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Updated: May 19, 2026
A Murine Model of Fetal Exposure to Maternal Inflammation to Study the Effects of Acute Chorioamnionitis on Newborn Intestinal Development
Published on: June 24, 2020
Early-onset chronic inflammatory disease associated with maternal microchimerism
Tomoaki Ishikawa1, Yoshihiko Sakurai, Tomohiro Takeda
1Department of Pediatrics, Nara City Hospital, Nara 630-8305, Japan.
Abstract:
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.
Insights
Maternal microchimerism (mMc), the presence of maternal cells in a child, may contribute to unexplained fevers and autoimmune symptoms in infants. This case study highlights mMc as a potential factor in infantile autoinflammatory disorders.
Area of Science:
- Immunology
- Pediatrics
- Genetics
Background:
- Maternal microchimerism (mMc) involves maternal cells persisting in offspring.
- The role of mMc in pediatric autoimmune diseases is debated.
- Infantile-onset autoinflammatory disorders often present with complex, undiagnosed symptoms.
Purpose of the Study:
- To investigate the potential role of maternal microchimerism in a child with persistent unexplained fever and autoimmune features.
- To explore the diagnostic challenges in cases of infantile autoinflammatory disorders.
Main Methods:
- Case report of an 11-year-old boy with prolonged fever, rashes, and autoimmune markers.
- Clinical examination, laboratory tests (including autoantibodies, inflammatory markers, IL-6, BAFF), liver biopsy, and HLA typing.
- Detection of female cells in patient's skin and lymph nodes via histology.
Main Results:
- The patient presented with persistent fever, skin rashes, hepatic dysfunction, thrombocytopenia, and positive anti-dsDNA antibodies.
- Elevated inflammatory markers, IL-6, and B-cell activating factor were observed.
- Female cells were detected in the patient's tissues, indicating maternal microchimerism, despite negative HLA typing for noninherited maternal antigens.
Conclusions:
- Maternal microchimerism is suggested as a potential contributor to the pathogenesis of unexplained fever in infants.
- This case underscores the complexity of diagnosing infantile autoinflammatory disorders.
- Further research is needed to elucidate the mechanisms linking mMc to pediatric autoimmune conditions.
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