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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Severe hepatopathy and neurological deterioration after start of valproate treatment in a 6-year-old child with mitochondrial tryptophanyl-tRNA synthetase deficiency

Fig. 1

Light and electron microscopy. Legend: (a) Cytokeratin 7 immunostaining is positive in numerous small cells throughout the liver parenchyma. Some form abortive bile ducts. Their proliferation is proof of active liver regeneration. b Inclusions in macrophages indicate phagocytosis of debris from damage. PAS after diastase digestion of glycogen; nuclear counterstain with hematoxylin. Parenchymal cells at the right show large empty circular spaces, these are drops of dissolved fat. c Hepatocytes show mitochondria (rounded granules) with different degrees of brown reaction product of cytochrome c oxidase activity. Nuclei are unstained. 2 μm plastic section, after postosmication. Obj. 100×, oil immersion. d Mitochondrial mosaic observed by electron microscopy after reaction for cytochrome c oxidase activity. Mitochondria in the hepatocyte at left show dark reaction product in their cristae. Three unstained round profiles in this cell are peroxisomes. Also seen are two large fat globules that are partly dissolved. The other two hepatocytes have many mitochondria. Their cristae show little or no reaction product. The dark granules in the cytoplasm of all three cells are glycogen rosettes × 7000

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