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Original ArticlesClinical Nephrology
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Hemolytic Uremic Syndrome in Pregnancy and Postpartum

Alexandra Bruel, David Kavanagh, Marina Noris, Yahsou Delmas, Edwin K.S. Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi, Chantal Loirat, Véronique Frémeaux-Bacchi and Fadi Fakhouri
CJASN August 2017, 12 (8) 1237-1247; DOI: https://doi.org/10.2215/CJN.00280117
Alexandra Bruel
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David Kavanagh
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Marina Noris
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Yahsou Delmas
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Edwin K.S. Wong
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Elena Bresin
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François Provôt
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Vicky Brocklebank
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Caterina Mele
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Giuseppe Remuzzi
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Chantal Loirat
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Véronique Frémeaux-Bacchi
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Fadi Fakhouri
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Abstract

Background Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation.

Design, setting, participants, & measurements We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene 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 postpartum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions, and four (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached ESRD, 15 (19%) had CKD, and 18 (28%) patients experienced hemolytic uremic syndrome relapse. Twenty-four patients (27%) received a kidney transplant and a recurrence of hemolytic uremic syndrome occurred in 13 (54%) patients. 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 nonrelated to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.

  • hemolytic uremic syndrome
  • complement
  • pregnancy
  • thrombotic microangiopathy
  • Antibodies, Monoclonal, Humanized
  • Atypical Hemolytic Uremic Syndrome
  • Complement Pathway, Alternative
  • Female
  • Follow-Up Studies
  • France
  • Humans
  • Italy
  • Kidney Failure, Chronic
  • kidney transplantation
  • Plasma Exchange
  • Postpartum Period
  • Pregnancy
  • Recurrence
  • renal dialysis
  • Retrospective Studies
  • Thrombotic Microangiopathies
  • United Kingdom
  • chemotactic factor inactivator
  • eculizumab
  • Received January 10, 2017.
  • Accepted May 5, 2017.
  • Copyright © 2017 by the American Society of Nephrology
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Clinical Journal of the American Society of Nephrology: 12 (8)
Clinical Journal of the American Society of Nephrology
Vol. 12, Issue 8
August 07, 2017
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Hemolytic Uremic Syndrome in Pregnancy and Postpartum
Alexandra Bruel, David Kavanagh, Marina Noris, Yahsou Delmas, Edwin K.S. Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
CJASN Aug 2017, 12 (8) 1237-1247; DOI: 10.2215/CJN.00280117

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Hemolytic Uremic Syndrome in Pregnancy and Postpartum
Alexandra Bruel, David Kavanagh, Marina Noris, Yahsou Delmas, Edwin K.S. Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
CJASN Aug 2017, 12 (8) 1237-1247; DOI: 10.2215/CJN.00280117
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Cited By...

  • Recurrent case of pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS)
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  • Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy
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Keywords

  • hemolytic uremic syndrome
  • complement
  • pregnancy
  • thrombotic microangiopathy
  • Antibodies, Monoclonal, Humanized
  • Atypical Hemolytic Uremic Syndrome
  • Complement Pathway, Alternative
  • female
  • follow-up studies
  • France
  • humans
  • Italy
  • Kidney Failure, Chronic
  • kidney transplantation
  • Plasma Exchange
  • Postpartum Period
  • recurrence
  • renal dialysis
  • Retrospective Studies
  • thrombotic microangiopathies
  • United Kingdom
  • chemotactic factor inactivator
  • eculizumab

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