Pueyo Gil C, Oshiro K, Elías Pollina J, Esteban Ibarz J A, Puri P
Hospital Infantil Universitario Miguel Servet, Zaragoza.
Cir Pediatr. 2001 Jul;14(3):103-7.
Infantile hypertrophic pyloric stenosis (IHPS) consists of hypertrophy of the muscular layer of the pylorus. Its etiology is still unknown. In the last years only few jobs that studied the extracellular matrix (ECM) in the muscular layer in the IHPS have been reported. Our aim was to investigate the expression of two ECM molecules: chondroitin-sulfate proteoglycan (CSPG) and fibronectin (FN), and fibroblasts.
Full-thickness muscle biopsy specimens were obtained from 33 IHPS patients at pyloromyotomy and 12 controls. Indirect immunohistochemistry was performed using CSPG, FN and fibroblasts monoclonal antibodies. The results were showed by a semiquantitative scale as follows: strong (++), moderate (+), weak (+/-), and absent (-).
We demonstrated that the CSPG immunoreactivity was localized in the connective tissue septa and the expression of FN molecules in the pericellular space. Both molecules were significantly the increased in the muscle layer of the pylorus with IHPS in relation to control pylorus. We also demonstrated a marked increased expression in the number of fibroblasts in the muscle layer of the pylorus with IHPS. Even-though the most striking increase was localized in the septa, we also observed great number of fibroblasts amongst the smooth muscle cells.
We suggest that IHPS is characterized, not only by the muscle layer hypertrophy, but also by the increase of several ECM molecules, such as CSPG and FN. We also think that the increase of fibroblast could explain the higher expression of both ECM molecules in the muscle layer of pylorus in IHPS.
婴儿肥厚性幽门狭窄(IHPS)表现为幽门肌层肥厚。其病因尚不清楚。近年来,仅有少数研究报道了IHPS肌层细胞外基质(ECM)的情况。我们的目的是研究两种ECM分子:硫酸软骨素蛋白聚糖(CSPG)和纤连蛋白(FN)以及成纤维细胞的表达。
在幽门肌切开术时从33例IHPS患者获取全层肌肉活检标本,并选取12例对照。使用CSPG、FN和成纤维细胞单克隆抗体进行间接免疫组织化学。结果以半定量量表显示如下:强(++)、中(+)、弱(+/-)和无(-)。
我们发现CSPG免疫反应性定位于结缔组织间隔,FN分子表达于细胞周间隙。与对照幽门相比,IHPS患者幽门肌层中这两种分子均显著增加。我们还发现IHPS患者幽门肌层中成纤维细胞数量显著增加。尽管最显著的增加位于间隔,但我们在平滑肌细胞之间也观察到大量成纤维细胞。
我们认为,IHPS不仅以肌层肥厚为特征,还以几种ECM分子如CSPG和FN的增加为特征。我们还认为,成纤维细胞的增加可以解释IHPS患者幽门肌层中这两种ECM分子的高表达。