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Atypical hemolytic uremic syndrome with large vessel involvement
Alolika Nandi1, Prabhas Prasun Giri2, Agnisekhar Saha3, Rajiv Sinha4
1 Department of Pediatrics, Calcutta National Medical College and Hospital, Kolkata, West Bengal, India
2 Pediatric Intensive Care Unit, Institute of Child Health, Kolkata, West Bengal, India
3 Pediatric Intensive Care Unit, Institute of Child Health, Apollo Gleneagles Hospital, Kolkata, West Bengal, India
4 Pediatric Nephrology Unit, Institute of Child Health, Kolkata, West Bengal, India
Correspondence Address:
Alolika Nandi
C/o Dr. Siddhartha Nandi, Marikpara, Nawabganj, Ichapur, Barrackpur, North 24 Parganas, Kolkata - 743 144, West Bengal
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/2589-9309.305899
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Hemolytic uremic syndrome (HUS) is a rare, life-threatening disorder caused by thrombotic microangiopathy. Atypical hemolytic uremic syndrome (aHUS) is usually secondary to genetic mutation or autoantibody against the alternate complement pathway that produces a dysregulated complement activation, endothelial cell damage, and thrombosis. Here, we report the case of a 7-year-old boy who presented to us with aphasia, hemiparesis, and frank acute kidney injury and hemolysis, due to anti-complement factor H (anti-CFH) antibody associated aHUS. Investigations revealed multiple stenoses in bilateral internal carotid arteries and right proximal cerebral artery, producing a progressive massive infarct, an unusual finding in aHUS.
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