Kaulins Ricards, Rozite Laura Ramona, Pilmane Mara, Petersons Aigars
Institute of Anatomy and Anthropology, Riga Stradins University, 9 Kronvalda Boulevard, LV-1010 Riga, Latvia.
Department of Pediatric Surgery, Children's Clinical University Hospital, 45 Vienibas gatve Street, LV-1004 Riga, Latvia.
Diagnostics (Basel). 2021 Feb 12;11(2):289. doi: 10.3390/diagnostics11020289.
Congenital diaphragm hernia (CDH) is a congenital disease that occurs during prenatal development. Although the morbidity and mortality rate is rather significant, the pathogenesis of CDH has been studied insignificantly due to the decreased accessibility of human pathological material. Therefore the aim of our work was to evaluate growth factors (transforming growth factor-beta (TGF-β), basic fibroblast growth factor (bFGF), insulin-like growth factor 1 (IGF-1), hepatocyte growth factor (HGF)) and their receptors (fibroblast growth factor receptor 1 (FGFR1), insulin-like growth factor 1 (IGF-1R)), muscle (dystrophin, myosin, alpha actin) and nerve quality (nerve growth factor (NGF), nerve growth factor receptor (NGFR), neurofilaments (NF)) factors, local defense factors (ß-defensin 2, ß-defensin 4), programmed cell death (TUNEL), and separate gene (Wnt-1) expression in human pathological material to find immunohistochemical marker differences between the control and the CDH patient groups. A semi-quantitative counting method was used for the evaluation of the tissues and structures in the Biotin-Streptavidin-stained slides. Various statistically significant differences were found in immunoreactive expression between the patient and the control group tissue and the morphological structures as well as very strong, strong, and moderate correlations between immunoreactives in different diaphragm cells and structures. These significant changes and various correlations indicate that multiple morphopathogenetic pathways are affected in CDH pathogenesis. This work contains the evaluation of the causes for these changes and their potential involvement in CDH pathogenesis.
先天性膈疝(CDH)是一种发生于产前发育过程中的先天性疾病。尽管其发病率和死亡率相当高,但由于获取人类病理材料的难度增加,对CDH发病机制的研究较少。因此,我们研究的目的是评估生长因子(转化生长因子-β(TGF-β)、碱性成纤维细胞生长因子(bFGF)、胰岛素样生长因子1(IGF-1)、肝细胞生长因子(HGF))及其受体(成纤维细胞生长因子受体1(FGFR1)、胰岛素样生长因子1受体(IGF-1R))、肌肉(肌营养不良蛋白、肌球蛋白、α-肌动蛋白)和神经质量(神经生长因子(NGF)、神经生长因子受体(NGFR)、神经丝(NF))因子、局部防御因子(β-防御素2、β-防御素4)、程序性细胞死亡(TUNEL)以及人类病理材料中单个基因(Wnt-1)的表达,以发现对照组和CDH患者组之间免疫组织化学标志物的差异。采用半定量计数法评估生物素-链霉亲和素染色切片中的组织和结构。在患者组和对照组组织之间以及形态结构的免疫反应性表达中发现了各种具有统计学意义的差异,并且不同膈肌细胞和结构中的免疫反应物之间存在非常强、强和中度的相关性。这些显著变化和各种相关性表明,CDH发病机制中多种形态发病途径受到影响。这项研究评估了这些变化的原因及其在CDH发病机制中的潜在作用。