Atypical Hemolytic Uremic Syndrome With Obstructive Hydrocephalus in an Infant

Abstract

Hemolytic uremic syndrome (HUS) which is characterized by a triad of hemolytic anemia, low platelet count and acute renal failure was first described by Gasser and others in 1955. (1,2) Initially it used to have a mortality rate of 50 % to 80%. But the improved diagnostic methods and treatment strategies has reduced the mortality to 2%-8%. Three types of HUS have been identified, typical HUS after enterohemorrhagic. Escherichia coli, shiga toxin 1 & 2 (Stx-HUS/D+HUS) producing bloody diarrhea, atypical HUS (a HUS/D-HUS) with complement abnormalities and not associated with diarrhea and pneumococcal associated HUS-mediated by neuraminidase in the presence of streptococcus pneumonia infection. Multiple systems are involved in the pathology including gastrointestinal, renal, hematological, brain, pancreas and myocardium. (3,4,5) Here we present a case of an infant with atypical HUS who also developed obstructive hydrocephalus following respiratory infection due to streptococcus pneumonia. This is the first case report in the English literature so far.