A 33-day-old male infant was admitted to the neonatal intensive care n
ursery because of respiratory distress, grunting, cyanosis, and radiol
ogical findings of bilateral bronchopneumonia. He responded well to in
tensive therapy, but 11 days later developed hemolytic uremic syndrome
, which was treated conservatively with prednisone and plasma transfus
ions with good response. The hemolytic uremic syndrome resolved, but h
e subsequently developed severe recurrent infections of unknown etiolo
gy and died at the age of 78 days. Necropsy findings revealed necrotiz
ing enterocolitis as well as dysplasia of the thymus and other lymphoi
d tissues, compatible with the diagnosis of immunodeficiency disorder.