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

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)1237-1247
Number of pages11
JournalClinical journal of the American Society of Nephrology : CJASN
Volume12
Issue number8
DOIs
Publication statusPublished - Aug 7 2017

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Hemolytic-Uremic Syndrome
Postpartum Period
Pregnancy
Alternative Complement Pathway
Thrombotic Microangiopathies
Genes
Recurrence
Plasma Exchange
Gene Frequency
Italy
Chronic Kidney Failure
France
Dialysis
Transplants
Kidney

Keywords

  • Journal Article

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Hemolytic Uremic Syndrome in Pregnancy and Postpartum. / Bruel, Alexandra; Kavanagh, David; Noris, Marina; Delmas, Yahsou; Wong, Edwin K S; Bresin, Elena; Provôt, François; Brocklebank, Vicky; Mele, Caterina; Remuzzi, Giuseppe; Loirat, Chantal; Frémeaux-Bacchi, Véronique; Fakhouri, Fadi.

In: Clinical journal of the American Society of Nephrology : CJASN, Vol. 12, No. 8, 07.08.2017, p. 1237-1247.

Research output: Contribution to journalArticle

Bruel, A, Kavanagh, D, Noris, M, Delmas, Y, Wong, EKS, Bresin, E, Provôt, F, Brocklebank, V, Mele, C, Remuzzi, G, Loirat, C, Frémeaux-Bacchi, V & Fakhouri, F 2017, 'Hemolytic Uremic Syndrome in Pregnancy and Postpartum', Clinical journal of the American Society of Nephrology : CJASN, vol. 12, no. 8, pp. 1237-1247. https://doi.org/10.2215/CJN.00280117
Bruel, Alexandra ; Kavanagh, David ; Noris, Marina ; Delmas, Yahsou ; Wong, Edwin K S ; Bresin, Elena ; Provôt, François ; Brocklebank, Vicky ; Mele, Caterina ; Remuzzi, Giuseppe ; Loirat, Chantal ; Frémeaux-Bacchi, Véronique ; Fakhouri, Fadi. / Hemolytic Uremic Syndrome in Pregnancy and Postpartum. In: Clinical journal of the American Society of Nephrology : CJASN. 2017 ; Vol. 12, No. 8. pp. 1237-1247.
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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.",
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AU - Bruel, Alexandra

AU - Kavanagh, David

AU - Noris, Marina

AU - Delmas, Yahsou

AU - Wong, Edwin K S

AU - Bresin, Elena

AU - Provôt, François

AU - Brocklebank, Vicky

AU - Mele, Caterina

AU - Remuzzi, Giuseppe

AU - Loirat, Chantal

AU - Frémeaux-Bacchi, Véronique

AU - Fakhouri, Fadi

N1 - Copyright © 2017 by the American Society of Nephrology.

PY - 2017/8/7

Y1 - 2017/8/7

N2 - 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.

AB - 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.

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