nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.17650/2311-1267-2015-1-49-53nodgo-78Research ArticleОригинальные исследованияВнутричерепные кровоизлияния у детей с гемофилиейIntracranial haemorrhage in a children suffering from heamofiliaЛившицМатвей ИгоревичLivshitzM. I.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

milivshitz@mail.ruПоповВ. Е.PopovV. E.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

КолтуновИ. Е.KoltunovI. E.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

СвиринП. В.SvirinP. V.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

ЛевовА. В.LevovA. V.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

МироновИ. Б.MironovI. B.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

ЧигибаевМ. Ж.ChigibaevM. Zh.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

УмеренковВ. Н.UmerenkovV. N.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

ГерасимовЭ. Т.GerasimovE. T.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

КарповА. Б.KarpovA. B.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

БашлачевМ. Г.BashlachevM. G.

Россия, 119049, Москва, 4-й Добрынинский пер., 1/9

1/9 4th Dobryninskiy Per., Moscow, 119049, Russia

Центр по лечению цереброваскулярной патологии при ГБУЗ «Морозовская детская городская клиническая больница»РоссияCenter for the Treatment of Cerebrovascular Disease in Children and Adolescents in Morozov Children, s HospitalRussian Federation201523052015214953Copyright © Лившиц М.И., Попов В.Е., Колтунов И.Е., Свирин П.В., Левов А.В., Миронов И.Б., Чигибаев М.Ж., Умеренков В.Н., Герасимов Э.Т., Карпов А.Б., Башлачев М.Г., 20152015Лившиц М.И., Попов В.Е., Колтунов И.Е., Свирин П.В., Левов А.В., Миронов И.Б., Чигибаев М.Ж., Умеренков В.Н., Герасимов Э.Т., Карпов А.Б., Башлачев М.Г.Livshitz M.I., Popov V.E., Koltunov I.E., Svirin P.V., Levov A.V., Mironov I.B., Chigibaev M.Z., Umerenkov V.N., Gerasimov E.T., Karpov A.B., Bashlachev M.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/78

Гемофилия – это наследственное X-сцепленное заболевание, проявляющееся нарушением коагуляционного гемостаза (первичный геморрагический диатез). Гемофилия А – рецессивная мутация в X-хромосоме, которая приводит к отсутствию или недостаточной активности в крови фактора свертывания (ФС) VIII (антигемофильного глобулина). Частота ее составляет от 1:5000 до 1:10000 рожденных мальчиков. Гемофилия B обусловлена дефицитом ФС IX (рецессивная мутация в X-хромосоме), частота ее составляет от 1:25000 до 1:30000 рожденных мальчиков.

Частота встречаемости геморрагического инсульта у детей составляет 0,7–5,1 (в среднем – 2,9) на 100 000. Сообщения о внутричерепных кровоизлияниях (ВЧК) у мальчиков больных гемофилией большая редкость, однако, по данным зарубежных авторов, они встретились у 1,7–11,7 % пациентов, что при соответствующем перерасчете составило 290–2142:100 000 в год. Таким образом, риск развития ВЧК у пациентов с гемофилией в 56–420 раз выше, чем в общей популяции. Медиана возраста детей с ВЧК колеблется в интервале от 5,9 мес до 8 лет. Тактика оперативного вмешательства не выработана.

Haemophilia is a hereditary X-linked desease manifested in coagulatory hemostasis disorder. Haemophilia A is a recessive mutation in X-chromosome which results in the absence or malfunction of blood clotting factor VIII. Its frequency from 1:5000 to 1:10,000 of born boys. Haemophilia B is caused by the factor IX deficiency (recessive mutation in X-chromosome), with an incidence from 1:25,000 to 1:30,000 of born boys. Frequency of occurrence hemorrhagic stroke in children is 0.7–5.1 (average – 2.9) per 100,000. Reported intracranial hemorrhages (ICH) boys with haemophilia are rare, however, according to foreign authors encountered in 1.7–11.7 % of patients that with appropriate recalculation was 290–2142:100,000 per year. Thus, the risk of ICH patients with hemophilia in 56–420 times higher than the general population. The median age of children with ICH ranges from 5.9 months to 8 years. Operational tactics not developed.

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