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巨膀胱-小结肠-肠蠕动减少综合征:更多临床、放射学、手术及组织病理学方面的情况

Megacystis-microcolon-intestinal hypoperistalsis syndrome: additional clinical, radiologic, surgical, and histopathologic aspects.

作者信息

Young L W, Yunis E J, Girdany B R, Sieber W K

出版信息

AJR Am J Roentgenol. 1981 Oct;137(4):749-55. doi: 10.2214/ajr.137.4.749.

DOI:10.2214/ajr.137.4.749
PMID:6974971
Abstract

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.

摘要

匹兹堡儿童医院确诊了4例患有巨膀胱-微结肠-肠道蠕动功能减退综合征(MMIHS)的新生儿。这些病例为该综合征提供了更多见解,并拓宽了其范围。本报告包括一名患有MMIHS的男婴、一名长期存活者、新生儿梗阻性肠扭转的一种明显相关并发症、小肠蠕动功能减退的证据以及如下组织病理学发现:(1)早期活检中神经节细胞数量明显增加,后期活检中神经节细胞数量正常甚至减少,可能是由于肠扩张所致;(2)在三分之二的婴儿尸检中,有许多神经干(其中一例有类似神经瘤的一层),且膀胱有弹性组织变性。

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Imaging findings in megacystis-microcolon-intestinal hypoperistalsis syndrome.巨膀胱-小肠-肠蠕动不良综合征的影像学表现。
Pediatr Radiol. 2013 Apr;43(4):454-9. doi: 10.1007/s00247-012-2479-y. Epub 2012 Aug 29.
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Megacystis microcolon intestinal hypoperistalsis syndrome: systematic review of outcome.巨膀胱小结肠肠蠕动不良综合征:结局的系统评价
Pediatr Surg Int. 2011 Oct;27(10):1041-6. doi: 10.1007/s00383-011-2954-9.
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Megacystis microcolon intestinal hypoperistalsis syndrome.巨膀胱-小结肠-肠蠕动不良综合征
Indian J Pediatr. 1999 Nov-Dec;66(6):945-9. doi: 10.1007/BF02723874.
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Megacystis-microcolon-intestinal hypoperistalsis syndrome in a newborn girl whose brother had prune belly syndrome: common pathogenesis?
Pediatr Radiol. 1983;13(5):294-6. doi: 10.1007/BF00973353.
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Ultrasonographic anatomy and diagnosis of fetal uropathies affecting the upper urinary tract. I. Obstructive uropathies.影响上尿路的胎儿泌尿系统疾病的超声解剖与诊断。I. 梗阻性泌尿系统疾病。
Anat Clin. 1985;7(4):301-32. doi: 10.1007/BF01784646.
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Megacystis-microcolon-intestinal hypoperistalsis syndrome: confirmation of autosomal recessive inheritance.巨膀胱-小结肠-肠蠕动减弱综合征:常染色体隐性遗传的确证
J Med Genet. 1986 Aug;23(4):360-2. doi: 10.1136/jmg.23.4.360.
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