Summary
A case of autoimmune chronic active hepatitis (C.A.H.) in a 12-year old Down Syndrome child is described. This represents the second only documented case of autoimmune C.A.H. occurring in Down Syndrome; the first well-described case1 died from the disease. The patient described below had a satisfactory response to steroid therapy with restoration of normal liver histological appearances and function despite severe disease at presentation. Chronic hepatitis in Down Syndrome, although more usually of hepatitis B viral aetiology, may also be autoimune in nature and resolve satisfactorily with appropriate therapy.
References
McCulloch, A. J., Ince., P. G., Kendall-Taylor, P. Autoimmune chronic active hepatitis in Down Syndrome. J. Med. Genet. 1982: 19 (3): 232–234.
Szmuness, W., Prince, A. M. The epidemiology of serum hepatitis (SH) infections — a controlled study in two closed institutions. Am. J. Epidemiol. 1971: 94: 585–595.
Chaudhary, R. K., Perry, E., Cleary, T. E. Prevalence of hepatitis B infection among residents of an institution for the mentally handicapped. A. J. Epidemiol. 1977: 105: 123–126.
Whittingham, S., Sharma, D. L. B., Pitt, D. B., Mackay, I. R. Stress deficiency of the T-lymphocyte system exemplified by Down Syndrome. Lancet 1977: i, 163–166.
Burgio, G. R., Ugazio, A. G., Nespoli, L. et al. Derangements of immunoglobulin levels, phytohemagglutinin responsiveness and T and B cell markers in Down Syndrome at different ages. Eur. J. Immun. 1975: 5, Pt. 9: 600–603.
Ugazio, A. G., Maccario, R., Duse, M., Burgio, G. R. T-lymphocyte deficiency in Down Syndrome. Lancet 1977: i: 1062.
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O’Mahony, D., Whelton, M.J. & Hogan, J. Down syndrome and autoimmune chronic active hepatitis: Satisfactory outcome with therapy. I.J.M.S. 159, 21–22 (1990). https://doi.org/10.1007/BF02937210
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DOI: https://doi.org/10.1007/BF02937210