Fig. 2.

(a) Electromicrophotogaph from case 6 (Table 1) shows an hepatocyte with dilated cisternae of the ER containing fluffy semi-electron dense material corresponding to the classical appearance of AAT. The largest inclusions contain a large crystalline electrondense material. In addition to the crystalline structures, AAT inclusions contained fine or coarse electrondense granules quite similar to those of other AAT inclusions. This material was also displaying the peaks of calcium on EPMA (10.000 X). (b) Case 7 (a two years old boy with no visible calcium precipitates in H.E. stained preparations). The electronmicrophotograph shows dilated cisternae of ER. At least three AAT inclusions contain sparse electrondense granular precipitates similar to Fig. 2a. A few electrondense lysosomes with lipid inclusions are also seen (8000 X)