Slow Elevation in Protein C Activity without a PROC Mutation in a Neonate with Intracranial Hemorrhage

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  • Erika Uehara
    Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
  • Hiro Nakao
    Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
  • Yusuke Tsumura
    Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
  • Hisaya Nakadate
    Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan
  • Shoichiro Amari
    Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan
  • Hideshi Fujinaga
    Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan
  • Yoshiyuki Tsutsumi
    Department of Radiology, National Center for Child Health and Development, Tokyo, Japan
  • Dongchon Kang
    Department of Clinical Chemistry, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  • Shouichi Ohga
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  • Akira Ishiguro
    Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan

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<jats:title>Abstract</jats:title><jats:p>Severe protein C (PC) deficiency leads to purpura fulminans and stroke in newborns. However, the clinical impact of plasma PC activity on the development of neonatal cerebral disease remains elusive. We report a case of hemorrhagic stroke associated with neonatal asphyxia and severe PC deficiency. Plasma PC and protein S activity 7 days after birth was 12% and 43%, respectively. No PROC mutation was found. PC levels did not exceed 20% until 2 months of age, even in the absence of consumption coagulopathy or vitamin K deficiency. Neither thromboembolic nor hemorrhagic events occurred during the infusion of activated PC concentrate (twice weekly, up to 68 days after birth). The PC activity levels gradually increased to the standard value for age by 9 months of age. The present case showed that neonatal PC deficiency without a PROC mutation caused an intracranial hemorrhage before a slow increase in PC activity.</jats:p>

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