Coerdt Wiltrud, Michel Jörg-S, Rippin Gerd, Kletzki Semen, Gerein Valentin, Müntefering Horst, Arnemann Joachim
Department of Paediatric Pathology, Johannes Gutenberg University Hospital, 55101, Mainz, Germany.
Virchows Arch. 2004 Mar;444(3):239-46. doi: 10.1007/s00428-003-0951-7. Epub 2004 Jan 29.
An increased number and density of the so-called "giant ganglia" (seven or greater ganglion cells per ganglion) serve as histopathological criteria for a bowel motility disorder called intestinal neuronal dysplasia of the submucous plexus (IND B). However, because these morphological criteria have been defined based upon observations in constipated patients, the diagnostic value of previous studies is open to controversy. Moreover, no age-related reference data from unaffected controls are available. This study reports on data from unaffected controls on the variability of size and distribution of ganglia in the submucous plexus during development. Therefore, for the first time, the normal status has been defined. Four age groups have been defined: (a) premature births, gestational age less than 35 weeks; (b) 1-365 days; (c) 1-14 years and (d) 15 years to greater than 70 years). All of these groups revealed giant ganglia in the submucous plexus. With advancing age, there was a decrease in the number of giant ganglia (from 32.7% in group a to 11.2% in group d) accompanied by an inverse increase in the mean distance between all ganglia (from 0.52 mm in group a to 1.17 mm in group d). The data presented permit the conclusion that the criteria mentioned above are not apt to define IND B as an entity, since they do not allow a sufficient demarcation from the age-correlated normal values presented here.
所谓“巨神经节”(每个神经节有七个或更多神经节细胞)数量和密度的增加,是一种称为黏膜下丛肠道神经元发育异常(IND B)的肠道动力障碍的组织病理学标准。然而,由于这些形态学标准是基于对便秘患者的观察而定义的,以往研究的诊断价值存在争议。此外,目前尚无来自未受影响对照组的与年龄相关的参考数据。本研究报告了未受影响对照组在发育过程中黏膜下丛神经节大小和分布变异性的数据。因此,首次定义了正常状态。定义了四个年龄组:(a)早产,孕周小于35周;(b)1至365天;(c)1至14岁;(d)15岁至70岁以上。所有这些组在黏膜下丛中均发现了巨神经节。随着年龄的增长,巨神经节的数量减少(从a组的32.7%降至d组的11.2%),同时所有神经节之间的平均距离呈相反增加(从a组的0.52毫米增至d组的1.17毫米)。所呈现的数据得出结论,上述标准不适用于将IND B定义为一种独立的疾病,因为它们无法与这里呈现的与年龄相关的正常值进行充分区分。