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Neonatal Purpura Fulminans due to Homozygous Protein C Deficiency

Journal of the Korean Pediatric Society 1992;35(5):696-703.
Published online May 15, 1992.
Neonatal Purpura Fulminans due to Homozygous Protein C Deficiency
Jae-Hong Park1, Sung-Sub Sim1, Soo-Young Kim1, Hee-Joo Jeon1, Chan-Yung Kim1, Hong Keun Oh2, Jin-Yeong Jeong2
1Department of Pediatrics, College of Medicine, Pusan National University, Pusan, Korea
2Department of Pediatrics, Asan Medical Center Haesung Hospital, Ulsan, Korea
동형접합성 단백 C 결핍에 의한 신생아 전격성 자반증 1례
박재홍1, 심성섭1, 김수영1, 전희주1, 김찬영1, 오홍근2, 정지영2
1부산대학교 의과대학 소아과학교실
2아산 사회복지 사업재단 해성병원 소아과
Homozygous protein C deficiency is a rare herediatary disorder of blood coagulation resulting in microvascular and venous thromboses, usually purpura fulminans, starting shortly after birth. Protein C serves to inactivate the active forms of factor V and VIII, and increase the rate of fibrin degradation. For confirmation of homozygous protein C deficiency, the infant should have undetectable protein C activity and both parents should be heterozygous for protein C deficiency. We experienced a case of purpura fulminans due to homozygous protein C deficiency, which first apperared at age 14 hours with sudden onset of large ecchymoses on both flanks, buttocks, and thighs, and rapidly progressed. We confirmed the case by protein C study for patient and her family members, and report with the brief review of the literature.
Key Words: Homozygous protein C deficiency, Purpura fulminans

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