@article{c36d361621f74cb3897e793bef79624d,
title = "The natural course of pregnancies in women with primary atypical haemolytic uraemic syndrome and asymptomatic relatives",
abstract = "Pregnancy has been linked to various microangiopathies, including primary atypical haemolytic uraemic syndrome (aHUS). Complement dysregulation, often linked to rare variants in complement genes, is key for primary aHUS to manifest and may play a role in pregnancy complications of the mother and fetus. The burden of such complications is unknown, making counselling of women with primary aHUS and asymptomatic relatives difficult. We analyzed the maternal and fetal outcomes of 39 pregnancies from 17 women with primary aHUS and two asymptomatic relatives. Seven out of 39 pregnancies were complicated by pregnancy-associated aHUS. Five out of 32 pregnancies not linked to pregnancy-associated aHUS were complicated by pre-eclampsia or HELLP. Rare genetic variants were identified in 10 women (asymptomatic relatives, n = 2) who had a total of 14 pregnancies, including 10 uncomplicated pregnancies. Thirty-five out of 39 pregnancies resulted in live birth. Eight out of 19 women had progressed to end-stage kidney disease, with an incidence of 2·95 (95% confidence interval, 1·37–5·61) per 100 person-years after the first pregnancy. Thus, we emphasized the frequency of successful pregnancies in women with primary aHUS and asymptomatic relatives. Pregnancies should be monitored closely. Rare genetic variants cannot predict the risk of a given pregnancy.",
keywords = "primary atypical haemolytic uraemic syndrome, thrombotic microangiopathy, pregnancy, complement, genetics, COMPLEMENT INHIBITOR ECULIZUMAB, MEMBRANE COFACTOR PROTEIN, THROMBOTIC MICROANGIOPATHIES, FACTOR-H, HYPERTENSION, VARIANTS, OUTCOMES, DISEASE, AHUS",
author = "Timmermans, {Sjoerd A. M. E. G.} and Alexis Werion and Spaanderman, {Marc E. A.} and Reutelingsperger, {Chris P.} and Damoiseaux, {Jan G. M. C.} and Johann Morelle and Pieter Paassen",
note = "Funding Information: We gratefully thank the nephrologists affiliated with the Limburg Renal Registry and the members of the multidisciplinary TMA/HUS team at the Cliniques Universitaires Saint‐Luc and the UCLouvain Kidney Disease Network for the recruitment and excellent care of patients. Furthermore, we acknowledge N. Bijnens, E. Geelkens, H. van Rie and R. Theunissen (Maastricht University Medical Center) for their excellent technical assistance and S. Druart and Y. Cnops for the management of the biobank (Cliniques Universitaires Saint‐Luc). This work was supported in part by funding from the Fondation Saint‐Luc (J.M), the National Fund for Scientific Research (J.M.), the Fonds de Recherche des Cliniques Universitaires Saint‐Luc (J.M.) and the Association pour l{\textquoteright}Information et la Recherche sur les Maladies R{\'e}nales G{\'e}n{\'e}tiques (J.M.). Funding Information: We gratefully thank the nephrologists affiliated with the Limburg Renal Registry and the members of the multidisciplinary TMA/HUS team at the Cliniques Universitaires Saint-Luc and the UCLouvain Kidney Disease Network for the recruitment and excellent care of patients. Furthermore, we acknowledge N. Bijnens, E. Geelkens, H. van Rie and R. Theunissen (Maastricht University Medical Center) for their excellent technical assistance and S. Druart and Y. Cnops for the management of the biobank (Cliniques Universitaires Saint-Luc). This work was supported in part by funding from the Fondation Saint-Luc (J.M), the National Fund for Scientific Research (J.M.), the Fonds de Recherche des Cliniques Universitaires Saint-Luc (J.M.) and the Association pour l?Information et la Recherche sur les Maladies R?nales G?n?tiques (J.M.). Publisher Copyright: {\textcopyright} 2020 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd",
year = "2020",
month = aug,
doi = "10.1111/bjh.16626",
language = "English",
volume = "190",
pages = "442--449",
journal = "British Journal of Haematology",
issn = "0007-1048",
publisher = "John Wiley & Sons Inc.",
number = "3",
}