Three patients with hemolytic uremic syndrome (HUS) developed peripheral ga
ngrene. Bilateral carotid artery thromboses occurred in one of these patien
ts after recovery from HUS. One patient had a long history of juvenile rheu
matoid arthritis. In the second patient, a flu-like illness preceded the on
set of HUS. The third was one of two sisters, with the HUS appearing mon th
an 1 year apart. None had evidence of disseminated intravascular coagulatio
n or infection with Streptococcus pneumoniae. The patient with rheumatoid a
rthritis had renal cortical necrosis but recovered moderate renal function
after treatment with dialysis and plasmapheresis for 6 months. The child wi
th a genetic form of HUS died of renal failure and had massive cortical nec
rosis and vascular thrombosis at autopsy. This is the first report of perip
heral gangrene in children with idiopathic HUS and autosomal recessive HUS.