Wester T, O'Briain S, Puri P
Children's Research Center, Our Lady's Hospital for Sick Children, Dublin, Ireland.
J Pediatr Surg. 1998 Apr;33(4):619-22. doi: 10.1016/s0022-3468(98)90328-1.
BACKGROUND/PURPOSE: The diagnosis of intestinal neuronal dysplasia (IND) has traditionally been based on the finding of hyperplasia of the submucous plexus and increased acetylcholinesterase activity in parasympathetic nerve fibers in the lamina propria. However, recently it has been suggested that proposed diagnostic criteria relating to nerve cell density may overlap with age-related changes and that the finding of giant ganglia (ganglia containing more than seven ganglion cells) is the most relevant diagnostic parameter of IND. Ganglion cell counting is usually performed on conventional histological sections, whereas the topology of whole ganglia has been poorly studied. The aim of this study was to define the number of ganglion cells per ganglion and the ganglion cell density (the number of ganglion cells per surface area) in submucous whole-mount preparations of normal human colon.
Specimens from distal colon were obtained during postmortem examination from 14 patients who died of nongastrointestinal disease. The submucous layer was prepared as a whole mount and stained for NADPH diaphorase (a nitrergic neurotransmitter marker) and cuprolinic blue (a general neuronal marker). Ganglion cell density was estimated by counting at least 10 mm2. The number of ganglion cells per ganglion was counted in at least 20 ganglia per case.
Ganglion cell density (NADPH diaphorase) fell markedly during the first 5 to 6 years of life (r = -0.60, P < .05). Most ganglion cells formed ganglia of 3 to 64 cuprolinic blue staining cells. The mean number of ganglion cells per ganglion did not vary with age.
The density of NADPH diaphorase-positive ganglion cells in the submucous plexus of human distal colon decreases markedly with age. However, the number of ganglion cells per ganglion remains constant. These findings indicate that the age of the patient has crucial importance for the histolopathologic evaluation of enteric nervous system disorders.
背景/目的:传统上,肠道神经元发育异常(IND)的诊断是基于黏膜下神经丛增生以及固有层副交感神经纤维中乙酰胆碱酯酶活性增加这一发现。然而,最近有人提出,与神经细胞密度相关的拟诊断标准可能与年龄相关变化重叠,并且巨大神经节(包含超过7个神经节细胞的神经节)的发现是IND最相关的诊断参数。神经节细胞计数通常在传统组织学切片上进行,而整个神经节的拓扑结构研究较少。本研究的目的是确定正常人结肠黏膜下整装标本中每个神经节的神经节细胞数量以及神经节细胞密度(每单位表面积的神经节细胞数量)。
从14例死于非胃肠道疾病的患者尸检中获取远端结肠标本。将黏膜下层制成整装标本,并用NADPH黄递酶(一种一氧化氮能神经递质标志物)和铜叶绿酸蓝(一种通用神经元标志物)染色。通过计数至少10平方毫米来估计神经节细胞密度。每例至少在20个神经节中计数每个神经节的神经节细胞数量。
在生命的最初5至6年中,神经节细胞密度(NADPH黄递酶)显著下降(r = -0.60,P <.05)。大多数神经节细胞形成了由3至64个铜叶绿酸蓝染色细胞组成的神经节。每个神经节的神经节细胞平均数量不随年龄变化。
人类远端结肠黏膜下神经丛中NADPH黄递酶阳性神经节细胞的密度随年龄显著降低。然而,每个神经节的神经节细胞数量保持恒定。这些发现表明,患者年龄对于肠道神经系统疾病的组织病理学评估至关重要。