Impact of

Fabry disease galactosidases globotriaosylceramide humans prevalence

Journal

Circulation. Genomic and precision medicine
ISSN: 2574-8300
Titre abrégé: Circ Genom Precis Med
Pays: United States
ID NLM: 101714113

Informations de publication

Date de publication:
04 Dec 2023
Historique:
medline: 4 12 2023
pubmed: 4 12 2023
entrez: 4 12 2023
Statut: aheadofprint

Résumé

The diagnosis of Fabry disease (FD) has relevant implications related to the management. Thus, a clear assignment of We searched the EMBASE and PubMed databases on February 21, 2023. Observational studies evaluating the prevalence of FD and reporting the identified Of the 3941 studies identified, 110 met the inclusion criteria. The pooled prevalence of FD was significantly different according to the clinical setting and criteria used for the pathogenicity assessment. Using the American College of Medical Genetics and Genomics criteria, the pooled prevalence was 1.2% in patients with left ventricular hypertrophy/hypertrophic cardiomyopathy (26 studies; 10 080 patients screened), 0.3% in end-stage renal disease/chronic kidney disease (38 studies; 62 050 patients screened), 0.7% in stroke (25 studies; 15 295 patients screened), 0.7% in cardiac conduction disturbance requiring pacemaker (3 studies; 1033 patients screened), 1.0% in small-fiber neuropathy (3 studies; 904 patients screened), and 0.01% in newborns (15 studies; 11 108 793 newborns screened). The pooled prevalence was different if the This systematic review and meta-analysis describe the prevalence of FD among newborns and high-risk populations, highlighting the need for a periodic reassessment of the URL: https://crd.york.ac.uk/PROSPERO/; Unique identifier: CRD42023401663.

Sections du résumé

BACKGROUND UNASSIGNED
The diagnosis of Fabry disease (FD) has relevant implications related to the management. Thus, a clear assignment of
METHODS UNASSIGNED
We searched the EMBASE and PubMed databases on February 21, 2023. Observational studies evaluating the prevalence of FD and reporting the identified
RESULTS UNASSIGNED
Of the 3941 studies identified, 110 met the inclusion criteria. The pooled prevalence of FD was significantly different according to the clinical setting and criteria used for the pathogenicity assessment. Using the American College of Medical Genetics and Genomics criteria, the pooled prevalence was 1.2% in patients with left ventricular hypertrophy/hypertrophic cardiomyopathy (26 studies; 10 080 patients screened), 0.3% in end-stage renal disease/chronic kidney disease (38 studies; 62 050 patients screened), 0.7% in stroke (25 studies; 15 295 patients screened), 0.7% in cardiac conduction disturbance requiring pacemaker (3 studies; 1033 patients screened), 1.0% in small-fiber neuropathy (3 studies; 904 patients screened), and 0.01% in newborns (15 studies; 11 108 793 newborns screened). The pooled prevalence was different if the
CONCLUSIONS UNASSIGNED
This systematic review and meta-analysis describe the prevalence of FD among newborns and high-risk populations, highlighting the need for a periodic reassessment of the
REGISTRATION UNASSIGNED
URL: https://crd.york.ac.uk/PROSPERO/; Unique identifier: CRD42023401663.

Identifiants

pubmed: 38047356
doi: 10.1161/CIRCGEN.123.004252
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e004252

Auteurs

Emanuele Monda (E)

Department of Translational Medical Sciences, Inherited and Rare Cardiovascular Diseases, University of Campania Luigi Vanvitelli, Naples, Italy (E.M., G.D., M.R., G.L.).

Gaetano Diana (G)

Department of Translational Medical Sciences, Inherited and Rare Cardiovascular Diseases, University of Campania Luigi Vanvitelli, Naples, Italy (E.M., G.D., M.R., G.L.).

Francesca Graziani (F)

Department of Cardiovascular Sciences, IRCCS, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy (F.G.).

Marta Rubino (M)

Department of Translational Medical Sciences, Inherited and Rare Cardiovascular Diseases, University of Campania Luigi Vanvitelli, Naples, Italy (E.M., G.D., M.R., G.L.).

Athanasios Bakalakos (A)

Institute of Cardiovascular Science, University College London, United Kingdom (E.M., A.B., P.M., G.L.).

Ales Linhart (A)

2nd Department of Medicine, Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic (A.L.).

Dominique P Germain (DP)

Division of Medical Genetics, APHP Paris-Saclay University, University of Versailles, Montigny-le-Bretonneux, France (D.P.G.).

Maurizio Scarpa (M)

Regional Coordinator Centre for Rare Diseases, University Hospital of Udine, Italy (M.S.).

Elena Biagini (E)

Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero, Universitaria di Bologna, Italy (E.B.).

Maurizio Pieroni (M)

Institute of Cardiovascular Science, University College London, United Kingdom (E.M., A.B., P.M., G.L.).
Cardiovascular Department, San Donato Hospital, Arezzo, Italy (M.P.).

Perry Mark Elliott (PM)

Institute of Cardiovascular Science, University College London, United Kingdom (E.M., A.B., P.M., G.L.).

Giuseppe Limongelli (G)

Department of Translational Medical Sciences, Inherited and Rare Cardiovascular Diseases, University of Campania Luigi Vanvitelli, Naples, Italy (E.M., G.D., M.R., G.L.).
Institute of Cardiovascular Science, University College London, United Kingdom (E.M., A.B., P.M., G.L.).

Classifications MeSH