Evidence map›Paper›PMID 34300196›Full record

ReviewJournal of clinical medicine2021

Fabry Cardiomyopathy: Current Treatment and Future Options.

Irfan Vardarli, Manuel Weber, Christoph Rischpler, Dagmar Führer, Ken Herrmann, Frank Weidemann

Open access · goldAbstract readReview
In one paragraph

Review in Journal of clinical medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
1.8field-weighted citation impact, top 15% 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

7 citing papers in PubMed, 15 citations in OpenAlex.

  1. Review
  2. Hemodynamics in Left-Sided Cardiomyopathies.Reviews in cardiovascular medicine · 2024
    Review
  3. Review
  4. Article
  5. Review
  6. Article
  7. Article
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 1 country.

Irfan VardarliDepartment of Medicine I, Klinikum Vest GmbH, Knappschaftskrankenhaus Recklinghausen, Academic Teaching Hospital, Ruhr-University Bochum, 45657 Recklinghausen, Germany.
Manuel WeberDepartment of Nuclear Medicine, University Hospital Essen, 45147 Essen, Germany.
Christoph RischplerDepartment of Nuclear Medicine, University Hospital Essen, 45147 Essen, Germany.
Dagmar FührerDepartment of Endocrinology, Diabetes and Metabolism, Clinical Chemistry-Division of Laboratory Research, Endocrine Tumor Center, WTZ/Comprehensive Cancer Center, University Hospital Essen, University of Duisburg-Essen, 45147 Essen, Germany.
Ken HerrmannDepartment of Nuclear Medicine, University Hospital Essen, 45147 Essen, Germany.
Frank WeidemannDepartment of Medicine I, Klinikum Vest GmbH, Knappschaftskrankenhaus Recklinghausen, Academic Teaching Hospital, Ruhr-University Bochum, 45657 Recklinghausen, Germany.
Essen University Hospital · DEKlinikum Vest · DEKnappschaftskrankenhaus Recklinghausen · DEUniversity of Duisburg-Essen · DE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Fabry disease is a multisystem X-linked lysosomal storage disorder caused by a mutation in the alpha-galactosidase A gene. Deficiency or reduced activity of alpha-galactosidase A (GLA) is leading to progressive intracellular accumulation of globotriaosylceramide (GL3) in various organs, including the heart, kidney and nerve system. Cardiac involvement is frequent and is evident as concentric left ventricular hypertrophy. Currently, the standard treatment is enzyme replacement therapy or chaperone therapy. However, early starting of therapy, before myocardial fibrosis has developed, is essential for long-term improvement of myocardial function. For future treatment options, various therapeutic approaches including gene therapy are under development. This review describes the current and potential future therapy options for Fabry cardiomyopathy.

Indexed as

cardiomyopathyFabryoptionstreatment

Identifiers

PMID34300196
PMCPMC8305771
OpenAlexW3179181180

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

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.