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婴儿肥厚性幽门狭窄患儿幽门肌中胰岛素样生长因子和血小板衍生生长因子系统增加。

Increased insulin-like growth factor and platelet-derived growth factor system in the pyloric muscle in infantile hypertrophic pyloric stenosis.

作者信息

Ohshiro K, Puri P

机构信息

Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.

出版信息

J Pediatr Surg. 1998 Feb;33(2):378-81. doi: 10.1016/s0022-3468(98)90467-5.

Abstract

BACKGROUND

Infantile hypertrophic pyloric stenosis (IHPS) is characterized by hypertrophy of the pyloric muscle. The etiology of IHPS is unknown. The growth of smooth muscle cells (SMC) is regulated by several growth factors. Insulin-like growth factor (IGF) and platelet-derived growth factor (PDGF) act synergistically to stimulate SMC proliferation. The effects of IGF-I and PDGF are mediated via their receptors.

METHODS

Full-thickness muscle biopsy specimens were obtained from eight IHPS patients (age range, 14 to 46 days) at pyloromyotomy and from eight age-matched controls without gastrointestinal disease at autopsy performed within 4 hours after death. Indirect three-step immunohistochemistry was performed using anti-IGF-I, IGF-I receptor alpha (IGF-IR alpha), IGF-IR beta, PDGF-BB and PDGF receptor (PDGF-R) antibodies and visualized by peroxidase staining.

RESULTS

The most striking difference between tissues from IHPS patients and controls was the marked increase in IGF-I, IGF-IR alpha, IGF-IR beta and PDGF-R in the hypertrophic circular muscle layer, and, to a lesser degree, in the longitudinal muscle in pyloric stenosis.

CONCLUSION

The findings suggest that the upregulated local IGF and PDGF systems may play a role in the development of pyloric muscle hypertrophy in IHPS.

摘要

背景

婴儿肥厚性幽门狭窄(IHPS)的特征是幽门肌肥厚。IHPS的病因尚不清楚。平滑肌细胞(SMC)的生长受多种生长因子调节。胰岛素样生长因子(IGF)和血小板衍生生长因子(PDGF)协同作用刺激SMC增殖。IGF-I和PDGF的作用通过其受体介导。

方法

在幽门肌切开术时从8例IHPS患者(年龄范围14至46天)获取全层肌肉活检标本,并在死后4小时内从8例年龄匹配的无胃肠道疾病的对照者尸检中获取标本。使用抗IGF-I、IGF-I受体α(IGF-IRα)、IGF-IRβ、PDGF-BB和血小板衍生生长因子受体(PDGF-R)抗体进行间接三步免疫组织化学,并通过过氧化物酶染色进行可视化。

结果

IHPS患者组织与对照组织之间最显著的差异是肥厚的环形肌层中IGF-I、IGF-IRα、IGF-IRβ和PDGF-R显著增加,在幽门狭窄的纵行肌层中增加程度较小。

结论

研究结果表明,局部IGF和PDGF系统上调可能在IHPS幽门肌肥厚的发生中起作用。

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