Fadda B, Maier W A, Meier-Ruge W, Schärli A, Daum R
Z Kinderchir. 1983 Oct;38(5):305-11. doi: 10.1055/s-2008-1059994.
61 cases of neuronal intestinal dysplasia are compared in a follow-up study. Two clinically and bioptically different types of intestinal dysplasia can be distinguished. One type with involvement of the sympathetic nerves and the clinical signs of intestinal spasticity, ulcerative colitis with haemorrhagic stools. Histotopochemically, this disease pattern shows aplasia or hypoplasia of the sympathetic nerves with enhanced parasympathetic activity (elevated acetylcholinesterase activity in the lamina propria mucosae and orbicular musculature). One type with involvement of the plexus submucosus clinically accompanied by adynamia of the colon with megacolon formation. This type becomes manifest usually around the 6th month of life. Bioptically there are large groups of ganglion cells and Schwann's cells, but there is also acetylcholinesterase activity as in the other type. This 2nd form is seen more frequently. A third form is a combination of both diseases. The incidence rate of neuronal intestinal dysplasia is equal to that of Hirschsprung's disease.
在一项随访研究中，对61例神经元性肠发育异常病例进行了比较。临床上和活检可区分出两种不同类型的肠发育异常。一种类型累及交感神经，伴有肠道痉挛、血性粪便的溃疡性结肠炎等临床症状。组织化学上，这种疾病模式表现为交感神经发育不全或发育不良，副交感神经活动增强（黏膜固有层和环形肌层乙酰胆碱酯酶活性升高）。另一种类型累及黏膜下神经丛，临床上伴有结肠动力不足和巨结肠形成。这种类型通常在出生后第6个月左右出现。活检可见大量神经节细胞和施万细胞，但也有与另一种类型一样的乙酰胆碱酯酶活性。第二种形式更为常见。第三种形式是两种疾病的组合。神经元性肠发育异常的发病率与先天性巨结肠相等。
