Watanabe Yoshio, Ando Hisami, Seo Takahiko, Katsuno Shinsuke, Marui Yuji, Ono Yasuyuki, Torihashi Shigeko
Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Showa-ku, Nagoya, Japan.
Pediatr Int. 2002 Apr;44(2):145-8. doi: 10.1046/j.1442-200x.2002.01518.x.
Esophageal achalasia (EA) is a rare disease in children, the etiology and pathogenesis of which remain controversial. Previous studies have suggested that a specific class of interstitial cells of Cajal (ICC) act as mediators in nitrergic inhibitory neurotransmission in the lower esophageal sphincter (LES). The aim of this investigation is to clarify the status of ICC and nitrergic inhibitory neurons in the LES of EA using immunohistochemistry.
Specimens were obtained from two patients with EA (aged 6 and 10 years) and two patients with esophageal carcinoma (aged 56 and 63 years) not involving the lower esophagus as controls. Immunohistochemistry was used to study the distribution of ICC and nitrergic inhibitory neuron.
The LES contains the c-kit positive ICC in the muscle layers, which form close relationships with nitric oxide synthase (NOS)-containing nerve fibers in the controls. The distribution of ICC was almost the same between samples with EA and controls. However, such nerve fibers were absent in EA with a longer duration of the symptoms, but were reduced in a shorter duration.
Decreased nitrergic inhibitory neurotransmission to ICC in LES is a possible cause of sphincter achalasia in pediatric patients with EA. The decrease in NOS-positive neurons of patients with achalasia may be gradual, which may account for the long duration of symptoms prior to treatments. Further advancement of esophageal motility damage was suspected in pediatric EA.
食管失弛缓症（EA）在儿童中是一种罕见疾病，其病因和发病机制仍存在争议。既往研究表明，特定类型的 Cajal 间质细胞（ICC）在食管下括约肌（LES）的氮能抑制性神经传递中起介导作用。本研究旨在通过免疫组织化学明确 EA 患者 LES 中 ICC 和氮能抑制性神经元的状态。
从两名 EA 患者（年龄分别为 6 岁和 10 岁）以及两名未累及食管下段的食管癌患者（年龄分别为 56 岁和 63 岁）获取标本作为对照。采用免疫组织化学研究 ICC 和氮能抑制性神经元的分布。
LES 的肌层中含有 c-kit 阳性的 ICC，在对照组中其与含一氧化氮合酶（NOS）的神经纤维形成紧密联系。EA 样本与对照组之间 ICC 的分布几乎相同。然而，症状持续时间较长的 EA 患者中此类神经纤维缺失，而症状持续时间较短的患者中此类神经纤维减少。
LES 中向 ICC 的氮能抑制性神经传递减少可能是小儿 EA 患者括约肌失弛缓的一个原因。失弛缓症患者中 NOS 阳性神经元的减少可能是渐进性的，这可能解释了治疗前症状持续时间较长的原因。小儿 EA 患者疑似存在食管动力损害的进一步进展。
