Evidence map›Paper›PMID 39669233›Full record

ReviewGenetics in medicine open2023

New insights in efficacy of different ERT dosages in Fabry disease: Switch and switch-back studies data following agalsidase beta shortage. Update of systematic review.

Eleonora Riccio, Carlo Garofalo, Ivana Capuano, Pasquale Buonanno, Guido Iaccarino, Teodolinda Di Risi, Massimo Imbriaco, Federica Riccio Cuomo, Antonio Pisani

Abstract readReview
In one paragraph

Review in Genetics in medicine open, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed, 1 pooled it
–field-weighted citation impact
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

4 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Review
  3. Fabry Disease: A Focus on the Role of Oxidative Stress.Antioxidants (Basel, Switzerland) · 2026
    Review
  4. Review
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

9 authors.

Eleonora RiccioInstitute for Biomedical Research and Innovation, National Research Council of Italy (IRIB CNR), Palermo, Italy.
Carlo GarofaloDivision of Nephrology, University of Campania "Luigi Vanvitelli," Naples, Italy.
Ivana CapuanoChair of Nephrology, Department of Public Health, University Federico II of Naples, Naples, Italy.
Pasquale BuonannoDepartment of Neurosciences, Reproductive and Odontostomatological Sciences, University of Naples Federico II, Naples, Italy.
Guido IaccarinoDepartment of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy.
Teodolinda Di RisiCEINGE - Biotecnologie Avanzate, Via Gaetano Salvatore, Naples, Italy.
Massimo ImbriacoDepartment of Radiology, Federico II University of Naples, Naples, Italy.
Federica Riccio CuomoUniversisad Anáhuac Puebla, San Andrés Cholula, Puebla, México.
Antonio PisaniChair of Nephrology, Department of Public Health, University Federico II of Naples, Naples, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

In 2016, a systematic review and a meta-analysis of existing data on the effects of switch from agalsidase beta to alfa in patients with Fabry disease showed that the switch was well tolerated and associated with stable disease progression. However, additional evidence that supports the need for an update of the review on the long-term effects of switching to agalsidase alfa, with a mention on the effects of reswitch to agalsidase beta, has emerged. Relevant papers were identified on PubMed, Cochrane, ISI Web, and Scopus databases from September 2015 to December 2021. Analyzed parameters were clinical events, changes in organ function or structure, disease related symptoms, lyso-globotriasolylceramide 3 (lyso-Gb3) plasma levels, presence of antidrug antibodies, and adverse effects. In total, 15 publications were evaluated, with a total of 353 subjects. The results of the review confirmed some points of the previous analysis with some new important information. After the switch from agalsidase beta to alfa, an increased number of clinical events, a significant loss of renal function, and an increase in lyso-Gb3 levels were reported; conversely, lyso-Gb3 levels decreased after the switch from agalsidase alfa to beta. The results confirm the importance of dose and recommend that patients be monitored through intensified surveillance, including lyso-Gb3 levels every 6 months.

Indexed as

Agalsidase alfaAgalsidase betaEnzymatic replacement therapyFabry diseaseSwitch

Identifiers

PMID39669233
PMCPMC11613606

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Registered trials

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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.