Evidence map›Paper›PMID 33826112›Full record

ArticleJournal of nephrology2021

Pregnancy-triggered atypical hemolytic uremic syndrome (aHUS): a Global aHUS Registry analysis.

Fadi Fakhouri, Marie Scully, Gianluigi Ardissino, Imad Al-Dakkak, Benjamin Miller, Eric Rondeau

Registry-linked trialOpen access · hybridAbstract read
In one paragraph

Article in Journal of nephrology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01522183 (An Observational, Non-Interventional, Multi-Center, Multi-National Study of Patients With Atypical Hemolytic-Uremic Syndrome), which is not on this map. Cited by 31 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
31citing papers in PubMed, 2 pooled it
3.7field-weighted citation impact, top 6% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT01522183 recruitingnot on this map

An Observational, Non-Interventional, Multi-Center, Multi-National Study of Patients With Atypical Hemolytic-Uremic Syndrome (aHUS Registry)

Typeobservational_patient_registrySponsorAlexion Pharmaceuticals, Inc.Ran2013 to 2031Enrolled3,000ConditionsAtypical Hemolytic-Uremic Syndrome
3 · Its place in the literature

Who cites it

31 citing papers in PubMed, 2 syntheses or guidelines pooled it, 56 citations in OpenAlex.

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  17. Placental and Renal Pathways Underlying Pre-Eclampsia.International journal of molecular sciences · 2024
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 4 institutions in 4 countries.

Fadi FakhouriService of Nephrology and Hypertension, University of Lausanne, Lausanne, Switzerland. fadi.fakhouri@unil.ch.
Marie ScullyUniversity College London, London, UK.
Gianluigi ArdissinoCenter for HUS Control, Prevention and Management, Milan, Italy.
Imad Al-DakkakAlexion Pharmaceuticals, Inc., Boston, MA, USA.
Benjamin MillerAlexion Pharmaceuticals, Inc., Boston, MA, USA.
Eric RondeauHôpital Tenon, Paris, France.
Alexion Pharmaceuticals (United States) · USAssistance Publique – Hôpitaux de Paris · FRUniversity College London · GBUniversity Hospital of Lausanne · CH

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundAtypical hemolytic uremic syndrome (aHUS) is a rare disease in which uncontrolled terminal complement activation leads to systemic thrombotic microangiopathy (TMA). Pregnancy can trigger aHUS and, without complement inhibition, many women with pregnancy-triggered aHUS (p-aHUS) progress to end-stage renal disease (ESRD) with a high risk of morbidity. Owing to relatively small patient numbers, published characterizations of p-aHUS have been limited, thus the Global aHUS Registry (NCT01522183, April 2012) provides a unique opportunity to analyze data from a large single cohort of women with p-aHUS.

methodsThe demographics and clinical characteristics of women with p-aHUS (n = 51) were compared with those of women of childbearing age with aHUS and no identified trigger (non-p-aHUS, n = 397). Outcome evaluations, including renal survival according to time to ESRD, were compared for patients with and without eculizumab treatment (a complement C5 inhibitor) in both aHUS groups.

resultsBaseline demographics and clinical characteristics were broadly similar in both groups. The proportion of women with p-aHUS and non-p-aHUS with pathogenic variant(s) in complement genes and/or anti-complement factor H antibodies was similar (45% and 43%, respectively), as was the proportion with a family history of aHUS (12% and 13%, respectively). Eculizumab treatment led to significantly improved renal outcomes in women with aHUS, regardless of whether aHUS was triggered by pregnancy or not: adjusted hazard ratio for time to ESRD was 0.06 (p = 0.006) in the p-aHUS group and 0.20 (p < 0.0001) in the non-p-aHUS group.

conclusionFindings from this study support the characterization of p-aHUS as a complement-mediated TMA.

Indexed as

Atypical Hemolytic Uremic SyndromeThrombotic MicroangiopathiesComplement Inactivating AgentsComplement System ProteinsFemaleHumansPregnancyRegistriesComplement Inactivating AgentsComplement System ProteinsAtypical hemolytic uremic syndrome (aHUS)Complement C5 inhibitorComplement-mediated TMAEnd-stage renal disease (ESRD)Pregnancy

Identifiers

PMID33826112
PMCPMC8494679
OpenAlexW3142113446

What OpenQuestion holds

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LicenceCC BY
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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.