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Maternal microchimerism in Hirschsprung's disease.

Publication ,  Journal Article
Kiefer, AS; Lang, TR; Hein, MS; McNallan, KT; Moir, CR; Reed, AM
Published in: Am J Perinatol
February 2012

Hirschsprung's 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.

Duke Scholars

Published In

Am J Perinatol

DOI

EISSN

1098-8785

Publication Date

February 2012

Volume

29

Issue

2

Start / End Page

71 / 78

Location

United States

Related Subject Headings

  • Sequence Analysis, DNA
  • Pregnancy
  • Obstetrics & Reproductive Medicine
  • Male
  • Intestine, Large
  • Intestinal Mucosa
  • Intestinal Atresia
  • Infant, Newborn
  • In Situ Hybridization
  • Immunohistochemistry
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Kiefer, A. S., Lang, T. R., Hein, M. S., McNallan, K. T., Moir, C. R., & Reed, A. M. (2012). Maternal microchimerism in Hirschsprung's disease. Am J Perinatol, 29(2), 71–78. https://doi.org/10.1055/s-0031-1295645
Kiefer, Autumn S., Tara R. Lang, Molly S. Hein, Kelly T. McNallan, Christopher R. Moir, and Ann M. Reed. “Maternal microchimerism in Hirschsprung's disease.Am J Perinatol 29, no. 2 (February 2012): 71–78. https://doi.org/10.1055/s-0031-1295645.
Kiefer AS, Lang TR, Hein MS, McNallan KT, Moir CR, Reed AM. Maternal microchimerism in Hirschsprung's disease. Am J Perinatol. 2012 Feb;29(2):71–8.
Kiefer, Autumn S., et al. “Maternal microchimerism in Hirschsprung's disease.Am J Perinatol, vol. 29, no. 2, Feb. 2012, pp. 71–78. Pubmed, doi:10.1055/s-0031-1295645.
Kiefer AS, Lang TR, Hein MS, McNallan KT, Moir CR, Reed AM. Maternal microchimerism in Hirschsprung's disease. Am J Perinatol. 2012 Feb;29(2):71–78.
Journal cover image

Published In

Am J Perinatol

DOI

EISSN

1098-8785

Publication Date

February 2012

Volume

29

Issue

2

Start / End Page

71 / 78

Location

United States

Related Subject Headings

  • Sequence Analysis, DNA
  • Pregnancy
  • Obstetrics & Reproductive Medicine
  • Male
  • Intestine, Large
  • Intestinal Mucosa
  • Intestinal Atresia
  • Infant, Newborn
  • In Situ Hybridization
  • Immunohistochemistry