Abstract
In adult cholestatic liver diseases, hepatocytic microfilament accumulation has been considered as a non specific change. By contrast in experimental models (e.g. phalloidin), cholestasis has been ascribed to microfilament accumulation and dysfunction. These changes have received little attention in familial cholestatic diseases affecting children. Therefore, canalicular regions were blindly studied by two examiners (AW, PB) in 3 Byler's Like Syndrome (B) 5 North American Indian Cirrhosis (NAC) 5 Ductular Hypoplasia (DH) 3 uncorrected biliary atresia (BA) and 4 non cholestatic cryptogenic cirrhosis (CC). A rating of (O) to (+++) was applied to canalicular changes; microfilament accumulation; increase in lysosomes. Results were expressed as percent of moderate to severe anomalies (++) and (+++).
In the familial entities (B, DH, NAC) microfilament accumulation (21, 52, 42% respectively) was striking and in B paralleled bile canalicular changes (31%). Lysosomal bodies accumulation was marked in DH (60%) and NAC (43%). In BA and CC microfilaments accumulated (33, 42%) despite little changes in canaliculi (18, 14%). In conclusion, microfilament accumulation appears to precede canalicular changes in early obstruction suggesting an adaptive mechanism. In familial pediatric cholestasis both canalicular changes and microfilament accumulation are seen but ultrastructural studies do not permit to conclude about microfilament contractility.
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Weber, A., Tuchweber, B., Beauparlant, J. et al. ULTRASTRUCTURAL HALLMARKS OF SOME CHOLESTATIC PEDIATRIC DISEASES. Pediatr Res 18 (Suppl 4), 216 (1984). https://doi.org/10.1203/00006450-198404001-00741
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DOI: https://doi.org/10.1203/00006450-198404001-00741