Neonatal liver disease associated with placental transfer of anti-mitochondrial antibodies.
BACKGROUND
Anti-mitochondrial antibody is the diagnostic hallmark of primary biliary cirrhosis. Its role in the aetiology of primary biliary cirrhosis is controversial.


METHODS
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.


RESULTS
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.


CONCLUSIONS
The simultaneous disappearance of hepatitis and anti-mitochondrial antibody in the infants suggests a possible causal link between the two.
Hannam
Simon
S
Department of Child Health, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
Bogdanos
Dimitrios-Petrou
DP
Davies
Edward T
ET
Hussain
Munther J
MJ
Portmann
Bernard C
BC
Mieli-Vergani
Giorgina
G
Vergani
Diego
D
D000328:Adult; D000906:Antibodies / Q000276:immunology*; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000276:immunology / Q000473:pathology; D008107:Liver Diseases / Q000276:immunology*; D008297:Male; D008928:Mitochondria / Q000276:immunology*; D010920:Placenta / Q000276:immunology*
D000906:Antibodies