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目的探讨甲状腺转录因子-1(TTF-1)和错配修复基因2(h MSH-2)在儿童先天性肺气道畸形(CPAM)中的表达及意义。方法采用免疫组化Eli Vision法检测TTF-1和h MSH-2蛋白在52例先天性肺气道畸形手术存档蜡块标本和9例正常肺组织中的表达。结果在正常儿童肺组织中TTF-1主要在远端细支气管的非纤毛柱状上皮及肺泡Ⅱ型上皮的细胞核内表达。在病例组中,TTF-1主要表达在囊壁的柱状上皮、立方上皮细胞的细胞核内。在CPAMⅠ型、Ⅱ型、Ⅲ型中TTF-1阳性率分别为2%、40%、80%。统计学分析结果表明三者之间表达有明显差异(P<0.01)。h MSH-2蛋白阳性产物在儿童正常肺组织定位于支气管及肺泡上皮细胞的细胞核内,在CPAM中,h MSH-2蛋白染色主要位于囊壁上皮的细胞核内,其阳性率明显低于正常肺组织(P<0.05)。在CPAMⅠ、Ⅱ型的表达无明显差异(P>0.05),但在CPAMⅠ、Ⅱ型中的表达明显强于CPAMⅢ型(P<0.05)。结论 TTF-1在CPAMⅠ、Ⅱ、Ⅲ型中的表达有差异,可以作为CPAM组织分型的一个重要标记。h MSH-2蛋白在病例组CPAMⅠ、Ⅱ、Ⅲ型中表达量均低于正常儿童肺组织,提示在CPAM中有h MSH-2基因转录水平的下降,参与CPAM的形成。
Objective To investigate the expression of thyroid transcription factor-1 (TTF-1) and mismatch repair gene 2 (h MSH-2) in children with congenital pulmonary artery disease (CPAM). Methods The expression of TTF-1 and h-MSH-2 protein in 52 paraffin-embedded specimens of paravalvulmonary congenital abnormalities and 9 normal lung tissues were detected by immunohistochemical Eli Vision method. Results TTF-1 was mainly expressed in the nuclei of non-ciliated columnar epithelium and type II alveolar epithelium of distal bronchioles in normal children. In the case group, TTF-1 was mainly expressed in the cytoplasm of columnar epithelium and cuboidal epithelial cells in the cyst wall. The positive rates of TTF-1 in CPAMⅠ, Ⅱ and Ⅲ were 2%, 40% and 80% respectively. Statistical analysis showed that there was a significant difference between the three (P <0.01). h The expression of MSH-2 protein in normal lung tissue of children was localized in the nuclei of bronchial and alveolar epithelial cells. In CPAM, h MSH-2 protein staining was mainly located in the nucleus of the cyst wall epithelium, and its positive rate was significantly lower than that of normal lung Tissue (P <0.05). There was no significant difference in the expression of CPAM Ⅰ and Ⅱ (P> 0.05), but the expression in CPAM Ⅰ and Ⅱ was significantly stronger than that of CPAM Ⅲ (P <0.05). Conclusion The expression of TTF-1 in CPAM Ⅰ, Ⅱ and Ⅲ is different and can be used as an important marker of CPAM tissue typing. h The expression level of MSH-2 in CPAMⅠ, Ⅱ and Ⅲ of case group was lower than that in normal children, suggesting that MSH-2 gene transcription was decreased in CPAM and involved in the formation of CPAM.