Table 2

Characteristics of paediatric patients with antimitochondrial antibody (AMA) positivity in primary biliary cirrhosis (PBC) and other liver or non-liver related diseases
Reference Age Sex Pathology Antibodies Detected Histology
Zamfir et al. 6 yrs M ITP AMA, ANA -
15 yrs M AHA AMA, ANA -
Gregorio et al. 12 yrs F AIH AMA Portoseptal mononuclear cell infiltration and extensive interface hepatitis, with lymphocytic periportal necrosis. Consistent with chronic hepatitis.
Hannam et al. Birth F NNH AMA
Birth M NNH AMA Cholestasis, hepatitis, mild cholangiolitic changes and multi-nucleated giant hepatocytes. Mild portal fibrosis.
Aoki et al. 6 months M IL-2Rα deficiency AMA Intense mononuclear lymphocyte infiltration of the portal tracts with preservation of the lobular architecture.
Tsuda et al. 11 yrs M IPEX AMA -
Melegh et al. 6 yrs F PBC AMA At 6 years: Fibrotic degeneration of the portal tracts, loss of bile ducts, ductal proliferation, periportal hepatocytes separated by mononuclear inflammatory cells.
Dahlan et al. 11 yrs F PBC AMA

At 16 years: Stage II PBC

At 21 years: Stage IV PBC (explanted liver)

15 yrs F PBC AMA, ANA At 17 years: Stage II PBC

Detailed description of cases is given within the text. ITP: Idiopathic thrombocytopaenic purpura; AHA: Autoimmune haemolytic anaemia; AIH: Autoimmune hepatitis; NNH: Neonatal hepatitis; IPEX: Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome; ANA: Antinuclear antibody.

Invernizzi et al.

Invernizzi et al. BMC Gastroenterology 2012 12:92   doi:10.1186/1471-230X-12-92

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