PMC9434010_01
Microscopic findings of the multisystemic GACI compared with normal structures (arrows): (A) Calcifications of the medium-sized arteries of the lung. (B) Intimal calcifications of the arteries of the tongue. (C) Calcifications of the hepatocytes. (D) Necrosis of the brain parenchyma. (E) Hydropic changes of the myocardial fibers.
fped-10-922379-g0001.jpg
PMC9434010_01_fped-10-922379-g0001.jpg
F1
Protein dystrophy was seen in the myocardial fibers (Figure 1), without signs of myocardial ischemia or histological modifications of the coronary arteries. The intima of large and medium-sized arteries showed marked thickness with degenerative calcifications of lamina elastica interna (Figure 1). Muscle Actin (SMA) and Cluster of Differentiation 34 (CD34) was predominant, without proliferation of Cluster of Differentiation 31 (CD31) -marked endothelial cells (Figure 2). Myointimal calcifications at the level of the hepatic vessels associated with necrosis of the perivascular hepatocytes (Figure 1). Microscopically, the calcification of the intimal layer of the arteries was identified as similar to that of the pulmonary vessels (Figure 1). Cerebral edema, tonsillar herniation, and extensive areas of necrosis were seen in the brain parenchyma (Figure 1).
Histological modifications of the pulmonary arteries (arrows). (A) Calcifications of the lamina elastica; (B,C) Thickening of the intima with calcifications and stenosis of the arterial lumen. (D) The proliferated cells are negative for CD31. (E,F) Compared with normal arteries, marked proliferation of myofibroblasts is emphasized with smooth muscle actin (SMA).
fped-10-922379-g0002.jpg
PMC9434010_01_fped-10-922379-g0002.jpg
F2
Muscle Actin (SMA) and Cluster of Differentiation 34 (CD34) was predominant, without proliferation of Cluster of Differentiation 31 (CD31) -marked endothelial cells (Figure 2). Myointimal calcifications at the level of the hepatic vessels associated with necrosis of the perivascular hepatocytes (Figure 1).