Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis
Background and Aim. Neonatal hemochromatosis (NH) is characterised by severe liver injury and extrahepatic siderosis sparing the reticuloendothelial system. Its aetiology is obscure, although it has been proposed as an alloimmune disease, resulting from immunological reaction to self-antigens (alloa...
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| Format: | Article |
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Wiley
2013-01-01
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| Series: | Clinical and Developmental Immunology |
| Online Access: | http://dx.doi.org/10.1155/2013/642643 |
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| author | Daniel S. Smyk Maria G. Mytilinaiou Tassos Grammatikopoulos A. S. Knisely Giorgina Mieli-Vergani Dimitrios P. Bogdanos Diego Vergani |
| author_facet | Daniel S. Smyk Maria G. Mytilinaiou Tassos Grammatikopoulos A. S. Knisely Giorgina Mieli-Vergani Dimitrios P. Bogdanos Diego Vergani |
| author_sort | Daniel S. Smyk |
| collection | DOAJ |
| description | Background and Aim. Neonatal hemochromatosis (NH) is characterised by severe liver injury and extrahepatic siderosis sparing the reticuloendothelial system. Its aetiology is obscure, although it has been proposed as an alloimmune disease, resulting from immunological reaction to self-antigens (alloantigens) which the body recognizes as foreign. We studied an infant with NH and his mother whose sera contained antimitochondrial antibody (AMA), the hallmark of primary biliary cirrhosis (PBC). Material and Methods. To investigate the origin of AMA in the infant, we studied isotype distributions in serum from the mother and infant. Serum samples were obtained at diagnosis of NH, after liver transplantation (LT; age 1 month), and over the ensuing 17 months. Results. At NH diagnosis, infant and maternal serum contained AMA of the IgG isotype, predominantly of the G3 and G1 subclasses. AMA strongly reacted against the pyruvate dehydrogenase complex E2 subunit (PDC-E2), the major PBC-specific AMA autoantigen. Anti-PDC-E2 responses in both infant and mother declined over time, being present 2 months after LT (mother and child) and absent 10 months later (mother) and 17 months later (child). Conclusion. The association of maternally transferred IgG1 and IgG3 subclass AMA with the appearance of liver damage in an infant with NH may suggest a causal link between antibody and liver damage. |
| format | Article |
| id | doaj-art-f281c03c02e0427cbb282b5d3155f9e5 |
| institution | Kabale University |
| issn | 1740-2522 1740-2530 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical and Developmental Immunology |
| spelling | doaj-art-f281c03c02e0427cbb282b5d3155f9e52025-02-03T06:14:10ZengWileyClinical and Developmental Immunology1740-25221740-25302013-01-01201310.1155/2013/642643642643Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal HaemochromatosisDaniel S. Smyk0Maria G. Mytilinaiou1Tassos Grammatikopoulos2A. S. Knisely3Giorgina Mieli-Vergani4Dimitrios P. Bogdanos5Diego Vergani6Institute of Liver Studies, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKInstitute of Liver Studies, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKPaediatric Liver, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKInstitute of Liver Studies, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKInstitute of Liver Studies, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKInstitute of Liver Studies, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKInstitute of Liver Studies, GI and Nutrition Centre, King’s College London School of Medicine at King’s College Hospital, Denmark Hill Campus, London SE5 9RS, UKBackground and Aim. Neonatal hemochromatosis (NH) is characterised by severe liver injury and extrahepatic siderosis sparing the reticuloendothelial system. Its aetiology is obscure, although it has been proposed as an alloimmune disease, resulting from immunological reaction to self-antigens (alloantigens) which the body recognizes as foreign. We studied an infant with NH and his mother whose sera contained antimitochondrial antibody (AMA), the hallmark of primary biliary cirrhosis (PBC). Material and Methods. To investigate the origin of AMA in the infant, we studied isotype distributions in serum from the mother and infant. Serum samples were obtained at diagnosis of NH, after liver transplantation (LT; age 1 month), and over the ensuing 17 months. Results. At NH diagnosis, infant and maternal serum contained AMA of the IgG isotype, predominantly of the G3 and G1 subclasses. AMA strongly reacted against the pyruvate dehydrogenase complex E2 subunit (PDC-E2), the major PBC-specific AMA autoantigen. Anti-PDC-E2 responses in both infant and mother declined over time, being present 2 months after LT (mother and child) and absent 10 months later (mother) and 17 months later (child). Conclusion. The association of maternally transferred IgG1 and IgG3 subclass AMA with the appearance of liver damage in an infant with NH may suggest a causal link between antibody and liver damage.http://dx.doi.org/10.1155/2013/642643 |
| spellingShingle | Daniel S. Smyk Maria G. Mytilinaiou Tassos Grammatikopoulos A. S. Knisely Giorgina Mieli-Vergani Dimitrios P. Bogdanos Diego Vergani Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis Clinical and Developmental Immunology |
| title | Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis |
| title_full | Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis |
| title_fullStr | Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis |
| title_full_unstemmed | Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis |
| title_short | Primary Biliary Cirrhosis-Specific Antimitochondrial Antibodies in Neonatal Haemochromatosis |
| title_sort | primary biliary cirrhosis specific antimitochondrial antibodies in neonatal haemochromatosis |
| url | http://dx.doi.org/10.1155/2013/642643 |
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