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

Erika Uehara; Hiro Nakao; Yusuke Tsumura; Hisaya Nakadate; Shoichiro Amari; Hideshi Fujinaga; Yoshiyuki Tsutsumi; Dongchon Kang; Shouichi Ohga; Akira Ishiguro

doi:10.1055/s-0038-1639614

American Journal of Perinatology Reports, Inhaltsverzeichnis

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

¹Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan

,

Hiro Nakao

¹Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan

²Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan

,

Yusuke Tsumura

¹Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan

,

Hisaya Nakadate

²Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan

³Division of Hematology, National Center for Child Health and Development, Tokyo, Japan

,

Shoichiro Amari

⁴Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan

,

Hideshi Fujinaga

⁴Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan

,

Yoshiyuki Tsutsumi

⁵Department of Radiology, National Center for Child Health and Development, Tokyo, Japan

,

Dongchon Kang

⁶Department of Clinical Chemistry, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

,

Shouichi Ohga

⁷Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

,

Akira Ishiguro

¹Department of Postgraduate Education and Training, National Center for Child Health and Development, Tokyo, Japan

³Division of Hematology, National Center for Child Health and Development, Tokyo, Japan

› Institutsangaben

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.

Keywords

neonatal stroke - hemorrhagic infarction - protein C deficiency - PROC mutation

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Referenzen

References
1 Ohga S, Kang D, Kinjo T. , et al. Paediatric presentation and outcome of congenital protein C deficiency in Japan. Haemophilia 2013; 19 (03) 378-384
2 Ohga S, Ishiguro A, Takahashi Y. , et al; Japan Childhood Thrombophilia Study Group. Protein C deficiency as the major cause of thrombophilias in childhood. Pediatr Int 2013; 55 (03) 267-271
3 Takahashi Y, Yoshioka A. Hemostasis and its regulation system in childhood. Jpn J Pediatr Hematol 1994; 8: 389-397
4 Manco-Johnson MJ, Abshire TC, Jacobson LJ, Marlar RA. Severe neonatal protein C deficiency: prevalence and thrombotic risk. J Pediatr 1991; 119 (05) 793-798
5 Matsunaga Y, Ohga S, Kinjo T. , et al. Neonatal asphyxia and renal failure as the presentation of non-inherited protein C deficiency. J Perinatol 2013; 33 (03) 239-241