Abnormal Platelet and Vascular Prostaglandin Synthesis in an Infant with Hemolytic Uremic Syndrome
1 Department of Pediatrics, The State University of New York, Upstate Medical Center, Syracuse
2 Department of Surgery, The State University of New York, Upstate Medical Center, Syracuse
The hemolytic-uremic syndrome (HUS) is a disorder characterized by a microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Pathologic examination reveals thrombotic occlusions in the microcirculation, with the kidney being the main target organ involved. Recently, Remuzzi et al1,2 have demonstrated abnormalities in vascular prostacyclin or prostaglandin I2 (PGI2) formation in HUS. We report further characterization of the abnormality in PGI2 synthesis in a patient with HUS. An abnormality in platelet prostaglandin endoperoxide formation was also observed.
CASE REPORT
A 4-month-old male infant was hospitalized with anemia, thrombocytopenia, and proteinuria. The neonatal period had been complicated by hyperbilirubinemia necessitating an exchange transfusion.




