Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism
Maternal microchimerism (mMc) refers to the presence of a small population of cells originating from the mother. Whether mMc leads to autoimmune responses in children remains controversial. We describe here an 11-year-old boy with persistent fever and elevated levels of C-reactive protein from infan...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2012-01-01
|
| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2012/323681 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832551790214643712 |
|---|---|
| author | Tomoaki Ishikawa Yoshihiko Sakurai Tomohiro Takeda Hiroshi Suzuki |
| author_facet | Tomoaki Ishikawa Yoshihiko Sakurai Tomohiro Takeda Hiroshi Suzuki |
| author_sort | Tomoaki Ishikawa |
| collection | DOAJ |
| description | Maternal microchimerism (mMc) refers to the presence of a small population of cells originating from the mother. Whether mMc leads to autoimmune responses in children remains controversial. We describe here an 11-year-old boy with persistent fever and elevated levels of C-reactive protein from infancy onward. During infancy, the patient presented with high fever, skin rashes, and hepatic dysfunction. Careful examination including a liver biopsy failed to reveal the cause. At 4 years old, petechiae developed associated with thrombocytopenia and positive anti-dsDNA autoantibodies. Steroid pulse therapy was effective, but the effect of low-dose prednisone was insufficient. At age 9, an extensive differential diagnosis was considered especially for infantile onset autoinflammatory disorders but failed to make a definitive diagnosis. On admission, the patient exhibited short stature, hepatosplenomegaly, generalized superficial lymphadenopathy, and rashes. Laboratory findings revealed anemia, elevated levels of inflammation markers, and hypergammaglobulinemia. Serum complement levels were normal. Serum levels of IL-6 and B-cell activating factor were elevated. Viral infections were not identified. Although HLA typing revealed no noninherited maternal antigens in lymphocytes, female cells were demonstrated in the patient’s skin and lymph nodes, suggesting that maternal microchimerism might be involved in the pathogenesis of fever without source in infants. |
| format | Article |
| id | doaj-art-2415d85dbb394fddad9cef4d0e2f0096 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-2415d85dbb394fddad9cef4d0e2f00962025-02-03T06:00:30ZengWileyCase Reports in Pediatrics2090-68032090-68112012-01-01201210.1155/2012/323681323681Early-Onset Chronic Inflammatory Disease Associated with Maternal MicrochimerismTomoaki Ishikawa0Yoshihiko Sakurai1Tomohiro Takeda2Hiroshi Suzuki3Department of Pediatrics, Nara City Hospital, Nara 630-8305, JapanDepartment of Pediatrics, Nara Medical University School of Medicine, Kashihara 634-8521, JapanDepartment of Pediatrics, Nara Medical University School of Medicine, Kashihara 634-8521, JapanDepartment of Pediatrics, Nara City Hospital, Nara 630-8305, JapanMaternal microchimerism (mMc) refers to the presence of a small population of cells originating from the mother. Whether mMc leads to autoimmune responses in children remains controversial. We describe here an 11-year-old boy with persistent fever and elevated levels of C-reactive protein from infancy onward. During infancy, the patient presented with high fever, skin rashes, and hepatic dysfunction. Careful examination including a liver biopsy failed to reveal the cause. At 4 years old, petechiae developed associated with thrombocytopenia and positive anti-dsDNA autoantibodies. Steroid pulse therapy was effective, but the effect of low-dose prednisone was insufficient. At age 9, an extensive differential diagnosis was considered especially for infantile onset autoinflammatory disorders but failed to make a definitive diagnosis. On admission, the patient exhibited short stature, hepatosplenomegaly, generalized superficial lymphadenopathy, and rashes. Laboratory findings revealed anemia, elevated levels of inflammation markers, and hypergammaglobulinemia. Serum complement levels were normal. Serum levels of IL-6 and B-cell activating factor were elevated. Viral infections were not identified. Although HLA typing revealed no noninherited maternal antigens in lymphocytes, female cells were demonstrated in the patient’s skin and lymph nodes, suggesting that maternal microchimerism might be involved in the pathogenesis of fever without source in infants.http://dx.doi.org/10.1155/2012/323681 |
| spellingShingle | Tomoaki Ishikawa Yoshihiko Sakurai Tomohiro Takeda Hiroshi Suzuki Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism Case Reports in Pediatrics |
| title | Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism |
| title_full | Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism |
| title_fullStr | Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism |
| title_full_unstemmed | Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism |
| title_short | Early-Onset Chronic Inflammatory Disease Associated with Maternal Microchimerism |
| title_sort | early onset chronic inflammatory disease associated with maternal microchimerism |
| url | http://dx.doi.org/10.1155/2012/323681 |
| work_keys_str_mv | AT tomoakiishikawa earlyonsetchronicinflammatorydiseaseassociatedwithmaternalmicrochimerism AT yoshihikosakurai earlyonsetchronicinflammatorydiseaseassociatedwithmaternalmicrochimerism AT tomohirotakeda earlyonsetchronicinflammatorydiseaseassociatedwithmaternalmicrochimerism AT hiroshisuzuki earlyonsetchronicinflammatorydiseaseassociatedwithmaternalmicrochimerism |