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. 2022 Feb 28;11(5):1344. doi: 10.3390/jcm11051344

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© 2022 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).

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Pathologic evaluation of myocardial tissue (in pre-hypertrophic cardiomyopathy) and intestinal tissue (with diarrhea and malabsorption) in a 29-year-old man (Case 4) affected by Fab-ry disease before and after 2 years of agalsidase-α. (A–F) show in the myocardium unchanged cardiomyocyte dimensions and vacuolar area in H&E (A,B), immunofluorescence for GL3 (C,D) and electron-microscopy (E,F) comparing pre (A,C,E) and post (B,D,F) findings. Panel (G–J) shows in the intestinal tissue mobilization of Gb3 in immunofluorescence (G,H) and ultrastructur-al examination (J,K), comparing pre (G,J) and post–treatment (H,K) features.