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Intra-Hepatic Bile Duct Primary Cilia In Biliary Atresia

Frassetto, Roberta ; Parolini, Filippo ; Marceddu, Salvatore ; Satta, Giulia ; Papacciuoli, Valeria ; Pinna, Maria Antonia ; Mela, Alessandra ; Secchi, Giannina ; Galleri, Grazia ; Manetti, Roberto ; Bercich, Luisa ; Villanacci, Vincenzo ; Dessanti, Antonio ; Antonucci, Roberto ; Tanda, Francesco ; Alberti, Daniele ; Schwarz, Kathleen B ; Clemente, Maria Grazia

Hepatology research, 2018-01 [Periódico revisado por pares]

Netherlands

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  • Título:
    Intra-Hepatic Bile Duct Primary Cilia In Biliary Atresia
  • Autor: Frassetto, Roberta ; Parolini, Filippo ; Marceddu, Salvatore ; Satta, Giulia ; Papacciuoli, Valeria ; Pinna, Maria Antonia ; Mela, Alessandra ; Secchi, Giannina ; Galleri, Grazia ; Manetti, Roberto ; Bercich, Luisa ; Villanacci, Vincenzo ; Dessanti, Antonio ; Antonucci, Roberto ; Tanda, Francesco ; Alberti, Daniele ; Schwarz, Kathleen B ; Clemente, Maria Grazia
  • É parte de: Hepatology research, 2018-01
  • Descrição: The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intra-hepatic bile duct cilia (IHBC) in BA at the diagnosis and correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM). Surgical liver biopsies taken at the diagnosis from 22 BA infants (age range: 39 - 116 days) and from 8 children with non-BA chronic cholestasis (age range: 162 days -16,8 years) were evaluated for IHBC, both by immunofluorescence (IF) and by SEM. A minimum 18 -month-follow-up after surgery was available for all patients. By IF, cilia were present in 6/8 (75%) non-BA but only in 3/22 (14%) BA, while cilia were reduced or absent in 19/22 (86%) BA and 2/8 (25%) non-BA livers (p<0.01). In BA, cilia presence was found associated with clearance of jaundice at 6-month-follow-up (p<0.05). However, high overall survival rates with native liver, >90% at 12 months and >70% at 24 months post-surgery, were recorded regardless of cilia presence/absence at the diagnosis. Electron microscopy was able to detect bile ducts and cilia in routine liver biopsies, revealing significant abnormalities in 100% BA livers. The presence of IHBC in BA livers at the diagnosis was associated with resolution of cholestasis although was not predictive of short-term survival with native liver. SEM represents a powerful new tool to study routine liver biopsies in biliary disorders. Cilia dysfunction in BA pathogenesis and/or disease progression warrants further investigation.
  • Editor: Netherlands
  • Idioma: Inglês
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  • Realização: ABCD-USP USP   Logos de Redes Sociais: Freepik

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