bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/02345730-2020-65-1-61-69bloodjour-187Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSCлучай интраоперационного выявления дефицита протеина C у больного, оперированного по поводу цианотического порока сердцаA clinical case of protein C deficiency revealed in a patient during surgery for cyanotic heart defecthttps://orcid.org/0000-0003-2122-7813ГончаровА. А.GoncharovA. A.

Гончаров Андрей Андреевич – врач — анестезиолог-реаниматолог отделения анестезиологии-реанимации.

121552, Москва

Andrey A. Goncharov – Anesthesiologist-Reanimatologist, Anesthesiology and Intensive Care Department.

121552, Moscow

goncharov141290@mail.ruhttps://orcid.org/0000-0001-8206-8794РыбкаМ. М.RybkaM. M.

Рыбка Михаил Михайлович – доктор медицинских наук, заведующий отделением анестезиологии-реанимации.

121552, Москва

Mikhail M. Rybka – Dr. Sci. (Med.), Head of the Anesthesiology and Intensive Care Department.

121552, Moscow

rybkamikh@mail.ruhttps://orcid.org/0000-0002-4161-7416ХинчаговД. Я.KhinchagovD. Ya.

Хинчагов Джумбер Яковлевич – кандидат медицинских наук, врач анестезиолог-реаниматолог отделения анестезиологии-реанимации.

121552, Москва

Djumber Ya. Khinchagov – Cand. Sci. (Med.), Anesthesiologist-Reanimatologist, Anesthesiology and Intensive Care Department.

121552, Moscow

khinch@mail.ruhttps://orcid.org/0000-0003-3327-1723РогальскаяЕ. А.RogalskayaE. A.

Рогальская Екатерина Анатольевна – кандидат медицинских наук, врач клинической лабораторной диагностики лаборатории гематологии.

121552, Москва

Ekaterina A. Rogalskaya – Cand. Sci. (Med.), Pathologist, Department of Hematology.

121552, Moscow

rogalskaya.ea@yandex.ruФГБУ «Национальный медицинский исследовательский центр сердечно-сосудистой хирургии им. А.Н. Бакулева» Министерства здравоохранения Российской ФедерацииРоссияA.N. Bakoulev Scientific Center for Cardiovascular SurgeryRussian Federation2020110320206516169Copyright © Гончаров А.А., Рыбка М.М., Хинчагов Д.Я., Рогальская Е.А., 20202020Гончаров А.А., Рыбка М.М., Хинчагов Д.Я., Рогальская Е.А.Goncharov A.A., Rybka M.M., Khinchagov D.Y., Rogalskaya E.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/187Введение

Введение. Дефицит протеина С является редким заболеванием. Однако у недоношенных детей с цианотическими пороками сердца данная патология может встречаться часто.

Цель — представить клиническое наблюдение интраоперационного выявления дефицита протеина С у новорожденного больного, оперированного по поводу цианотического порока сердца.

Основные сведения

Основные сведения. Описано клиническое наблюдение интраоперационного рецидивирующего тромбоза системнолегочных шунтов у новорожденного больного с цианотическим пороком сердца. Новорожденные и недоношенные дети с цианотическими пороками сердца являются категорией высокого риска развития интраоперационных тромбозов из-за дефицита естественных антикоагулянтов, который может возникнуть как вследствие генетически обусловленного дефицита протеина С, так и вследствие недоношенности ребенка, незрелости печени, снижения ее синтетической функции, сердечной недостаточности и гипоксемии. Необходим дополнительный предоперационный скрининг на наличие дефицита естественных антикоагулянтов у данной группы больных, а в стационарах, в которых им выполняют операции, должна быть возможность проведения заместительной терапии концентратом протеина С.

Background

Background. Protein C deficiency is a rare condition. However, this pathology occurs more frequently in premature babies with cyanotic heart disease.

Aim

Aim. To present a clinical case of an intraoperative detection of protein C deficiency in a patient operated for cyanotic heart disease.

General findings

General findings. A clinical case of an intraoperative recurrent thrombosis of systemic-to-pulmonary shunts in a newborn patient with cyanotic heart disease is described. Newborn and premature babies with cyanotic heart defects belong to a high risk group of developing intraoperative thrombosis due to a deficiency of natural anticoagulants, which can occur as a result of genetically conditioned protein C deficiency, prematurity, immaturity of the liver or its reduced synthetic function, heart failure and hypoxemia. Additional pre-operative screening is required for detecting a deficiency of natural anticoagulants in this group of patients. The hospitals that perform such surgery should be equipped with protein C concentrates.

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The authors declare that there are no conflicts of interest present.