Schröder J M, Dieler R, Skopnik H, Steinau G
Institut für Neuropathologie, Medizinischen Fakultät, Klinikum der Rheinisch-Westfälischen Technischen Hochschule Aachen, Deutschland.
Acta Histochem Suppl. 1992;42:341-4.
Pyloric biopsies obtained at pyloromyotomy from 46 infants were studied by light and electron microscopy and compared to 8 autopsy control cases without any evidence of infantile hypertrophic pyloric stenosis (IHPS). A positive family history of this disorder was recorded in 8 cases (2 girls and 6 boys). The most frequent changes in the myenteric plexus comprised axonal alterations. In glial cells, cytoplasmic vacuolisation or an increase of intermediate filaments occurred. In ganglion cells, vacuolisation of perikaryal cytoplasm or dense bodies were observed. No obvious differences were seen between sporadic and hereditary cases. In addition to these fine structural alterations, immunohistochemistry in 6 IHPS cases and 4 controls revealed differences in the distribution of substance P, bombesin, calcitonin gene related peptide and enkephalin-like immunoreactivity within the myenteric plexus between IHPS and control cases. The immunoreactivity, however, was unevenly distributed from case to case and even within individual cases. The reduction of immunoreactivity corresponded, at least in part, to an increase of neurofilaments or abnormal organelles within axons. Increased immunoreactivity was apparently related to focal accumulation of dense cored vesicles noted in the preceding study. It is suggested that these and other changes reported interfere with the normal gastrointestinal reflex mechanisms leading to intestinal obstruction.
对46例婴儿在幽门肌切开术中获取的幽门活检组织进行了光镜和电镜研究,并与8例无婴儿肥厚性幽门狭窄(IHPS)证据的尸检对照病例进行了比较。8例(2例女孩和6例男孩)记录有该疾病的阳性家族史。肌间神经丛最常见的变化包括轴突改变。在神经胶质细胞中,出现了细胞质空泡化或中间丝增加。在神经节细胞中,观察到核周细胞质空泡化或致密小体。散发性和遗传性病例之间未见明显差异。除了这些细微的结构改变外,对6例IHPS病例和4例对照进行的免疫组织化学研究显示,IHPS病例和对照病例的肌间神经丛中P物质、蛙皮素、降钙素基因相关肽和脑啡肽样免疫反应性的分布存在差异。然而,免疫反应性在不同病例之间甚至在单个病例内分布不均。免疫反应性的降低至少部分与轴突内神经丝或异常细胞器的增加相对应。免疫反应性增加显然与先前研究中发现的致密核心小泡的局灶性聚集有关。提示所报道的这些及其他变化干扰了正常的胃肠反射机制,导致肠梗阻。