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. Author manuscript; available in PMC: 2011 Apr 1.
Published in final edited form as: Mol Genet Metab. 2009 Dec 11;99(4):396–407. doi: 10.1016/j.ymgme.2009.12.003

Fig. 7. Evaluation of aorta from a patient with MPS VII.

Fig. 7

This patient was the first to be diagnosed with MPS VII [19, 49]. A. Chest radiograph. At 18 years-of-age, a posterior-anterior radiograph of this patient demonstrates dilatation of the ascending aorta, as indicated by the black arrows. B to D. Elastin stain. The patient died at 19 years of age and an autopsy was performed. Sections of the aorta were stained with VVG. B. Atheroma. A low power view demonstrated a large atheroma (arrow) on the inner surface of the aorta. Scale bar = 400 µm. C. Lysosomal storage at low power. The white arrow identifies a region in the inner third of the aorta with lysosomal storage and elastin fragmentation. The intima is at the upper right and the adventitia is at the lower left. Scale bar = 200 µm. D. Lysosomal storage and elastin fragmentation at high power. A high power image was obtained from the inner aortic media. The black arrow identifies fragmented elastin while the white arrowhead identifies the clear-appearing lysosomal storage. Scale bar = 25 µm.