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罕见疾病导致的胃肠道动力障碍——肠壁纤维化

Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.

作者信息

Daum R, Nützenadel W, Roth H, Zachariou Z

机构信息

Dept. of Pediatric Surgery, University of Heidelberg, FRG.

出版信息

Prog Pediatr Surg. 1989;24:193-201. doi: 10.1007/978-3-642-74493-8_21.

Abstract

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.

摘要

我们报告了两名因慢性肠梗阻入院但无机械性梗阻的儿童。在4个月大的女新生儿中,切除交感神经母细胞瘤后进行了高剂量放疗。几年内,小肠肌层发生化生,导致吸收不良综合征。尽管切除了受损的肠段,但病情仍在进展,患儿死亡。在第二个病例中,一名10岁儿童因肠道纤维化出现慢性肠梗阻。多次进行剖腹探查,未发现机械性梗阻。最可能的诊断是一种主要累及消化道而无皮肤受累的硬皮病形式。患者死于严重恶病质。

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