Sickle cell anemia in the newborn.

The clinical presentation of homozygous sickle cell disease is unusual in the neonatal period. Recently, we have encountered a newborn infant whose disease was apparent at birth and who died at 5 days of age. The findings at autopsy suggested a sickle cell crisis with multisystem involvement that was present prior to birth. Laboratory findings confirmed homozygous sickle cell disease without the presence of elevated levels of hemoglobin S. The cause of the unusually severe clinical course of the disease in our patient is the object of the discussion.