Kahn E, Markowitz J, Simpser E, Aiges H, Daum F
Department of Laboratories, North Shore University Hospital, Manhasset, NY 11030.
J Pediatr Gastroenterol Nutr. 1989 May;8(4):447-53. doi: 10.1097/00005176-198905000-00005.
Reactive systemic or secondary amyloidosis occurs in 1-29% of adults with Crohn's disease, but only sporadic cases of amyloidosis have been recognized in children with inflammatory bowel disease. We therefore have studied operative specimens (ileal, ileocolonic, and colonic) from 46 children (30 with Crohn's disease and 16 with ulcerative colitis) to determine the frequency of amyloid deposits. Sections of bowel, skin, and lymph nodes (n = 940) were stained by Congo red and examined by light microscopy and by polarized light. Amyloid deposits were found in only one of 46 subjects, an 18-year-old girl who had had Crohn's disease for 6 years. Intestinal amyloid deposits, present 16 months before the clinical diagnosis of amyloidosis, were patchy and seen predominantly in the intestinal mucosa. We conclude that amyloidosis is rare in children requiring surgery for Crohn's disease and ulcerative colitis. Examination of Congo red-stained sections can detect even subclinical amyloidosis. The amyloid deposits in our patient, which were both patchy and consistently mucosal, suggest that multiple endoscopic biopsy samples, not necessarily containing submucosa, are sufficient for diagnosis.
反应性全身性或继发性淀粉样变性在1%至29%的成年克罗恩病患者中出现，但在患有炎症性肠病的儿童中仅发现散发性淀粉样变性病例。因此，我们研究了46名儿童（30名克罗恩病患儿和16名溃疡性结肠炎患儿）的手术标本（回肠、回结肠和结肠），以确定淀粉样沉积物的发生率。对肠道、皮肤和淋巴结的切片（共940片）进行刚果红染色，并通过光学显微镜和偏光显微镜检查。在46名受试者中，仅在一名18岁患克罗恩病6年的女孩身上发现了淀粉样沉积物。肠道淀粉样沉积物在淀粉样变性临床诊断前16个月就已存在，呈斑片状，主要见于肠黏膜。我们得出结论，对于因克罗恩病和溃疡性结肠炎而需要手术的儿童，淀粉样变性很少见。检查刚果红染色切片甚至可以检测到亚临床淀粉样变性。我们患者的淀粉样沉积物呈斑片状且始终位于黏膜层，这表明多个内镜活检样本（不一定包含黏膜下层）就足以进行诊断。
