先天性肥厚性幽门狭窄一氧化氮合成酶及肠间质细胞的免疫组化研究

An Immunohistochemical Study for NOS and ICs in Hypertrophic Pyloric Stenosis

目的:了解先天性肥厚性幽门狭窄(CHPS)与一氧化氮(NO)及肠间质细胞(ICs)的关系,以进一步研究 CHPS 的发病机理。方法:通过还原型辅酶Ⅱ二磷酸酶组织化学及酪氨酸激酶受体(c-kit)免疫组化方法观察了一氧化氮合成酶(NOS)及 ICs 在27例 CHPS 及12例正常对照组幽门肌中的分布情况。结果:对照组幽门环肌层内含有丰富的 NOS 染色阳性神经纤维,肌间神经丛及粘膜下神经丛神经元呈 NOS 染色阳性,纵肌层 NOS 染色阳性神经纤维明显少于环肌层;多量ICs 分布于肌间神经节周围和环肌层内,并联结成网状结构。在 CHPS 幽门环肌层中缺乏 NOS 染色阳性神经纤维,纵肌层内 NOS 染色阳性神经纤维较对照组减少,而肌间神经丛神经元则呈NOS 染色阳性;在 CHPS 肥厚的环肌层内及肌间神经节周围几乎无 ICs,仅见有少量 ICs 残留在粘膜下之浅表环肌层内。结论:NOS 及 ICs 的异常分布可能与 CHPS 的发病机理有关。

Objective:To investigate the relationship between the congenital hypertrophic py- loric stenosis(CHPS)with the nitric oxide(NO),and interstitial cells(ICs)for further explo- ration of the etiology of CHPS.Methods:The distribution of NO synthase(NOS)and ICs in the pyloric muscular specimens from 27 cases of CHPS and 12 cases of normal control were studied by NADPH-diaphorase histochemistry and c-kit immunohistochemistry.Results:In control group, NOS positive nerve fibers were abundant in the circular muscularis and the neurons of the myen- teric plexus and the submucosal plexus,and NOS positive nerve fibers in the longitudinal muscu- laris were much less.More ICs distributed in the circular muscularis and around the myenteric plexus,and formed a network.In CHPS group,NOS positive nerve fibers were absent in the hy- pertrophic circular muscularis,and scanty in the longitudinal muscularis.The neurons of the myenteric plexus were NOS positive.ICs was almost absent in the inner part of the circular mus- cularis and none around the myenteric plexus.Only a few ICs were found near the submucosal lay- er of the circular muscularis in the specimens for CHPS.Conclusions:These findings suggest an abnormal distribution of NOS and ICs in the pylorus be responsible for the functional obstruction of pylorus in patients with CHPS.