PURPURA FULMINANT AND CEREBRAL,VENOUS THROMBOSIS IN 3 NEWBORNS WITH HOMOZYGOUS PROTEIN-C DEFICIENCY

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ANSARI SH.; Jallali M.; SAMAII H.

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Journal: Razi Journal of Medical Sciences Year:2001 | Volume:8 | Issue:25 Page(s): 262-264

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Title

PURPURA FULMINANT AND CEREBRAL,VENOUS THROMBOSIS IN 3 NEWBORNS WITH HOMOZYGOUS PROTEIN-C DEFICIENCY

Author(s)

ANSARI SH. | Jallali M. | SAMAII H. | Issue Writer Certificate

Keywords

PROTEIN-C DEFICIENCYQ4

PURPURA FULMINANTQ4

SKIN NECROSISQ4

Abstract

PROTEIN-C DEFICIENCY is a rare disease that is two form: Homozygote and Heterozygote. Protein-c circulates in healthy adult Plasma is value ranging from 70-140 Percent. Protein-C level in newborns are 20-40 Percent of normal adult level. Newborns with homozygote PROTEIN-C DEFICIENCY almost always manifest SKIN NECROSIS, fulminant purpura after birth and or central nervous system thrombosis. Mutation is near the active site of the protein-C and DNA analysis shows double variant.Treatment option includes Fresh Frozen Plasma (FFP) and Protein-C concentrate in the acute phase follows by oral anticoagulant therapy.Three patients were referred to Hazrat Ali Asghar Pediatrics Hospital, with developed SKIN NECROSIS ,on the abdomen and upper extremities, after 24 hours of life. One case had CNS hemorrhage after anti-coagulation therapy. Their protein-C activity were less than 1% and for their parents were less than 60%.

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APA: Copy

ANSARI, SH., Jallali, M., & SAMAII, H.. (2001). PURPURA FULMINANT AND CEREBRAL,VENOUS THROMBOSIS IN 3 NEWBORNS WITH HOMOZYGOUS PROTEIN-C DEFICIENCY. RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES), 8(25), 262-264. SID. https://sid.ir/paper/10731/en

Vancouver: Copy

ANSARI SH., Jallali M., SAMAII H.. PURPURA FULMINANT AND CEREBRAL,VENOUS THROMBOSIS IN 3 NEWBORNS WITH HOMOZYGOUS PROTEIN-C DEFICIENCY. RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES)[Internet]. 2001;8(25):262-264. Available from: https://sid.ir/paper/10731/en

IEEE: Copy

SH. ANSARI, M. Jallali, and H. SAMAII, “PURPURA FULMINANT AND CEREBRAL,VENOUS THROMBOSIS IN 3 NEWBORNS WITH HOMOZYGOUS PROTEIN-C DEFICIENCY,” RAZI JOURNAL OF MEDICAL SCIENCES (JOURNAL OF IRAN UNIVERSITY OF MEDICAL SCIENCES), vol. 8, no. 25, pp. 262–264, 2001, [Online]. Available: https://sid.ir/paper/10731/en

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