Pediatric Surgery International

, Volume 9, Issue 4, pp 242–247 | Cite as

Abnormal expression of blood group-associated antigen (BGA) in colon of Hirschsprung's disease

  • Takao Fujimoto
  • Takeshi Miyano
Main Topic


The entire segment of resected colon obtained from 15 patients with Hirschsprung's disease (HD) was examined with conventional mucin histochemistry (PASAB, HID-AB) to investigate the mucin composition and blood group-associated antigens (BGA) (sialosyl-Lea, Lea, Ley, Leb) for detecting mucosal cell differentiation. The BGA Leb were absent in normal distal colon and rectum but present in fetal colon. Immunoreactivity of Leb, which is a marker of undifferentiated crypt cells, was observed through out the crypts in aganglionic colon. Depletion of sulphated and neutral mucin was also a characteristic finding in the aganglionic mucosa. These findings suggest that the colonic mucosa of aganglionic bowel represents persistence of a fetal stage of development. These cellular abnormalities may be a factor in the pathogenesis of enterocolitis complicating HD.

Key words

Hirschsprung's disease Enterocolitis Blood group-associated antigens Mucin 


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  1. 1.
    Berry CL (1969) Persistent changes in the large bowel following the enterocolitis associated with Hirschsprung's disease. J Pathol 97: 731–732Google Scholar
  2. 2.
    Bill AH, Champman ND (1962) The enterocilitis of Hirschsprung's disease; history and treatment. Am J Surg 103: 70–74Google Scholar
  3. 3.
    Bishop AE, Polak JM, Lake BD, et al (1981) Abnormality of the colon regulatory peptides in Hirschsprung's disease. Histopathology 5: 679–688Google Scholar
  4. 4.
    Brown A, Ellis IO, Embleton MJ, Baldwin RW, Turner DR, Hardcastle JD (1984) Immunohistochemical localization of Y hapten and the structurally related H type-2 blood group antigen on large bowel tumours and normal adult tissues. Int J Cancer 33: 727–736Google Scholar
  5. 5.
    Coopers HS, Haesler WE (1978) Blood group substance as tumor antigen in distal colon. Am J Clin Pathol 69: 594–598Google Scholar
  6. 6.
    Cordon-Card C, Lloyd DO, Sakamoto J, MacGroarty ME, Old LJ, Melamed MP (1976) Immunohistochemical expression of blood group antigens in normal human gastrointestinal tract and colonic carcinoma. Int J Cancer 37: 667–676Google Scholar
  7. 7.
    Fujimoto T (1988) Natural history and pathophysiology of enterocilitis in Piebald lethal mouse model of Hirschsprung's disease. J Pediatr Surg 23: 237–242Google Scholar
  8. 8.
    Fujimoto T, Puri P (1988) Persistence of enterocolitis following diversion of faecal steam in Hiirschsprung's disease: a study of mucosal defence mechanism. Pediatr Surg Int 3: 141–146Google Scholar
  9. 9.
    Fujimoto T, Reen DJ, Puri P (1988) Inflammatory response in enterocolitis in the Piebald lethal mouse model of Hirschsprung's disease. Pediatr Res 24: 152–155Google Scholar
  10. 10.
    Hansson GC, Karlsson KA, Carson G, McKibbin JM, Blaszcyk M, Herlyn M, Steplewski Z, Koprowski H (1983) Mouse monoclonal antibodies against human cancer cell line with specificities for blood group related antigens; characterization by antibody binding to glycosphingolipids in a chromatogram binding assay. J Biol Chem 258: 4091–4097Google Scholar
  11. 11.
    Kluck P, van Muijen GNP, van der Kamp AWM (1984) Hirschsprung's disease studied with monoclonal antineurofilament antibodies on tissue sections. Lancet I: 652–654Google Scholar
  12. 12.
    Lillie JG, Crispin AR (1971) Hirschsprung's disease in newborn. Ann Radiol 14: 265–269Google Scholar
  13. 13.
    Lloyd-Still JD, Dermers LM (1978) Hirschsprung's enterocolitis: prostaglandins and response to cholestyramine. J Pediar Surg 13: 417–418Google Scholar
  14. 14.
    Nixon HH (1982) Hirschsprung's disease in newborn. In: Holschneider Am (ed) Hirschsprung's disease. Hippokrates, Stuttgart, pp 103–113Google Scholar
  15. 15.
    O'Doriso MS, Wood CL, O'doriso TM (1985) Vasoactive intestinal peptide and neuropeptide modulation of the immune response. J Immunol 135: 792–796Google Scholar
  16. 16.
    Rhodes JD, Black RR, Gallemore R, Savage A (1985) Histochemical demonstration of desialation and desulphation of normal and inflammatory bowel disease rectal mucus by faecal extracts. Gut 26: 1312–1318Google Scholar
  17. 17.
    Ruggiero F, Cooper HS, Steplewski Z (1988) Immunohistochemical study of colorectal adenomas with monoclonal antibodies against blood group antigen (Sialosyl-Lea, Lea, Leb, Lex, Leu, A, B, and H). Lab Invest 59: 96–108Google Scholar
  18. 18.
    Sakamoto J, Furukawa K, Cordon-cardo C, Yin BWT, Rettig WJ, Oettgen HF, Old LJ, Lloyd KO (1986) Expression of Lewisa, Lewisb, X and Y blood group antigens in human colonic tumors and normal tissue and human tumor derived cell lines. Cancer Res 46: 1553–1561Google Scholar
  19. 19.
    Soeda J, O'Briain S, Puri P (1993) Reduced intestinal neuroendocrine cell populations in enterocolitis complicating Hirschsprung's disease. J Pediatr Surg (In press)Google Scholar
  20. 20.
    Szulman AE, Marcus DM (1973) The histological distribution of blood group substance in man as disclosed by immunoflourescence VI. The Lea and Leb antigens during fetal development. Lab Invest 28: 565–574Google Scholar
  21. 21.
    Taguchi T, Tanaka K, Ikeda K (1985) Immunohistochemical study of neuron specific enolase and S-100 protein in Hirschsprungs's disease. Virchows Arch [A] 405: 399–409Google Scholar
  22. 22.
    Thomas DFM, Fenie DS, Bayston R, Sptiz L, Nixon HH (1986) Enterocolitis Hirschsprung's disease: a controlled study of the etiologic role ofClostridium difficile. J Pediatr Surg 21: 22–25Google Scholar
  23. 23.
    Tuto T, Okamura H, Fukui K (1985) An immunohistochemical investigation of gut hormones in the colon of patients with Hirschsprung's disease. J Pediatr Surg 20: 266–270Google Scholar

Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • Takao Fujimoto
    • 1
  • Takeshi Miyano
    • 1
  1. 1.The Department of Paediatric Surgery Juntendo UniversitySchool of MedicineBankyo, Tokyo 113Japan

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