Hemolytic Uremic Syndrome in Pregnancy and Postpartum

Details

Serval ID
serval:BIB_A01F3BD9971C
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Hemolytic Uremic Syndrome in Pregnancy and Postpartum
Journal
Clin J Am Soc Nephrol
Author(s)
Bruel A., Kavanagh D., Noris M., Delmas Y., Wong E. K. S., Bresin E., Provot F., Brocklebank V., Mele C., Remuzzi G., Loirat C., Fremeaux-Bacchi V., Fakhouri F.
ISSN
1555-905X (Electronic)
ISSN-L
1555-9041
Publication state
Published
Issued date
2017
Volume
12
Number
8
Pages
1237-1247
Language
english
Notes
Bruel, Alexandra
Kavanagh, David
Noris, Marina
Delmas, Yahsou
Wong, Edwin K S
Bresin, Elena
Provot, Francois
Brocklebank, Vicky
Mele, Caterina
Remuzzi, Giuseppe
Loirat, Chantal
Fremeaux-Bacchi, Veronique
Fakhouri, Fadi
eng
WT_/Wellcome Trust/United Kingdom
MR/K023519/1/MRC_/Medical Research Council/United Kingdom
MR/R000913/1/MRC_/Medical Research Council/United Kingdom
Multicenter Study
Clin J Am Soc Nephrol. 2017 Aug 7;12(8):1237-1247. doi: 10.2215/CJN.00280117. Epub 2017 Jun 8.
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.
Keywords
Adolescent, Adult, Antibodies, Monoclonal, Humanized/therapeutic use, Complement Activation/drug effects/genetics, Complement Factor H/genetics, Complement Factor I/genetics, Complement Inactivating Agents/therapeutic use, Disease Progression, Europe, Female, Genetic Predisposition to Disease, Genetic Variation, Hemolytic-Uremic Syndrome/complications/genetics/immunology/therapy, Humans, Kidney Failure, Chronic/etiology, Middle Aged, Phenotype, Plasma Exchange, *Postpartum Period, Pregnancy, *Pregnancy Complications/genetics/immunology/therapy, Recurrence, Renal Dialysis, Renal Insufficiency, Chronic/etiology, Retrospective Studies, Time Factors, Treatment Outcome, Young Adult, Antibodies, Monoclonal, Humanized, Atypical Hemolytic Uremic Syndrome, Complement Pathway, Alternative, Follow-Up Studies, France, Italy, Kidney Failure, Chronic, Postpartum Period, Thrombotic Microangiopathies, United Kingdom, chemotactic factor inactivator, complement, eculizumab, hemolytic uremic syndrome, kidney transplantation, thrombotic microangiopathy
Pubmed
Create date
01/03/2022 10:18
Last modification date
02/03/2022 6:36
Usage data