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


CONCLUSIONS
Arterial steno-occlusive lesions appear as potential complications of dysregulated complement activation in aHUS. Endovascular treatment should be considered cautiously in this setting.
Pediatric Nephrology Department, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, Université Paris 7, Paris, France. chantal.loirat@rdb.aphp.fr
Chantal
C
Loirat
Marie-Alice
MA
Macher
Monique
M
Elmaleh-Berges
Theresa
T
Kwon
Georges
G
Deschênes
Timothy H J
TH
Goodship
Charles
C
Majoie
Jean-Claude
JC
Davin
Raphael
R
Blanc
Julien
J
Savatovsky
Jacques
J
Moret
Véronique
V
Fremeaux-Bacchi
D000293:Adolescent; D001157:Arterial Occlusive Diseases / Q000209:etiology*; D011415:Complement Factor B / Q000235:genetics*; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications*; D006801:Humans; D009154:Mutation; D017582:Renal Replacement Therapy / Q000009:adverse effects
D011415:Complement Factor B