Manzione N C, Kram M, Kram E, Das K M
Department of Medicine, Albert Einstein College of Medicine, Bronx, New York.
Am J Gastroenterol. 1988 Nov;83(11):1294-7.
A patient with Turner's syndrome who developed ulcerative colitis (UC) is reported, and reports from the literature of 16 cases of Turner's syndrome with inflammatory bowel disease (IBD) are reviewed. Most of the patients previously described had severe disease. Half of the patients had ulcerative colitis and half had Crohn's disease (CD). Of those with Crohn's, most had colonic involvement. Of note, also, half of the patients had an isochromosome for the long arm of X which usually is found in only 17% of all patients with Turner's syndrome. A high incidence of autoimmune diseases is associated with this syndrome. We demonstrate the presence of specific anticolonic antibodies in this patient and propose some possible explanations for the association of this particular chromosomal disorder with autoimmune diseases.
本文报告了1例患有溃疡性结肠炎(UC)的特纳综合征患者,并回顾了文献中16例特纳综合征合并炎症性肠病(IBD)的病例报告。之前描述的大多数患者病情严重。一半患者患有溃疡性结肠炎,另一半患有克罗恩病(CD)。在患有克罗恩病的患者中,大多数累及结肠。同样值得注意的是,一半患者有X染色体长臂等臂染色体,而这通常仅见于17%的特纳综合征患者。自身免疫性疾病的高发病率与该综合征相关。我们在该患者中证实了特异性抗结肠抗体的存在,并对这种特殊染色体疾病与自身免疫性疾病的关联提出了一些可能的解释。