罕见疾病引起的胃肠道运动障碍——肠壁纤维化。

R Daum, W Nützenadel, H Roth, Z Zachariou
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引用次数: 3

摘要

我们报告两名儿童谁是入院慢性肠梗阻没有机械阻塞。在4个月大的女性新生儿中,切除交感母细胞瘤后应用高剂量辐射。在几年内,小肠肌层发生化生,导致吸收不良综合征。虽然受损的肠道部分被切除了,但手术仍在继续,孩子最终死亡。在第二个病例中,慢性肠梗阻在10岁时发展为肠道纤维化的结果。多次开腹手术均未发现机械性梗阻。最可能的诊断是一种硬皮病,主要影响消化道,不累及皮肤。病人死于严重的恶病质。
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Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.

We report on two children who were admitted with chronic ileus without mechanical obstruction. In the 4-month-old female newborn, high-dose radiation was applied after extirpation of a sympathicoblastoma. Within a few years a metaplasia of the muscle coat of the small intestine developed with a resulting malabsorption syndrome. Although the damaged part of the intestine was resected, the process progressed and the child died. In the second case, a chronic ileus developed at the age of 10 years as a result of fibrosis of the intestinal tract. Repeated laparotomies were performed, and no mechanical obstruction could be found. The most probable diagnosis is a form of scleroderma affecting mainly the alimentary tract without any skin involvement. The patient died in a severe cachexia.

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