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BRIEF REPORT
Year : 2020  |  Volume : 3  |  Issue : 2  |  Page : 64-66

Atypical hemolytic uremic syndrome with large vessel involvement


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
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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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