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Electron microscopy of liver diseases- 2020
- 1. Electron Microscopy in Liver Diseases Dr. Heena Wadhwa Senior Resident Department of Pathology
- 2. Electron Microscope 2 Types • TEM - Transmission Electron Microscope • SEM - Scanning Electron Microscope
- 4. Technical Aspects • Submitting tissue in 2.5% glutaraldehyde for electron microscopy. • The specimen should not be larger than 1 mm in each direction to allow for good penetration of the fixative.
- 5. Indications • Investigating genetic and metabolic diseases, viral infection. • Not otherwise identified by light microscopy or serology. • Certain drug-induced liver injuries (e.g., Valproate toxicity)
- 8. • Scanning electron micrograph (SEM) showing several cell types. Hepatocytes (H) contain a nucleus (N), and at the junction between cells the bile canaliculus (bc) and the intercellular surface are clearly defined. The sinusoidal surface is seen and in the sinusoidal area a Kupffer cell (Kc), endothelial cell (Ec) and two perisinusoidal cells (psc) are present.
- 12. Disorders of Lipoprotein and Lipid Metabolism • α-Galactosidase A deficiency (Fabry disease) • The Gangliosidoses • Wolman and Cholesterol ester storage diseases • Glycosyl ceramide lipidosis (Gaucher disease) • Sphingomyelin lipidosis (Niemann–Pick disease)
- 13. α-Galactosidase A Deficiency (Fabry Disease)
- 14. α-Galactosidase A Deficiency (Fabry Disease)
- 16. Cholesterol Ester Storage Disease Cholesterol ester storage disease. (A) Kupffer cells are markedly hypertrophied and have a foamy, light tan-coloured cytoplasm; their nuclei are pyknotic. Hepatocytes show microvesicular steatosis. (H&E)
- 17. Wolman Disease
- 18. Gauchers Disease
- 21. Disorders of Glycoprotein and Glycolipid Metabolism • Mucopolysaccharidoses • Mucolipidoses
- 23. Mucolipidosis
- 24. Disorders of Carbohydrate Metabolism : Pompes Disease
- 25. Disorders of Amino Acid Metabolism
- 26. Mitochondrial Cytopathy Oxyphilic and microvesiculated hepatocytes are intermingled with groups of smaller hepatocytes with basophilic cytoplasm. Electron micrograph showing densely packed mitochondria separated by small lipid droplets. Note the mitochondrial granular appearance and loss of cristae. (×5800)
- 27. Reyes Syndrome Figure 3.50 Reye syndrome. Electron micrograph showing swollen mitochondria with loss of matrix density and both fragmentation and reduction in the number of cristae. Note absence of dense granules in the mitochondria (×20 000).
- 28. Endoplasmic Reticulum Storage Diseases • α1-Antitrypsin deficiency • Afibrinogenaemia and hypofibrinogenaemia
- 29. Alpha- 1 Antitrypsin Deficiency
- 30. Alpha- 1 Antitrypsin Deficiency
- 32. Peroxisomal Disorders Peroxisomes (microbodies) are seen in hepatocytes as round, ellipsoid, slightly angular or elongated organelles.
- 33. Disorder of Copper Metabolism – Wilsons Disease
- 34. Disorder of Copper Metabolism – Wilsons Disease
- 35. Cholestasis
- 36. Cholestasis
- 37. Disorders of Porphyrin Metabolism : Erythropoietic Protoporphyria Erythropoietic protoporphyria. Electron micrograph showing a mass of radiating, hair-like pigment crystals in a dilated canaliculus.(star burst Erythropoietic protoporphyria. Brown deposits are present in canaliculi, hepatocytes and Kupffer cells.
- 44. Mallory–Denk bodies • Three ultra structurally distinct forms of Mallory–Denk bodies have been described: type I comprise bundles of filaments in parallel arrays; • Type II are seen as clusters of randomly oriented fibrils; • Type III are identified as granular or amorphous substance containing only scattered fibrils.
- 45. Mallory–Denk bodies • Alcoholic Hepatitis • Non-Alcoholic Steatohepatitis • Prolonged Cholestasis • Primary Biliary Cirrhosis • Wilsons Disease • Indian Childhood Cirrhosis • Focal Nodular Hyperplasia • Hepatocellular Carcinoma
- 46. THANKS
Editor's Notes
- X-linked disorder of glycosphingolipid metabolism. a defect in the lysosomal hydrolase, α-galactosidase A (α-GLA)785 which results in accumulation of globotriaosylceramide in tissue lysosomes throughout the body . laminated lysosomal inclusions with a periodicity of 5–6 nm
- 3 types: infantile, juvenile and adult
- absent or a reduced (3–8%) activity of the enzyme lysosomal acid lipase.
- absent or a reduced (3–8%) activity of the enzyme lysosomal acid lipase.708,709
- deficiency in acid β-glucosidase , lysosomal accumulation of glucosylceramide (glucocerebroside), mainly in macrophages. The pale-staining cytoplasm of the large, multinucleated Gauch cells, show by TEM large lysosomes containing long tubules, some twisted and intermingled
- The syndrome of fatty liver and encephalopathy
- The major pathological finding is the absence of peroxisomes in the cerebrohepatorenal syndrome of Zellweger. Conditions with fewer peroxisomes (Table I) comprise Infantile Refsum disease (Fig. 18) whereas abundant organelles were reported in Reye syndrome, alcoholic liver disease, clofibrate and 6-mercaptopurine therapy and cholestatic jaundice of pregnancy
- ferrochelatase, the defective enzyme, catalyses the insertion of iron into protoporphyrin as the final step in haem synthesis. insoluble aggregates of protoporphyrin form crystals in hepatocytes, canaliculi and proximal bile ducts, resulting in ‘black liver disease’