Blog
About

0
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      The natural course of pregnancies in women with primary atypical haemolytic uraemic syndrome and asymptomatic relatives

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Summary

          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.

          Related collections

          Most cited references 25

          • Record: found
          • Abstract: found
          • Article: not found

          Preeclampsia and the risk of end-stage renal disease.

          It is unknown whether preeclampsia is a risk marker for subsequent end-stage renal disease (ESRD). We linked data from the Medical Birth Registry of Norway, which contains data on all births in Norway since 1967, with data from the Norwegian Renal Registry, which contains data on all patients receiving a diagnosis of end-stage renal disease (ESRD) since 1980, to assess the association between preeclampsia in one or more pregnancies and the subsequent development of ESRD. The study population consisted of women who had had a first singleton birth between 1967 and 1991; we included data from up to three pregnancies. ESRD developed in 477 of 570,433 women a mean (+/-SD) of 17+/-9 years after the first pregnancy (overall rate, 3.7 per 100,000 women per year). Among women who had been pregnant one or more times, preeclampsia during the first pregnancy was associated with a relative risk of ESRD of 4.7 (95% confidence interval [CI], 3.6 to 6.1). Among women who had been pregnant two or more times, preeclampsia during the first pregnancy was associated with a relative risk of ESRD of 3.2 (95% CI, 2.2 to 4.9), preeclampsia during the second pregnancy with a relative risk of 6.7 (95% CI, 4.3 to 10.6), and preeclampsia during both pregnancies with a relative risk of 6.4 (95% CI, 3.0 to 13.5). Among women who had been pregnant three or more times, preeclampsia during one pregnancy was associated with a relative risk of ESRD of 6.3 (95% CI, 4.1 to 9.9), and preeclampsia during two or three pregnancies was associated with a relative risk of 15.5 (95% CI, 7.8 to 30.8). Having a low-birth-weight or preterm infant increased the relative risk of ESRD. The results were similar after adjustment for possible confounders and after exclusion of women who had kidney disease, rheumatic disease, essential hypertension, or diabetes mellitus before pregnancy. Although the absolute risk of ESRD in women who have had preeclampsia is low, preeclampsia is a marker for an increased risk of subsequent ESRD. 2008 Massachusetts Medical Society
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

            In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome.

              Several studies have demonstrated genetic predisposition in non-shigatoxin-associated hemolytic uremic syndrome (HUS), involving regulatory proteins of the complement alternative pathway: Factor H (FH) and membrane co-factor protein (CD46). Regarding the observations of thrombotic thrombocytopenic purpura patients, in whom a von Willebrand factor protease (ADAMST-13) deficiency may be inherited or acquired secondary to IgG antibodies, it was speculated that HUS might occur in a context of an autoimmune disease with the development of anti-FH antibodies leading to an acquired FH deficiency. The presence of FH autoantibodies was investigated by an ELISA method using coated purified human FH in a series of 48 children who presented with atypical HUS and were recruited from French university hospitals. Anti-FH IgG antibodies were detected in the plasma of three children who presented with recurrent HUS. The anti-FH specificity was conserved by the Fab'2 fraction. The plasma FH activity was found to be decreased, whereas plasma FH antigenic levels and FH gene analysis were normal, indicating that the presence of anti-FH antibodies led to an acquired functional FH deficiency. This report supports for the first time that HUS may occur in a context of an autoimmune disease with the development of anti-FH-specific antibody leading to an acquired FH deficiency. This new mechanism of functional FH deficiency may lead to the design of new approaches of diagnosis and treatment with a particular interest in plasma exchanges or immunosuppressive therapies.
                Bookmark

                Author and article information

                Contributors
                p.vanpaassen@maastrichtuniversity.nl
                Journal
                Br J Haematol
                Br. J. Haematol
                10.1111/(ISSN)1365-2141
                BJH
                British Journal of Haematology
                John Wiley and Sons Inc. (Hoboken )
                0007-1048
                1365-2141
                27 April 2020
                August 2020
                : 190
                : 3 ( doiID: 10.1111/bjh.v190.3 )
                : 442-449
                Affiliations
                [ 1 ] Department of Nephrology and Clinical Immunology Maastricht University Medical Center Maastricht the Netherlands
                [ 2 ] Department of Biochemistry Cardiovascular Research Institute Maastricht (CARIM) Maastricht University Maastricht the Netherlands
                [ 3 ] Division of Nephrology Cliniques Universitaires Saint‐Luc Brussels Belgium
                [ 4 ] Department of Obstetrics and Gynecology Maastricht University Medical Center Maastricht the Netherlands
                [ 5 ] Department of Central Diagnostic Laboratory Maastricht University Medical Center Maastricht the Netherlands
                [ 6 ] Institut de Recherche Expérimentale et Clinique UCLouvain Brussels Belgium
                Author notes
                [* ] Correspondence: Pieter van Paassen, Department of Nephrology and Clinical Immunology, Maastricht University Medical Center, PO Box 5800, 6202 AZ, Maastricht, The Netherlands.

                E‐mail: p.vanpaassen@ 123456maastrichtuniversity.nl

                Article
                BJH16626
                10.1111/bjh.16626
                7496636
                © 2020 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

                Page count
                Figures: 0, Tables: 3, Pages: 8, Words: 6026
                Product
                Funding
                Funded by: Fondation Saint‐Luc
                Funded by: National Fund for Scientific Research
                Funded by: Fonds de Recherche des Cliniques Universitaires Saint‐Luc
                Funded by: Association pour l’Information et la Recherche sur les Maladies Rénales Génétiques
                Categories
                Research Paper
                Transplantation
                Custom metadata
                2.0
                August 2020
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.9.0 mode:remove_FC converted:11.09.2020

                Comments

                Comment on this article