Megacystis-microcolon-intestinal hypoperistalsis syndrome: additional clinical, radiologic, surgical, and histopathologic aspects.
Megacystis-microcolon-intestinal hypoperistalsis syndrome: additional clinical, radiologic, surgical, and histopathologic aspects.
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巨囊菌-小结肠-肠蠕动减退综合征：其他临床、放射学、手术和组织病理学方面。
DOI：
10.2214/ajr.137.4.749
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发表时间：
1981
期刊：
影响因子：
--
通讯作者：
W. Sieber
中科院分区：
文献类型：
--
作者：
L. W. Young;E. Yunis;B. Girdany;W. Sieber
匹兹堡儿童医院确诊了4名患有巨囊症-微结肠-肠低动力综合征(MMIHS)的新生儿。这些病例提供了对该综合征的更多洞察，并拓宽了其范围。本报告包括一名男婴的MMIHS，一名长期存活的婴儿，明显相关的新生儿梗阻性扭转并发症，小肠动力不足的证据，以及下列组织病理发现：(1)早期活检中神经节细胞明显增多，而后期活检中神经节细胞数量明显增加，甚至减少，可能是由于肠扩张；(2)三名婴儿中的两名，尸检时可见多条神经干(一条神经样层在一条神经干上)，并有膀胱弹力性扩张。
Four newborn infants with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) were identified at Children's Hospital of Pittsburgh. These cases provide additional insight into the syndrome and broaden its spectrum. This report includes MMIHS in an infant boy, one long-term survivor, an apparently related complication of neonatal obstructive volvulus, evidence of small intestinal hypoperistalsis, and histopathologic findings as follows: (1) apparently increased numbers of ganglion cells in early biopsies and normal or even decreased numbers of ganglion cells in later biopsies probably due to bowel dilatation; and (2) in two of three infants at autopsy, there were many nerve trunks (a neuromalike layer in one), and there was elastosis of the urinary bladder.