Deficiency of factor VII (hypoproconvertinemia): case report
DOI: 10.32364/2618-8430-2024-7-2-15
A.V. Dmitriev1,2, N.V. Fedina1, M.S. Yudakova3, S.N. Dronova3, K.O. Slabachkov1, A.L. Zaplatnikov2
1Ryazan State Medical University, Ryazan, Russian Federation
2Russian Medical Academy of Continuous Professional Education, Moscow,
Russian Federation
3N.V. Dmitrieva Regional Children's Clinical Hospital, Ryazan, Russian Federation
Factor VII (proconvertin) is a pivotal initial step in the activation of the extrinsic pathway of blood coagulation. Factor VII deficiency is a rare coagulopathy that can manifest in a wide range of clinical symptoms, from asymptomatic deficiency to severe hemorrhagic syndrome, which can often lead to death due to hemorrhages, including brain hemorrhages. The article provides a brief overview of the epidemiology of the disease, its clinical manifestations, diagnostic approaches, and treatment options. The authors present a case of relatively late diagnosis of hypoproconvertinemia, which manifested with severe bleeding in the neonatal period. In the first year of life, the child experienced recurrent hemorrhagic syndrome, which was managed by administering menadione sodium bisulfite trihydrate solution. Examination did not reveal deficiency of factor VIII or IX. The diagnosis was established at the age of 3 years, and the child received recombinant factor VII. However, on-demand therapy and the lack of long-term preventive administration of the drug did not provide a sufficient hemostatic effect, as evidenced by repeated episodes of hemarthrosis and severe gastrointestinal bleeding at the age of 15 years. These complications necessitated a switch to continuous administration of recombinant factor VII.
Keywords: hypoproconvertinemia, hemophilia, children, bleeding, recombinant FVII.
For citation: Dmitriev A.V., Fedina N.V., Yudakova M.S., Dronova S.N., Slabachkov K.O., Zaplatnikov A.L. Deficiency of factor VII (hypoproconvertinemia): case report. Russian Journal of Woman and Child Health. 2024;7(2):184–188 (in Russ.). DOI: 10.32364/2618-8430-2024-7-2-15.
ABOUT THE AUTHORS:
Andrey V. Dmitriev — Dr. Sc. (Med.), Professor, Head of the Department of Children Diseases with the Course of Hospital Pediatrics, Ryazan State Medical University; 9, Vysokovol'tnaya str., Ryazan, 390026, Russian Federation; professor of the Prof. N.N. Gavryushov Department of Neonatology, Russian Medical Academy of Continuous Professional Education; 2/1, Barrikadnaya str., Moscow, 125993, Russian Federation; ORCID iD 0000-0002-8202-3876.
Natalya V. Fedina — C. Sc. (Med.), associate professor of the Department of Children Diseases with the Course of Hospital Pediatrics, Ryazan State Medical University; 9, Vysokovol'tnaya str., Ryazan, 390026, Russian Federation; ORCID iD 0000-0001-6307-7249.
Marina S. Yudakova — hematologist of the Department of Chemotherapy of Cancer and Hematologic Patients, N.V. Dmitrieva Regional Children's Clinical Hospital; 1Z, Internatsional'naya str., Ryazan, 390039, Russian Federation; ORCID iD 0009-0009-7040-2690.
Svetlana N. Dronova — hematologist of the Department of Chemotherapy of Cancer and Hematologic Patients, N.V. Dmitrieva Regional Children's Clinical Hospital; 1Z, Inter-natsional'naya str., Ryazan, 390039, Russian Federation; ORCID iD 0009-0008-0007-7426.
Kirill O. Slabachkov — student of the Medical Faculty, Ryazan State Medical University; 9, Vysokovol'tnaya str., Ryazan, 390026, Russian Federation; ORCID iD 0000-0003-4638-9560.
Andrey L. Zaplatnikov — Dr. Sc. (Med.), Professor, Vice-chancellor for Instructional Work, Head of the Prof. N.N. Gavryushov Department of Neonatology, professor of the Acad. G.N. Speranskiy Department of Pediatrics, Russian Medical Academy of Continuous Professional Education; 2/1, Barrikadnaya str., Moscow, 125993, Russian Federation; ORCID iD 0000-0003-1303-8318.
Contact information: Natalya V. Fedina, e-mail: k2ataka@mail.ru.
Financial Disclosure: no authors have a financial or property interest in any material or method mentioned.
There is no conflict of interest.
Received 11.03.2024.
Revised 03.04.2024.
Accepted 26.04.2024.
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