Luis A L, Pederiva F, Encinas J L, Ruiz E, Rodriguez J I, Martinez L, Tovar J A
Hospital Infantil La Paz, Pediatric Surgery, Madrid, Spain.
Eur J Pediatr Surg. 2011 Aug;21(4):246-9. doi: 10.1055/s-0031-1273778. Epub 2011 Apr 8.
Parathyroid and thymic anomalies related to embryonic neural crest dysfunction have been demonstrated in rats with congenital diaphragmatic hernia (CDH). These rats, like infants with CDH, have conotruncal, facial, and other neurocristal defects. The present study examines whether parafollicular C-cells (CC) of the thyroid, whose embryogenesis is related to that of the parathyroids and thymus, might also be abnormal in babies with CDH.
Autopsy sections of the thyroids of 12 babies dead from CDH and of 11 controls were stained with anti-calcitonin antibodies. Calcitonin-stained areas and the histological distribution of CC within the thyroid gland were assessed. Mann-Whitney tests were used for comparison, with p<0.05 considered significant.
The proportion of stained surface to total thyroid surface was significantly smaller in CDH babies than in controls (0.035±0.030% vs. 0.072±0.052%, p<0.05). A normal central CC location was demonstrated in both groups.
Parafollicular thyroid C-cells are deficient in patients with CDH. These findings further support the involvement of neural crest dysregulation in the pathogenesis of CDH and the pertinence of using this experimental model to investigate the human condition. The clinical effects of this anomaly are unknown and probably irrelevant, but they are currently under scrutiny.
先天性膈疝(CDH)大鼠已证实存在与胚胎神经嵴功能障碍相关的甲状旁腺和胸腺异常。这些大鼠与患有CDH的婴儿一样,存在圆锥动脉干、面部及其他神经嵴缺陷。本研究旨在探讨甲状腺滤泡旁C细胞(CC),其胚胎发生与甲状旁腺和胸腺相关,在患有CDH的婴儿中是否也存在异常。
对12例死于CDH的婴儿及11例对照婴儿的甲状腺尸检切片用抗降钙素抗体进行染色。评估降钙素染色区域及CC在甲状腺内的组织学分布。采用Mann-Whitney检验进行比较,p<0.05认为具有统计学意义。
CDH婴儿甲状腺染色面积占甲状腺总面积的比例显著低于对照组(0.035±0.030%对0.072±0.052%,p<0.05)。两组均显示CC位于甲状腺中央的位置正常。
CDH患者的甲状腺滤泡旁C细胞数量不足。这些发现进一步支持神经嵴调节异常参与CDH的发病机制,以及使用该实验模型研究人类疾病的相关性。这种异常的临床影响尚不清楚且可能无关紧要,但目前正在研究中。