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Down syndrome and coeliac disease: five new cases with a review of the literature

  • Gastroenterology/Hepatology
  • Published:
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Abstract

We report five new patients with coeliac disease and Down syndrome and review the 11 cases previously reported in the literature. In 14 of these 16 patients diarrhoea was the presenting symptom and in 2 failure to thrive in combination with anaemia. The frequency of coeliac disease in children with Down syndrome was calculated as being 43 times greater than in children without Down syndrome. Delay between first symptoms and diagnosis in patients with combined coeliac disease and Down syndrome was 2.5 years, while in the other children with coeliac disease it was only 8 months. This distinctive difference could be caused by an underestimation of the seriousness of gastro-intestinal complaints in patients with Down syndrome. It is stressed that coeliac disease should be strongly considered in all children with Down syndrome and either persistent diarrhoea or failure to thrive.

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Abbreviations

AGA:

anti-gliadin antibodies

AU:

arbitrary units

CD:

coeliac disease

DS:

Down syndrome

DSPC:

Down syndrome percentile chart

References

  1. Amil Dias J, Walker-Smith J (1990) Down's syndrome and coeliac disease. J Pediatr Gastroenterol Nutr 10:41–43

    PubMed  Google Scholar 

  2. Auricchio S, Greco L, Troncone R (1988) Gluten-sensitive enteropathy in childhood. Pediatr Clin North Am 35:157–187

    PubMed  Google Scholar 

  3. Bentley D (1975) A case of Down's syndrome complicated by retinoblastoma and celiac disease. Pediatrics 56:131–133

    PubMed  Google Scholar 

  4. Bertotto A, Scalise F, Gerli R, Castellucci G, Fabietti GM, Spinozzi F, Sensi L, Vaccaro R (1992) Lymphocytes bearing the γδ T-cell receptors in Down's syndrome. Scand J Immunol 35:275–278

    PubMed  Google Scholar 

  5. Blazer S, Naveh Y, Berant M, Merzbach D, Sperber S (1984) Serum IgG antibodies to gliadin in children with celiac disease as measured by an immunofluorescence method. J Pediatr Gastroenterol Nutr 3:205–209

    PubMed  Google Scholar 

  6. Bürgin-Wolff A, Bertele RM, Berger R, Gaze H, Harms HK, Just M, Khanna S, Schürmann K, Signer E, Tomovic D (1983) A reliable screening test for childhood celiac disease: Fluorescent immunosorbent test for gliadin antibodies. Pediatrics 102: 655–660

    Google Scholar 

  7. Burgio GR, Ugazio AG (1978) Immunity in Down's syndrome. Eur J Pediatr 127:293–294

    PubMed  Google Scholar 

  8. Burgio GR, Ugazio AG, Nespoli L, Marcioni AF, Botelli AM, Pasquali F (1975) Derangements of immunoglobulin levels, phytohemagglutinin responsiveness and T and B cell markers in Down's syndrome at different ages. Eur J Immunol 5: 600–603

    PubMed  Google Scholar 

  9. Castro M, Crino A, Papadatou B, Purpura M, Giannotti A, Ferretti F, Mottola L, Digilio C, Lucidi V (1993) Down's syndrome and Coeliac disease: the prevalence of high IgA-antigliadin antibodies and HLA-DR and DQ antigens in trisomy 21. J Pediatr Gastroenterol Nutr 16:265–268

    PubMed  Google Scholar 

  10. Cornel MC, Breed ASPM, Beekhuis JR, Meerman te GJ, Kate ten LP (1993) Down Syndrome: effects of demographic factors and prenatal diagnosis on the future live birth prevalence. Hum Genet (in press)

  11. Cronk C, Crocker AC, Pueschel SM, Shea AM, Zackai E, Pickens C, Reed RB (1988) Growth charts for children with Down syndrome: 1 month to 18 years of age. Pediatrics 81: 102–110

    PubMed  Google Scholar 

  12. Grossi CE, Ciccone E, Migone N, Bottino C, Zarcone D, Mingari MC, Ferrini S, Tambussi G, Viale O, Casorati G, Millo R, Moretta L, Moretta A (1989) Human T cells expressing the γδ T-cell receptor (TcR-1): Cγ1- and Cγ2-encoded forms of the receptor correlate with distinctive morphology, cytoskeletal organization and growth characteristics. Proc Natl Acad Sci USA 86:1619–1623

    PubMed  Google Scholar 

  13. Howell SJL, Foster KJ, Reckless J (1983) Protracted survival in patients with Down's syndrome. BMJ 287:1429–1430

    Google Scholar 

  14. Nowak TV, Ghishan FK, Schulze-Delrieu K (1983) Celiac sprue in Down's syndrome: considerations on a pathogenetic link. Am J Gastroenterol 78:280–283

    PubMed  Google Scholar 

  15. Penrose LS, Smith GF (1966) Down's anomaly. JA Churchill LTD, London, p30

    Google Scholar 

  16. Ruch W, Schürmann K, Gordon P, Bürgin-Wolff A, Girard J (1985) Coexistent coeliac disease, Grave's disease and diabetes mellitus type I in a patient with Down syndrome. Eur J Pediatr 144:89–90

    PubMed  Google Scholar 

  17. Santer R, Sievers E, Oldigs HD (1991) Celiac disease in Down's syndrome. J Pediatr Gastroenterol Nutr 13:121

    PubMed  Google Scholar 

  18. Similä S, Kokkonen J (1990) Coexistence of eliac disease and Down syndrome. Am J Ment Ret 95:120–122

    Google Scholar 

  19. Storm W (1990) Prevalence and diagnostic significance of gliadin antibodies in children with Down syndrome. Eur J Pediatr 149:833–834

    PubMed  Google Scholar 

  20. Viney J, MacDonald TT, Spencer J (1990) Gamma/delta T cells in the gut epithelium. Gut 31:841–844

    PubMed  Google Scholar 

  21. Wauters EAK, Jansen J, Houwen RHJ, Veenstra J, Ockhuizen Th (1991) Serum IgG asnd IgA anti-gliadin antibodies as markers of mucosal damage in children with suspected celiac disease upon gluten challenge. J Pediatr Gastroenterol Nutr 13: 192–196

    PubMed  Google Scholar 

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Hilhorst, M.I., Brink, M., Wauters, E.A.K. et al. Down syndrome and coeliac disease: five new cases with a review of the literature. Eur J Pediatr 152, 884–887 (1993). https://doi.org/10.1007/BF01957522

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  • DOI: https://doi.org/10.1007/BF01957522

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