Sy Edgar D, Shan Yan-Shen, Lin Chyi-Her, Lin Pin-Wen
Division of Pediatric Surgery, Department of Surgery, National Cheng Kung University Hospital, Tainan, Taiwan.
J Formos Med Assoc. 2004 Jul;103(7):558-61.
Various etiologies have been suggested to be responsible for the development of idiopathic hypertrophic pyloric stenosis (IHPS) in the newborn. The purpose of this study was to determine the maturity of intrinsic nerves and nitrergic neurons in the pyloric muscle in IHPS.
Full thickness pyloric muscle specimens were obtained from 6 infants with IHPS with age ranging from 27 to 95 (mean 58) days old and subjected to immunohistochemical and double chemiluminescence staining for protein gene product 9.5 (PGP9.5) and neuronal nitric oxide synthase (nNOS).
The results showed absence of myenteric plexus between the circular and longitudinal muscle layers in 2 patients, decrease in 1 patient, normal myenteric plexus in 1 patient, and absence of nNOS-containing neurons in 4 patients. All 6 patients had expression of markers for supporting nerve cells and myogenesis in the pyloric smooth muscle.
These results suggest that absence or immaturity of intrinsic nerve and nNOS-containing neurons in the pyloric muscle is the cause of IHPS. The demonstrated lack of innervation or delayed innervation may be the responsible mechanism for the development of IHPS, but further study is needed to elucidate the pathogenesis.
已提出多种病因与新生儿特发性肥厚性幽门狭窄（IHPS）的发生有关。本研究的目的是确定IHPS患儿幽门肌层中固有神经和含一氧化氮合酶（nNOS）神经元的成熟度。
从6例年龄在27至95（平均58）天的IHPS患儿获取全层幽门肌标本，进行免疫组织化学和双化学发光染色，检测蛋白基因产物9.5（PGP9.5）和神经元型一氧化氮合酶（nNOS）。
结果显示，2例患者环形肌层和纵行肌层之间无肌间神经丛，1例患者减少，1例患者肌间神经丛正常，4例患者无含nNOS的神经元。所有6例患者幽门平滑肌中均有支持神经细胞和成肌标志物的表达。
这些结果提示，幽门肌层中固有神经和含nNOS神经元的缺失或不成熟是IHPS的病因。所证实的神经支配缺失或延迟可能是IHPS发生的相关机制，但需要进一步研究以阐明其发病机制。
