{"title":"罕见疾病引起的胃肠道运动障碍——肠壁纤维化。","authors":"R Daum, W Nützenadel, H Roth, Z Zachariou","doi":"10.1007/978-3-642-74493-8_21","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":76378,"journal":{"name":"Progress in pediatric surgery","volume":"24 ","pages":"193-201"},"PeriodicalIF":0.0000,"publicationDate":"1989-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"3","resultStr":"{\"title\":\"Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.\",\"authors\":\"R Daum, W Nützenadel, H Roth, Z Zachariou\",\"doi\":\"10.1007/978-3-642-74493-8_21\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":76378,\"journal\":{\"name\":\"Progress in pediatric surgery\",\"volume\":\"24 \",\"pages\":\"193-201\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1989-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"3\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Progress in pediatric surgery\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1007/978-3-642-74493-8_21\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Progress in pediatric surgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/978-3-642-74493-8_21","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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.