Congenital protein C deficiency in a newborn
Abstract
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.
About the Authors
S. M. Imanbay
NC JSC Karaganda Medical University;
MSI Multidisciplinary center for mother and child in Temirtau
Kazakhstan
Kazakhstan
Department of pediatrics and neonatology, Karaganda
R. M. Slanbekova
MSI Multidisciplinary center for mother and child in Temirtau
Kazakhstan
Kazakhstan
V. S. Idrysova
NC JSC Karaganda Medical University
Kazakhstan
Kazakhstan
Department of pediatrics and neonatology
Karaganda
L. S. Zhenisova
NC JSC Karaganda Medical University
Kazakhstan
Kazakhstan
Department of pediatrics and neonatology
Karaganda
B. Y. Konarbaeva
NC JSC Karaganda Medical University
Kazakhstan
Kazakhstan
Department of pediatrics and neonatology
Karaganda
S. T. Kizatova
NC JSC Karaganda Medical University
Kazakhstan
Kazakhstan
Saule Tanzilovna Kizatova – к.м.s., professor of the department of pediatrics and neonatology, тел.: +77785200994
Karaganda
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Review
For citations:
Imanbay S.M., Slanbekova R.M., Idrysova V.S., Zhenisova L.S., Konarbaeva B.Y., Kizatova S.T. Congenital protein C deficiency in a newborn. Medicine and ecology. 2022;(1):102-107.
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