In this 72‐year‐old male patient, large amounts of amyloid substances were deposited in the mesenchymal organs, but not in the parenchymal organs. Histochemical and electron microscopic studies revealed large amounts of amyloid substances and amyloid fibrils in the skeletal and smooth muscle tissues. In the skeletal muscle tissues, where large amounts of amyloid substances were deposited, glycosaminoglycan was also markedly deposited, especially chondroitin‐4‐sulfuric acid. The concentrations of silicon were also markedly increased in the amyloid fibrils. The concentrations of nickel and cobalt were increased but those of zinc were decreased in all organs measured, although both nickel and cobalt in another case of amyloidosis experienced were within normal values.
Liver biopsy materials of two siblings with type IV glycogenosis were studied by light and electron microscopy. Biochemical analysis was added using autopsy material in one of the two cases. Two kinds of polysaccharides were noted not only in the cardiac muscle, skeletal muscles, smooth muscles and reticuloendothelial cells, but also in the neutrophils and platelets. One was glycogen and the other was sMlar to amylopectin.Ultrastructurally, a large amount of fibrils, 60 A in width, glycogen rosettes and glycogen granules were detected in those cells. Branching glycosyltransferase deficiency was biochemically confirmed in one case examined, ACTA PATH. JAP. 25: 613-633, 1975. TYPE IV GLYCOGENOSISAct% Path. Jap. 25(5): 1975T. ISHIHARA a t at. 615 616 TYPE IV QLYCOQENOSIS Acta Path. Jap. the spleen 3 cm below the left costal margin. no lymphadenopathy.Ascites was present. There was Laboratory Jindings ; A hemogram showed slight anemia and neutrophilia. The glutamic oxalacetic transaminase (SCOT) was 148 u, the glutamic pyruvic transaminxse (sGPT) 81 u and the lactic dehydrogenase (LDH) 400 u. Fasting blood glucose was within normal limits. A glucose tolerance test was not done.The specimens were fixed in 10% buffered formalin. The paraffin sections were stained with hematoxylin-eosin and periodic acid Schiff (PAS) with and without diastase digestion.On the ninth day a liver biopsy was performed.Light 3microscopic $findings : Moderate cirrhosis was noted in the specimen.Hepatic parenchymal cells were enlarged and contained abundant slightly basophilic material strongly staining with PAS. This PAS positive material took various forms, such as spherical or polygonal, and its central core was diastase resistant.The Kupffer cells were swollen and contained slightly basophilic material in the cytoplasm. This material was strongly stained with PAS and showed diastase, resistancy. I n the Glisson's sheath proliferation of the bile duct, infiltration of lymphocytes and increase of connective tissue were detected. The proliferated connective tissue contained numerous macrophages with identical PAS positive diastase resistant material (Big. 2).However, the peripheral portion of it was not diastase resistant. 25(5): 1975 T. ISEIEARA e l al. 617 618 TYPE IV OLYCOOENOSIS Acta Path. Jap.
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