Hemolytic Uremic Syndrome in Pregnancy and Postpartum
Copyright © 2017 by the American Society of Nephrology..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2017 |
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| Erschienen: |
2017 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:12 |
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| Enthalten in: |
Clinical journal of the American Society of Nephrology : CJASN - 12(2017), 8 vom: 07. Aug., Seite 1237-1247 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bruel, Alexandra [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 30.04.2018 Date Revised 15.11.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.2215/CJN.00280117 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM272759937 |
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| 500 | |a Date Revised 15.11.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2017 by the American Society of Nephrology. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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%) | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Multicenter Study | |
| 650 | 4 | |a Antibodies, Monoclonal, Humanized | |
| 650 | 4 | |a Atypical Hemolytic Uremic Syndrome | |
| 650 | 4 | |a Complement Pathway, Alternative | |
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| 700 | 1 | |a Kavanagh, David |e verfasserin |4 aut | |
| 700 | 1 | |a Noris, Marina |e verfasserin |4 aut | |
| 700 | 1 | |a Delmas, Yahsou |e verfasserin |4 aut | |
| 700 | 1 | |a Wong, Edwin K S |e verfasserin |4 aut | |
| 700 | 1 | |a Bresin, Elena |e verfasserin |4 aut | |
| 700 | 1 | |a Provôt, François |e verfasserin |4 aut | |
| 700 | 1 | |a Brocklebank, Vicky |e verfasserin |4 aut | |
| 700 | 1 | |a Mele, Caterina |e verfasserin |4 aut | |
| 700 | 1 | |a Remuzzi, Giuseppe |e verfasserin |4 aut | |
| 700 | 1 | |a Loirat, Chantal |e verfasserin |4 aut | |
| 700 | 1 | |a Frémeaux-Bacchi, Véronique |e verfasserin |4 aut | |
| 700 | 1 | |a Fakhouri, Fadi |e verfasserin |4 aut | |
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