Castro M, Crinò A, Papadatou B, Purpura M, Giannotti A, Ferretti F, Colistro F, Mottola L, Digilio M C, Lucidi V
Division of Gastroenterology, Ospedale Bambino Gesù, Rome, Italy.
J Pediatr Gastroenterol Nutr. 1993 Apr;16(3):265-8.
Patients with Down's syndrome (DS) or celiac disease (CD) have altered immune systems. Autoimmune diseases have been described in both conditions; the coexistence of DS and CD has been occasionally reported, but a clear relationship has not been definitely established. In this study we determined IgA antigliadin antibodies (IgA-AGA) in 155 children with DS, and the results were compared with those of the control groups formed by 320 children affected by upper-respiratory tract infections and 115 children with gastrointestinal symptoms but with normal jejunal mucosa. High IgA-AGA levels were found in 26% of DS patients, in 1% of the first control group and in 10% of the second control group. Such differences are statistically significant. Twenty-one DS patients with high IgA-AGA levels and gastrointestinal symptoms underwent jejunal biopsy, and total villous atrophy was found in seven of them (33.33%). HLA-DR and -DQ antigens were also determined in 75 DS patients (20 with high and 55 with normal IgA-AGA levels), and the percentages of the different phenotypes were compared in the two groups and with those of a control group. No statistically significant difference was found, but DR3, DR7, and DQ2 alleles were always present in DS patients with jejunal atrophy. Our study confirms the data reported in the literature about higher levels of IgA-AGA in DS patients and the relatively high incidence of CD in this group of patients.
唐氏综合征（DS）或乳糜泻（CD）患者的免疫系统存在改变。在这两种疾病中均有自身免疫性疾病的描述；DS和CD的共存情况偶尔有报道，但尚未明确建立起明确的关系。在本研究中，我们测定了155例DS患儿的IgA抗麦醇溶蛋白抗体（IgA-AGA），并将结果与由320例上呼吸道感染患儿和115例有胃肠道症状但空肠黏膜正常的患儿组成的对照组进行了比较。在26%的DS患者、1%的第一对照组患者和10%的第二对照组患者中发现了高IgA-AGA水平。这些差异具有统计学意义。对21例IgA-AGA水平高且有胃肠道症状的DS患者进行了空肠活检，其中7例（33.33%）发现有全绒毛萎缩。还对75例DS患者（20例IgA-AGA水平高，55例正常）的HLA-DR和-DQ抗原进行了测定，并比较了两组以及与对照组不同表型的百分比。未发现统计学上的显著差异，但空肠萎缩的DS患者中总是存在DR3、DR7和DQ2等位基因。我们的研究证实了文献中报道的关于DS患者中IgA-AGA水平较高以及该组患者中CD发病率相对较高的数据。
