific treatment in 2 patients with this bleeding disorder. The patients' parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.