1969
DOI: 10.1203/00006450-196901000-00003
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Etude ultrastructurelle du foie dans la maladie de Morquio

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Cited by 18 publications
(4 citation statements)
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“…In some of them, a few more osmiophilic spherules, generally adjacent to the membrane, are observed. In Morquio's disease, a similar aspect is observed in Kupffer cells while hepatocytes appear almost normal (34). The ultrastructural aspect is also quite different from that described by Durand et al (10) and also found by us1 in cases of mucopolysaccharidosis by absence of a-fucosidase (38).…”
Section: Discussionmentioning
confidence: 58%
See 1 more Smart Citation
“…In some of them, a few more osmiophilic spherules, generally adjacent to the membrane, are observed. In Morquio's disease, a similar aspect is observed in Kupffer cells while hepatocytes appear almost normal (34). The ultrastructural aspect is also quite different from that described by Durand et al (10) and also found by us1 in cases of mucopolysaccharidosis by absence of a-fucosidase (38).…”
Section: Discussionmentioning
confidence: 58%
“…The atypical cell groups are not mentioned in ultrastructural descriptions of normal liver (26). On the other hand, a similar aspect has been described in Morquio's disease (34). It is possible that technical manipulations may induce membrane ruptures of overloaded cells and consequently a cytoplasmic flow in adjacent spaces.…”
Section: Acta Pudiat Scand 5smentioning
confidence: 93%
“…Extensive vacuolation of hepatocytes has also been noted consistently in many other AMP and glycolipid disorders, including mucopolysaccharidosis types I-III [3,20,33,34], fucosidosis [7,21], G Mlgangliosidosis [28,29], and MLS I [6,22]. Only in mucopolysaccharidosis type IV [30] and in Goldberg's MLS I variant [8] is vacuolation confined to Kupffer cells without hepatocellular alteration.…”
Section: Discussionmentioning
confidence: 99%
“…Liver Kupffer cells and dermal fibroblasts contain clear vacuoles, similar to those described in the first three types of mucopolysaccharidoses. In hepatocytes, however, most of the dense bodies have a normal appearance (Panizon, Sartorelli, and Perona, 1968;Tondeur and Loeb, 1969). Chondrocytes in epiphyseal plates are distended by numerous clear vacuoles (Maynard, Cooper, and Ponseti, 1973).…”
Section: Laboratory and Ultrastructure Datamentioning
confidence: 99%