medecolМедицина и экологияMedicine and ecology2305-60452305-6053Карагандинский медицинский университетmedecol-383Research ArticleНАБЛЮДЕНИЯ ИЗ ПРАКТИКИCLINICAL CASESВрожденный дефицит протеина С у новорожденногоCongenital protein C deficiency in a newbornИманбайС. М.ImanbayS. M.

Кафедра педиатрии и неонатологии, Караганда;

г. Темиртау

Department of pediatrics and neonatology, Karaganda

СланбековаР. М.SlanbekovaR. M.

г. Темиртау

ИдрисоваВ. С.IdrysovaV. S.

Кафедра педиатрии и неонатологии

Караганда

Department of pediatrics and neonatology

Karaganda

ЖенисоваЛ. С.ZhenisovaL. S.

Кафедра педиатрии и неонатологии

Караганда

Department of pediatrics and neonatology

Karaganda

КонарбаеваБ. Е.KonarbaevaB. Y.

Кафедра педиатрии и неонатологии

Караганда

Department of pediatrics and neonatology

Karaganda

КизатоваС. Т.KizatovaS. T.

Кафедра педиатрии и неонатологии

Караганда

Saule Tanzilovna Kizatova – к.м.s., professor of the department of pediatrics and neonatology, тел.:  +77785200994

Karaganda

Kizatova@qmu.kzНАО Медицинского Университета Караганды; КГП Многопрофильный центр матери и ребенкаКазахстанNC JSC Karaganda Medical University; MSI Multidisciplinary center for mother and child in TemirtauKazakhstanКГП Многопрофильный центр матери и ребенкаКазахстанMSI Multidisciplinary center for mother and child in TemirtauKazakhstanМедицинский университет КарагандыКазахстанNC JSC Karaganda Medical UniversityKazakhstan20220406202301102107Copyright © Иманбай С.М., Сланбекова Р.М., Идрисова В.С., Женисова Л.С., Конарбаева Б.Е., Кизатова С.Т., 20232023Иманбай С.М., Сланбекова Р.М., Идрисова В.С., Женисова Л.С., Конарбаева Б.Е., Кизатова С.Т.Imanbay S.M., Slanbekova R.M., Idrysova V.S., Zhenisova L.S., Konarbaeva B.Y., Kizatova S.T.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://medecol.qmu.kz/jour/article/view/383

Дефицит протеина С является редким, но опасным для жизни нарушением свертываемости крови, которое может проявиться в ближайшем неонатальном периоде. В этой статье представлен случай новорожденной девочки с острой и прогрессирующей молниеносной пурпурой новорожденных, тромбозом, ДВС синдромом, внутричерепным кровоизлиянием, развившиеся, в течение 4 дней после рождения как проявление дефицита протеина С. Активность протеина С была ниже 5%. Лечение включает коррекцию коагулопатии, интенсивный уход за раной, включая повязки с отрицательным давлением и пересадку кожи, а также поддерживающую терапию при проблемах с центральной нервной системой. Долгосрочное наблюдение состоит из пожизненной антикоагулянтной терапии, чтобы избежать повторения этих осложнений.

Protein C deficiency is a rare but life-threatening bleeding disorder that may present in the nearest neonatal period. This article presents the case of a newborn girl with acute and progressive neonatal fulminant purpura, thrombosis, DICsyndrome, intracranial hemorrhage, which developed within 4 days after birth as a manifestation of protein С deficiency. Protein С activity was below 5%. Treatment includes correction of coagulopathy, intensive wound care including negative pressure dressings and skin grafting, and supportive care for central nervous system problems. Long-term follow-up consists of lifelong anticoagulant therapy to avoid recurrence of these complications.

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