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Lookup NU author(s): Professor David KavanaghORCiD,
Dr Edwin Wong,
Dr Vicky Brocklebank
This is the authors' accepted manuscript of an article that has been published in its final definitive form by American Society of Nephrology, 2017.
For re-use rights please refer to the publisher's terms and conditions.
Background: Pregnancy is associated with various forms of thrombotic microangiopathy, including the hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical HUS linked to complement alternative pathway dysregulation. Design, setting, participants and measurements: We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway genes variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated hemolytic uremic syndrome. Results: Eighty-seven patients with pregnancy-associated hemolytic uremic syndrome were included. Hemolytic uremic syndrome occurred mainly during the first pregnancy (58%) and in the post-partum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions and 4 (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached end-stage renal disease, 15 (19%) had chronic kidney disease and 18 (28%) patients experienced hemolytic uremic syndrome relapse. Twenty four patients (27%) received a renal transplant and a recurrence of hemolytic uremic syndrome occurred in 13 (54%) cases. Variants in complement genes were detected in 49 (56%) patients, mainly in the CFH (30%) and CFI genes (9%). Conclusions: Pregnancy-associated hemolytic uremic syndrome and atypical hemolytic uremic syndrome non-related to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.
Author(s): Bruel A, Kavanagh D, Noris M, Delmas Y, Wong E, Bresin E, Provôt F, Brocklebank V, Mele C, Remuzzi G, Loirat C, Frémeaux-Bacchi V, Fakhouri F
Publication type: Article
Publication status: Published
Journal: Clinical Journal of the American Society of Nephrology
Print publication date: 07/08/2017
Online publication date: 08/06/2017
Acceptance date: 17/05/2017
Date deposited: 09/08/2017
ISSN (print): 1555-9041
ISSN (electronic): 1555-905X
Publisher: American Society of Nephrology
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