Journal Article

Non-atheromatous arterial stenoses in atypical haemolytic uraemic syndrome associated with complement dysregulation

Chantal Loirat, Marie-Alice Macher, Monique Elmaleh-Berges, Theresa Kwon, Georges Deschênes, Timothy H.J. Goodship, Charles Majoie, Jean-Claude Davin, Raphael Blanc, Julien Savatovsky, Jacques Moret and Véronique Fremeaux-Bacchi

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 25, issue 10, pages 3421-3425
Published in print October 2010 | ISSN: 0931-0509
Published online June 2010 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/gfq319
Non-atheromatous arterial stenoses in atypical haemolytic uraemic syndrome associated with complement dysregulation

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Background. A child, who presented atypical haemolytic uraemic syndrome (aHUS) at the age of 1 month, developed cerebral ischaemic events at the age of 10 years.

Results. Stenoses of both carotid arteries, left subclavian and vertebral arteries, several intracranial, right humeral, several coronary, and all pulmonary arteries were demonstrated. At the age of 13 years, left subclavian and right cervical carotid arteries were occluded. Right carotid recanalization induced intracranial dissection and death. The child had a Lys350Asp factor B mutation.

Conclusion. Arterial steno-occlusive lesions appear as potential complications of dysregulated complement activation in aHUS. Endovascular treatment should be considered cautiously in this setting.

Keywords: angioplasty; arterial stenosis; atypical haemolytic uraemic syndrome; complement factor B; complement factor H

Journal Article.  2235 words.  Illustrated.

Subjects: Nephrology

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