Liver ultrastructure and diagnosis of inborn metabolic disorders

Vanhoof, F.; Roels, Frank

doi:10.1016/0739-6260(89)90011-7

Cited by 7 publications

(2 citation statements)

References 6 publications

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“…4). Similar phenomena were found in a patient with the Pi Z-Z form of a,-antitrypsin deficiency (20) and in an infant with conjugated hyperbilirubinemia (21). In the infant this material had been shown immunochemically to be a,-antitrypsin.…”

Section: Resultssupporting

confidence: 73%

Peroxisomes in cirrhosis of the human liver: A cytochemical, ultrastructural and quantitative study

1993

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Hepatocellular peroxisomes in 32 patients with cirrhosis were studied by means of catalase cytochemical and morphometric analysis. Seven normal human livers were used as controls. The severity of the cirrhosis was determined with the Child-Turcotte criteria. Under the light microscope, a decrease in catalase staining was observed in 12 livers. Staining showed a weak inverse correlation with severity of the cirrhotic process. Peroxisomes revealed a perinuclear configuration in 24 patients. Morphometric analysis of peroxisomes was performed on 14 cirrhotic livers and revealed a near doubling of the number of organelles, with a compensatory decrease in mean peroxisomal diameter: no appreciable change in total volume of the peroxisome compartment was found. Cytoplasmic invaginations, protrusions and gastruloid cisternae were sparse. Apparently, peroxisomal proliferation in liver cells appeared early in the cirrhotic process. In all 10 livers with a perinuclear configuration of the peroxisomes that were processed for electron microscopy, a morphometrically confirmed increase in the number of peroxisomes was observed. Peroxisomes frequently showed transparent matrical spots and angular profiles. In two patients nucleoid-containing peroxisomes were observed. Although variation between individual patients was high, peroxisomal changes were observed in each cirrhotic liver. No relationship between morphological or morphometric alterations in peroxisomal compartment on one side and the severity of the disease or the type of cirrhotic nodules on the other side was observed. (HEPATOLOGY 1993;17:404-410.) Peroxisomes are ubiquitous cell organelles containing enzymes that intervene in several metabolic pathways (1). Their indispensable role is stressed by the existence of a group of inherited peroxisomal disorders: patients with one or more deficient peroxisomal enzymes exhibit

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Section: Resultssupporting

confidence: 73%

Peroxisomes in cirrhosis of the human liver: A cytochemical, ultrastructural and quantitative study

1993

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“…Images are shown in Dingemans et al (1983), Espeel et al (1991), Gatfield et al (1968), Ghatak et al (1981), Haas et al (1982), Hughes et al (1990), Kerckaert et al (1988), Manz et al (1980), Mooi et al (1983), Pfeifer and Sandhage (1979), Poulos et al (1984), Roels et al (1986Roels et al ( , 1991aRoels et al ( , 1993bRoels et al ( , 1995, Schaumburg et al (1974Schaumburg et al ( , 1975Schaumburg et al ( , 1977, Scotto et al (1982), Ulrich et al (1978), Vamecq et al (1986), Van Hoof and Roels (1989), Van Maldergem et al (1992).…”

Section: Trilamellar Inclusionsmentioning

confidence: 99%

Liver and chorion cytochemistry

Roels

Prest

Pestel

1995

J of Inher Metab Disea

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Microscopic visualization of peroxisomes in chorionic villus cytotrophoblast and in biopsy and autopsy samples of liver and kidney, the presence of enlarged liver macrophages containing lipid droplets insoluble in acetone and n-hexane as well as polarizing inclusions formed by stacks of trilamellar sheets are of diagnostic value in peroxisomal disorders. Methods are presented for evaluating these structures by light microscopy; trilamellar inclusions are only detected by electron microscopy. Macrophage features are preserved in archival paraffin blocks. In adrenal cortex, insoluble lipid, polarizing inclusions and trilamellar structures should be looked for. The stains are easily reproducible, and all reagents are commercially available.

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