Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children24510.24287/1726-1708-2019-18-2-59-65ORIGINAL ARTICLESОРИГИНАЛЬНЫЕ СТАТЬИExperience of protein C administration in children with acquired deficiencyПрименение концентрата протеина С у детей с приобретенным его дефицитомhttps://orcid.org/0000-0002-0053-0146ShifrinI. A.ШифринЮ. А.
Russian Federation

Iurii А. Shifrin, clinical pharmacist.

117997, Moscow, Samory Mashela st., 1.

Шифрин Юрий Александрович, врач-клинический фармаколог.

117997, Москва, ГСП-7, ул. Саморы Машела, 1.

doc.shifrin@gmail.comhttps://orcid.org/0000-0003-4384-6754ZharkovP. V.ЖарковП. А.
Russian Federation
https://orcid.org/0000-0003-0080-3367PashanovE. D.ПашановE. Д.
Russian Federation
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России2906201918259652906201929062019Copyright ©; 2019, «D. Rogachev NMRCPHOI»Copyright ©; 2019, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2019«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/245

Тhere is increasing experience of protein C concentrate administration in world practice, but despite that, information of this drug administration in patients with oncohematological diseases and primary immunodeficiency syndromes is lacking. Objective: to study the effectiveness of protein C concentrate administration in pediatric patients with acquired protein C deficiency during the treatment of oncological, hematological or immunological diseases. Medical charts of 12 patients who received inpatient treatment and protein C concentrate administration in the Dmitry Rogachev National Clinical Research Center from 01/01/2012–12/31/18 were analyzed. Depending on the presence or absence of thrombosis, the patients were divided into two groups. Single and daily doses, the number of injections per day, the duration of therapy and the percentage of activity of protein C activity were studied in both groups. Вoth groups included 6 patients, median of a single administrated dose of protein C was lower in the group of patients with thrombosis than in patients without them (20 and 71.4 IU/kg, p < 0.0001), while there were obtained no differences between treatment efficacy (p = 0.45). When comparing the administered dose of the drug in children with unresolved and resolved thrombosis, it was found that the median single dose in patients with ineffective treatment was lower than in those who had effective treatment (8.78 and 71.4 IU/kg, respectively, p < 0.0001); the median daily dose was also lower in the group with ineffective treatment (20 and 71.4 IU/kg, respectively, p < 0.005). Рrotein C administration in children with acquired deficiency for the purpose of antithrombotic prophylaxis can be potentially effective, especially in those patients who already have a thrombosis at the moment of administration. The effectiveness of such prophylaxis may depend on the dose of the injected concentrate. To determine the appropriate dose and mode of administration of the drug in children a prospective study is required. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology.

В мировой практике накоплен опыт применения концентрата протеина С, но сведения о применении данного препарата у больных, страдающих от онкогематологических заболеваний, у больных с первичными иммунодефицитами весьма ограничены. Цель исследования: изучение эффективности применения концентрата протеина С у педиатрических пациентов онкологического, гематологического, иммунологического профилей с приобретенной недостаточностью данного фактора. Проанализированы данные историй болезни 12 пациентов, находившихся на стационарном лечении в НМИЦ ДГОИ им. Дмитрия Рогачева в период с 01.01.2012 по 31.12.2018 и получавших терапию концентратом протеина С. В зависимости от наличия или отсутствия тромбозов пациенты были разделены на две группы – в каждой по 6 пациентов. В обеих группах изучали эффективность лечения при однократной и суточной дозах введенного препарата в зависимости от количества введений в сутки, длительности терапии, определяли процент активности протеина С. Медиана разовой дозы концентрата была ниже в группе пациентов с тромбозами – 20 МЕ/кг; у пациентов без тромбозов – 71,4 МЕ/кг (р < 0,0001), при этом разницы в эффективности лечения не было (p = 0,45). При сравнении введенной дозы препарата у детей с неразрешившимся и разрешившимся тромбозами выявлено, что медиана разовой дозы у пациентов с неэффективным лечением была ниже по сравнению с теми, у кого терапия была эффективна, – 8,78 и 71,4 МЕ/кг соответственно (р < 0,0001). Медиана суточной дозы также была ниже в группе с неэффективным лечением – 20 и 71,4 МЕ/кг соответственно (р < 0,005). Применение концентрата протеина С у детей с приобретенным его дефицитом с целью антитромботической профилактики может быть потенциально эффективным, особенно если у пациента на момент введения уже есть тромбоз. Эффективность такой профилактики может зависеть от дозы вводимого концентрата. Для определения адекватной дозы и режима введения препарата у детей требуется проведение проспективных исследований. Данное исследование поддержано Независимым этическим комитетом и утверждено решением Ученого совета НМИЦ ДГОИ.

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