Abstract
Extract: In the liver of a 25-month-old patient with mucolipidosis II (I-cell disease), intracellular vacuoles caused marked distension of portal mononuclear cells, sinusoidal Kupffer cells, and granulomatous epithelioid cells. It was determined by electronmicroscopy that these vacuoles were limited by single membranes and contained either fine fibrillogranular material, membranous lamellae, or lipoid globules. Hepatic parenchymal cells were only slightly affected by the storage process, and abnormal extracellular material was not evident. Histochemical staining revealed sudanophilic inclusions within Kupffer cells and demonstrated increased acid phosphatase activity within Kupffer cells and epithelioid cells.
Speculation: The observed intracellular storage vacuoles are possibly derived from altered lysosomes which have accumulated excesses of both acid mucopolysaccharides and glycolipids. The unusual occurrence of epithelioid cell granulomas in this case may reflect the impaired immunologic function of macrophages laden with storage substances.
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Kenyon, K., Sensenbrenner, J. & Wyllie, R. Hepatic Ultrastructure and Histochemistry in Mucolipidosis II (I-Cell Disease). Pediatr Res 7, 560–568 (1973). https://doi.org/10.1203/00006450-197306000-00003
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DOI: https://doi.org/10.1203/00006450-197306000-00003
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