CC-BY-NC-ND 4.0 · AJP Rep 2018; 08(02): e68-e70
DOI: 10.1055/s-0038-1639614
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

Erika Uehara
1  Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
,
Hiro Nakao
1  Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
2  Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan
,
Yusuke Tsumura
1  Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
,
Hisaya Nakadate
2  Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan
3  Division of Hematology, National Center for Child Health and Development, Tokyo, Japan
,
Shoichiro Amari
4  Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan
,
Hideshi Fujinaga
4  Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan
,
Yoshiyuki Tsutsumi
5  Department of Radiology, National Center for Child Health and Development, Tokyo, Japan
,
Dongchon Kang
6  Department of Clinical Chemistry, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
,
Shouichi Ohga
7  Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
,
Akira Ishiguro
1  Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan
3  Division of Hematology, National Center for Child Health and Development, Tokyo, Japan
› Author Affiliations
Further Information

Publication History

17 November 2017

12 February 2018

Publication Date:
12 April 2018 (online)

Abstract

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.

Statement of Ethics

The institutional review board of our hospital approved this investigation. Informed consent was obtained from the caretakers for publication of this case report and accompanying images.