“…At the morphological level, the most striking alterations have been found in these fibroblasts and other cell types of mesenchymal origin, which develop a lysosomal storage phenotype characterized by the accumulation of phase-dense inclusions that gave rise to the term “inclusion-cell disease” (Leroy and DeMars, 1967; Kenyon and Sensenbrenner, 1971; Aula et al , 1975; Martin et al , 1975; Nagashima et al , 1977). By contrast, other cell types exhibit few, if any, morphological alterations, including most cells of the hepatic parenchyma, CNS, and muscle (Kenyon et al , 1973; Martin et al , 1975, 1984; Nagashima et al , 1977). Interestingly, a more recent report described striking vacuolization at a novel site: the serous secretory cells of the pancreas and salivary glands of several infants with MLII (Elleder and Martin, 1998).…”