Abstract
Hirschsprungs disease (HD) presents with severe constipation due to absent ganglion cells in the distal rectum. We sought to determine whether maternal chimeric cells are present in aganglionic bowel. We hypothesize that chimeric cells are part of the unfavorable microenvironment that leads to the destruction of enteric neurons in HD. Intestinal biopsies and resections from seven male patients with HD were compared with four male patients with chronic constipation and six with bowel atresia. Fluorescence in situ hybridization was used to identify chimeric cells based on male/female (XX/XY) differences. The location and immunophenotype of chimeric cells were also studied. Chimeric cells were present more often in the small intestine and rectum, compared with the appendix and colon. Patients with HD had a greater number of chimeric cells per 10 magnification field than patients with chronic constipation or congenital atresia. Chimeric cells were predominantly in the submucosa and outer longitudinal muscle layer in HD. Immunophenotyping identified over 40% of chimeric cells as inflammatory. Chimeric cells are present in greater numbers in aganglionic bowel than in other disorders. Clustering of chimeric cells in areas of absent ganglia lends support to the proposed role of maternal microchimerism in allo-autoimmune responses.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 71-78 |
| Number of pages | 8 |
| Journal | American Journal of Perinatology |
| Volume | 29 |
| Issue number | 2 |
| DOIs | |
| State | Published - 2012 |
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Keywords
- chimerism
- Hirschsprungs disease
- intestinal atresia
- microchimerism
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Obstetrics and Gynecology
Cite this
Maternal microchimerism in hirschsprungs disease. / Kiefer, Autumn S.; Lang, Tara R.; Hein, Molly S.; McNallan, Kelly T.; Moir, Christopher R.; Reed, Ann M.
In: American Journal of Perinatology, Vol. 29, No. 2, 2012, p. 71-78.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Maternal microchimerism in hirschsprungs disease
AU - Kiefer, Autumn S.
AU - Lang, Tara R.
AU - Hein, Molly S.
AU - McNallan, Kelly T.
AU - Moir, Christopher R.
AU - Reed, Ann M.
PY - 2012
Y1 - 2012
N2 - Hirschsprungs disease (HD) presents with severe constipation due to absent ganglion cells in the distal rectum. We sought to determine whether maternal chimeric cells are present in aganglionic bowel. We hypothesize that chimeric cells are part of the unfavorable microenvironment that leads to the destruction of enteric neurons in HD. Intestinal biopsies and resections from seven male patients with HD were compared with four male patients with chronic constipation and six with bowel atresia. Fluorescence in situ hybridization was used to identify chimeric cells based on male/female (XX/XY) differences. The location and immunophenotype of chimeric cells were also studied. Chimeric cells were present more often in the small intestine and rectum, compared with the appendix and colon. Patients with HD had a greater number of chimeric cells per 10 magnification field than patients with chronic constipation or congenital atresia. Chimeric cells were predominantly in the submucosa and outer longitudinal muscle layer in HD. Immunophenotyping identified over 40% of chimeric cells as inflammatory. Chimeric cells are present in greater numbers in aganglionic bowel than in other disorders. Clustering of chimeric cells in areas of absent ganglia lends support to the proposed role of maternal microchimerism in allo-autoimmune responses.
AB - Hirschsprungs disease (HD) presents with severe constipation due to absent ganglion cells in the distal rectum. We sought to determine whether maternal chimeric cells are present in aganglionic bowel. We hypothesize that chimeric cells are part of the unfavorable microenvironment that leads to the destruction of enteric neurons in HD. Intestinal biopsies and resections from seven male patients with HD were compared with four male patients with chronic constipation and six with bowel atresia. Fluorescence in situ hybridization was used to identify chimeric cells based on male/female (XX/XY) differences. The location and immunophenotype of chimeric cells were also studied. Chimeric cells were present more often in the small intestine and rectum, compared with the appendix and colon. Patients with HD had a greater number of chimeric cells per 10 magnification field than patients with chronic constipation or congenital atresia. Chimeric cells were predominantly in the submucosa and outer longitudinal muscle layer in HD. Immunophenotyping identified over 40% of chimeric cells as inflammatory. Chimeric cells are present in greater numbers in aganglionic bowel than in other disorders. Clustering of chimeric cells in areas of absent ganglia lends support to the proposed role of maternal microchimerism in allo-autoimmune responses.
KW - chimerism
KW - Hirschsprungs disease
KW - intestinal atresia
KW - microchimerism
UR - http://www.scopus.com/inward/record.url?scp=84857064883&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84857064883&partnerID=8YFLogxK
U2 - 10.1055/s-0031-1295645
DO - 10.1055/s-0031-1295645
M3 - Article
C2 - 22105432
AN - SCOPUS:84857064883
VL - 29
SP - 71
EP - 78
JO - American Journal of Perinatology
JF - American Journal of Perinatology
SN - 0735-1631
IS - 2
ER -