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Fabry disease and COVID-19: international expert recommendations for management based on real-world experience

Authors
 Dawn A Laney  ;  Dominique P Germain  ;  João Paulo Oliveira  ;  Alessandro P Burlina  ;  Gustavo Horacio Cabrera  ;  Geu-Ru Hong  ;  Robert J Hopkin  ;  Dau-Ming Niu  ;  Mark Thomas  ;  Hernán Trimarchi  ;  William R Wilcox  ;  Juan Manuel Politei  ;  Alberto Ortiz 
Citation
 CLINICAL KIDNEY JOURNAL, Vol.13(6) : 913-925, 2020-12 
Journal Title
CLINICAL KIDNEY JOURNAL
ISSN
 2048-8505 
Issue Date
2020-12
Keywords
COVID-19 ; Fabry disease ; SARS-CoV-2 ; chloroquine ; enzyme replacement therapy ; lysosome ; pathogenesis ; prevention
Abstract
The rapid spread of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 has raised questions about Fabry disease (FD) as an independent risk factor for severe COVID-19 symptoms. Available real-world data on 22 patients from an international group of healthcare providers reveals that most patients with FD experience mild-to-moderate COVID-19 symptoms with an additional complication of Fabry pain crises and transient worsening of kidney function in some cases; however, two patients over the age of 55 years with renal or cardiac disease experienced critical COVID-19 complications. These outcomes support the theory that pre-existent tissue injury and inflammation may predispose patients with more advanced FD to a more severe course of COVID-19, while less advanced FD patients do not appear to be more susceptible than the general population. Given these observed risk factors, it is best to reinforce all recommended safety precautions for individuals with advanced FD. Diagnosis of FD should not preclude providing full therapeutic and organ support as needed for patients with FD and severe or critical COVID-19, although a FD-specific safety profile review should always be conducted prior to initiating COVID-19-specific therapies. Continued specific FD therapy with enzyme replacement therapy, chaperone therapy, dialysis, renin-angiotensin blockers or participation to clinical trials during the pandemic is recommended as FD progression will only increase susceptibility to infection. In order to compile outcome data and inform best practices, an international registry for patients affected by Fabry and infected by COVID-19 should be established.
Files in This Item:
T202007219.pdf Download
DOI
10.1093/ckj/sfaa227
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Hong, Geu Ru(홍그루) ORCID logo https://orcid.org/0000-0003-4981-3304
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/183903
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