J Nephropathol. 2020;9(4): e36.
doi: 10.34172/jnp.2020.36

Scopus ID: 85085333517
  Abstract View: 421
  PDF Download: 205


SARS-CoV-2 and Fabry nephropathy: potential risks and the pathophysiological perspective

Hernán Trimarchi 1 * ORCID logo

1 Nephrology Service, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
*Corresponding author: Hernán Trimarchi, Email: htrimarchi@hotmail.com


Fabry disease is an X-linked disorder due to mutations in alpha-galactosidase A gene. It affects the kidney in virtually all patients with classical and some late onset variants. Podocytes, endothelial cells, vascular smooth muscle, tubular and mesangial cells are involved in different ways. Proteinuria and chronic kidney disease are the result of the progressive accumulation of the enzyme substrates globotriaosylceramide (GB3) and lyso-GB3 in the cytoplasm of these cells (mainly in lysosomes), which leads to cellular and organ dysfunction and eventually renal failure and end-stage kidney disease. Specific enzyme replacement therapy and pharmacological chaperone are at present the main therapeutic approach. After enzyme infusion, the delivered enzyme is differentially uptaken by kidney cells in three different ways: By Mannose-6-phosphate receptor, megalin and sortilin. The delivered enzyme gradually clears cells from the accumulation of the glycosphingolipids and contributes to a cellular healthier status. The recent pandemic caused by SARS-CoV-2 has led to the collapse of health systems around the world and to thousands of deaths. Kidney involvement has been reported to range from proteinuria to acute kidney injury, 30% of which may require renal replacement therapy. In this review the potential causes for which Fabry patients should be at increased risk and the necessity not to discontinue therapy are discussed.
Keywords: Fabry nephropathy, SARS-CoV-2, Podocyte, Endothelium, Proteinuria, Angiotensin-converting enzyme-2

Implication for health policy/practice/research/medical education:

Fabry patients may be at increased risk of kidney morbidity during SARS-CoV-2 infection. Proteinuria and blood pressure may increase, and sodium and bicarbonate balance may be threatened, particularly at advanced stages of chronic kidney disease.

Please cite this paper as: Trimarchi H. SARS-CoV-2 and Fabry nephropathy: potential risks and the pathophysiological perspective. J Nephropathol. 2020;9(4):e36. DOI: 10.34172/jnp.2020.36.

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Abstract View: 421

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Submitted: 16 Apr 2020
Accepted: 02 May 2020
ePublished: 11 May 2020
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