Evidence map›Paper›PMID 32047641›Full record

ArticleCanadian journal of kidney health and disease2020

Clinical Characteristics and Outcome of Canadian Patients Diagnosed With Atypical Hemolytic Uremic Syndrome.

Anne-Laure Lapeyraque, Martin Bitzan, Imad Al-Dakkak, Mira Francis, Shih-Han S Huang, Roger Kaprielian, Loree Larratt, Katerina Pavenski, Christine Ribic, Axel Tosikyan and 2 more

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Canadian journal of kidney health and disease, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
0.6field-weighted citation impact, top 36% 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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

6 citing papers in PubMed, 12 citations in OpenAlex.

  1. Observational
  2. Article
  3. Review
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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

12 authors at 10 institutions in 2 countries.

Anne-Laure LapeyraqueCentre Hospitalier Universitaire Sainte-Justine, Montreal, QC, Canada.
Martin BitzanMontreal Children's Hospital, Montreal, QC, Canada.
Imad Al-DakkakAlexion Pharmaceuticals, Inc., New Haven, CT, USA.
Mira FrancisAlexion Pharma Canada Corp., Vaughan, ON, Canada.
Shih-Han S HuangVictoria Hospital, London Health Sciences Centre, London, ON, Canada.
Roger KaprielianAlexion Pharma Canada Corp., Vaughan, ON, Canada.
Loree LarrattUniversity of Alberta Hospital, Edmonton, AB, Canada.
Katerina PavenskiSt. Michael's Hospital, Toronto, ON, Canada.
Christine RibicSt. Joseph's Healthcare Hamilton, Hamilton, ON, Canada.
Axel TosikyanHôpital du Sacré-Cœur de Montréal, Montreal, QC, Canada.
Christoph LichtThe Hospital for Sick Children, Toronto, ON, Canada.
David PhilibertHôtel-Dieu de Québec, Quebec, QC, Canada.
Alberta Hospital Edmonton · CAAlexion Pharmaceuticals (United States) · USCentre Hospitalier Universitaire Sainte-Justine · CAHôpital du Sacré-Cœur de Montréal · CAHospital for Sick Children · CAHôtel-Dieu de Québec · CAMontreal Children's Hospital · CASt. Joseph’s Healthcare Hamilton · CASt. Michael's Hospital · CAVictoria Hospital · CA

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundAtypical hemolytic uremic syndrome (aHUS) is an extremely rare, heterogeneous disease of uncontrolled activation of the alternative complement pathway that is difficult to diagnose. We have evaluated the Canadian patients enrolled in the Global aHUS Registry to provide a Canadian perspective regarding the diagnosis and management of aHUS and the specific challenges faced.

objectiveTo evaluate Canadian patients enrolled in the Global aHUS Registry to provide a Canadian perspective regarding the diagnosis and management of aHUS and the specific challenges faced.

methodsThe Global aHUS Registry is an observational, noninterventional, multicenter study that has prospectively and retrospectively collected data from patients of all ages with an investigator-made clinical diagnosis of aHUS, irrespective of treatment. Patients of all ages with a clinical diagnosis of aHUS were eligible and invited for enrollment, and those with evidence of Shiga toxin-producing

resultsA total of 37 Canadian patients were enrolled (15 pediatric and 22 adult patients) between February 2014 and May 2017; the median age at initial aHUS presentation was 25.9 (interquartile range = 6.7-51.7) years; 62.2% were female and 94.6% had no family history of aHUS. Over three-quarters of patients (78.4%) had no conclusive genetic or anti-complement factor H (CFH) antibody information available, and most patients (94%) had no reported precipitating factors prior to aHUS diagnosis. Nine patients (8 adults and 1 child) experienced ESRD prior to the study. After initial presentation, there appears to be a trend that children are less likely to experience ESRD than adults, with 5-year ESRD-free survival of 93 and 56% (

conclusionsThis evaluation provides insight into the diagnosis and management of aHUS in Canadian patients and the challenges faced. More genetic or anti-CFH antibody testing is needed to improve the diagnosis of aHUS, and the management of children and adults needs to consider several factors such as the risk of progression to ESRD is based on age (more likely in adults), and that the location of extrarenal manifestations differs in children and adults.

Indexed as

complementhemolytic uremic syndromethrombotic microangiopathy

Identifiers

PMID32047641
PMCPMC6984425
OpenAlexW3002627750

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
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Registered trials

None linked

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.