1. Neonatal Liver Disease Associated with Placental Transfer of Anti-mitochondrial Antibodies
- Author
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Diego Vergani, Giorgina Mieli-Vergani, Bernard Portmann, Dimitrios-Petrou Bogdanos, E T Davies, Simon Hannam, and Munther Hussain
- Subjects
Adult ,Male ,Placenta ,Immunology ,Fluorescent Antibody Technique ,Immunofluorescence ,Antibodies ,Primary biliary cirrhosis ,medicine ,Humans ,Immunology and Allergy ,Hepatitis ,biology ,medicine.diagnostic_test ,business.industry ,Liver Diseases ,Infant, Newborn ,Autoantibody ,Infant ,medicine.disease ,Pyruvate dehydrogenase complex ,Isotype ,Mitochondria ,Neonatal hepatitis ,Liver ,biology.protein ,Female ,Antibody ,business - Abstract
Anti-mitochondrial antibody is the diagnostic hallmark of primary biliary cirrhosis. Its role in the aetiology of primary biliary cirrhosis is controversial.Two cases of neonatal hepatitis seropositive for anti-mitochondrial antibody are described. Anti-mitochondrial antibody Ig isotype and epitopic specificity were investigated by immunofluorescence and enzyme immunoassays.In both infants anti-mitochondrial antibody was of the G class, mainly G1 and G3 subclasses, and reacted with two synthetic peptides reproducing major M2 epitopicregions: innerlipoyl domain pyruvate dehydrogenase complex (PDC)-E2(162-176) and PDC-E3 binding protein (PDC-E3BP)86-100. One infant also reacted with outer lipoyl domain PDC-E2(35-49), and 2-oxoglutarate dehydrogenase complex (OGDC)-E2(99-113). An identical pattern of reactivity was present in their mothers, indicating the maternal origin of the antibodies. Anti-mitochondrial antibody disappeared in the infants with the disappearance of the liver pathology.The simultaneous disappearance of hepatitis and anti-mitochondrial antibody in the infants suggests a possible causal link between the two.
- Published
- 2002
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