Clinical Pediatric Hematology-Oncology  2011;18(1):54-57

A Case of Central Retinal Vein Occlusion by Protein C Deficiency

Beom Soo KIM 1 ; In Sang JEON

Affiliations

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Keywords

Protein C; Renal vein occlusion

Country

Republic of Korea

Language

Korean

Abstract

Protein C (PC) is a vitamin K-dependent plasma protein synthesized as a zymogen by liver. It's active form has an anti-thrombotic activity by cleaving the coagulation factor Va and VIIIa. During the last few years, a large number of cases with protein C deficiency (PCD) which contain variable mutations have been documented. Those with hereditary protein C deficiency tend to have an increased risk of thromboembolism. Hypertension, diabetes, hyperlipidemia and glaucoma are the main underlying diseases of the retinal vein occlusion (RVO). Rarely, thrombophilia (antithrombin III, protein S, protein C deficiency) results in the RVO which usually occurs after adolescent period. We recently experienced a case of central retinal vein occlusion by a PCD.