Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children10710.24287/1726-1708-2015-14-3-5-12HEMATOLOGYГЕМАТОЛОГИЯModern concepts of the pathogenesis of haemophilic arthropathyСовременные представления о патогенезе гемофилической артропатииPolyanskayaT. Yu.ПолянскаяТ. Ю.
Russian Federation
ZorenkoV. Yu.ЗоренкоВ. Ю.
Russian Federation
v.zorenko@mail.ruKarpovE. E.КарповЕ. Е.
Russian Federation
karpov.evg@mail.ruHematological CenterГематологический научный центр Минздрава России1909201514351219092018Copyright ©; 2015, «D. Rogachev NMRCPHOI»Copyright ©; 2015, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2015«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/107

The main manifestations of haemophilia are bleedings in the major joints. Haemarthrosis triggers the pathogenetic cascade, which can lead to development of haemophilic arthropathy. The blood has a negative effect on the status of the articular cartilage and synovial membrane. Numerous studies demonstrate the key role of prophylactic treatment in prevention of haemophilic arthropathy. The dose of coagulation factor can be corrected with consideration for its pharmacokinetic characteristics, so that the activity of the deficient factor is not reduced below 1% for a long time, in order to prevent spontaneous bleedings and development of arthropathy.

Основным проявлением гемофилии являются кровоизлияния в крупные суставы. Гемартроз запускает патогенети- ческий каскад, который может привести к развитию гемофилической артропатии. Кровь оказывает негативное влия- ние на состояние хрящевой ткани и синовиальной оболочки сустава. В ходе многочисленных исследований была показана ключевая роль профилактической гемостатической терапии в предотвращении развития гемофилической артропатии. Знание фармакокинетических характеристик препарата позволяет корректировать дозы вводимого кон- центрата фактора свертывания крови и избегать длительного снижения активности дефицитного фактора в крови менее 1%, что дает возможность предотвратить возникновение спонтанных кровотечений и развитие артропатии.

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