Homozygous protein C deficiency in a newborn. Clinicopathologic correlation
S. Tarras, C. Gadia, L. Meister, E. Roldan and J. B. Gregorios
Department of Neurology, University of Miami, FL 33101.
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.
