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February 1988

Homozygous Protein C Deficiency in a NewbornClinicopathologic Correlation

Author Affiliations

From the Departments of Neurology (Drs Tarras and Gadia), Pediatrics (Dr Meister), and Pathology (Drs Roldan and Gregorios), University of Miami.

Arch Neurol. 1988;45(2):214-216. doi:10.1001/archneur.1988.00520260102029

• A rare case of homozygous protein C deficiency occurred in a newborn. The patient presented with purpura fulminans in the first few hours after birth and showed multiple hemorrhagic lesions on computed tomography of the brain at 5 days of age. Neurologic symptoms developed at two weeks and the patient died at 37 weeks. His protein C level was less than 5%. Autopsy revealed thrombosis of the dural sinuses, multiple cortical infarcts, intraparenchymal hemorrhages, and hydrocephalus. The pathologic findings are correlated with the neurologic deficits and previously documented cases are reviewed.