Pregnancy Outcome after Exposure to Migalastat for Fabry Disease: A Clinical Report

Figure 3

Histology and transmission electron microscopy of kidney biopsy specimens obtained (a) in 2005, one month after birth of first child (second pregnancy) and (b) in 2014, during pregnancy with second child (third pregnancy). AFOG, acid fuchsin orange G; IgA, immunoglobulin A; IgG, immunoglobulin G; PAS, periodic acid-Schiff; TEM, transmission electron microscopy. (a) The first renal biopsy (2005) shows a diffuse, segmentally accentuated mesangial matrix and mesangial cell proliferation (PAS and AFOG) in >50% of the glomeruli, and segmentally obliterated capillary loops adherent to the Bowman’s capsule (AFOG). There is dominant segmental C3 deposition (C3) in the absence of IgG and IgA. Podocytes show characteristic lamellar and zebroid bodies by TEM; however, the classical appearance of “foamy” podocytes was less dominant by light microscopy (PAS) due to the segmental nature of the pathological changes. (b) The second renal biopsy (2014) shows characteristic foamy macrophages (PAS and AFOG) as well as segmental mesangial matrix and mesangial cell proliferation. Dominant C3 deposits (C3) correspond to mesangial electron dense deposits (*) by TEM (right) while almost all podocytes contain lamellar and zebroid inclusion bodies (a).

(a)

(b)