ABSTRACT
Three patients with encephalopathy clinically indistinguishable from Reye syndrome but associated with elevated cold-agglutinin titers and antiglobulin-I autoimmune hemolytic anemia are reported. The patients were treated with exchange transfusions, dexamethasone, controlled hyperventilation, and intracranial pressure monitoring. Liver biopsy specimens in two of the three patients showed fatty infiltration of the hepatocytes, but the light microscopic and electron microscopic appearance of the liver was not typical for Reye syndrome. These patients are reported to alert physicians to a metabolic encephalopathy in children which mimics Reye syndrome, but is distinctly different in clinical course and complicated by cold agglutinins and an autoimmune hemolytic anemia.
Subject(s)
Agglutinins/analysis , Anemia, Hemolytic, Autoimmune/complications , Autoantibodies/analysis , Brain Diseases/complications , Cold Temperature , Fatty Liver/complications , Anemia, Hemolytic, Autoimmune/therapy , Antibodies, Anti-Idiotypic , Brain Diseases/therapy , Child , Dexamethasone/therapeutic use , Diagnosis, Differential , Exchange Transfusion, Whole Blood , Fatty Liver/pathology , Fatty Liver/therapy , Female , Humans , Infant , Intracranial Pressure , Male , Reye Syndrome/diagnosis , Reye Syndrome/pathologyABSTRACT
The peripheral leukocytes of newborn infants and of adult volunteers were studied after separation of polymorphonuclear and mononuclear cells. Monocytes were identified and quantitated with the aid of histochemical staining. The in vitro killing capacity of PMN and of monocytes was assayed against Staphylococcus aureus and Escherichia coli. The monocytes of both infants and adults were significantly less active than were their PMN, but the bactericidal capacity did not differ appreciably between newborn and adult cells of either type.