Yu J, Gonzalez S, Rodriguez J I, Diez-Pardo J A, Tovar J A
Department of Surgery, Hospital Infantil Universitario La Paz, P. de la Castellana, 261, 28046 Madrid, Spain.
Pediatr Surg Int. 2001 May;17(4):294-8. doi: 10.1007/s003830100597.
Congenital diaphragmatic hernia (CDH) is often associated with other malformations. This study tests the hypothesis that the heart and great vessels, thymus, parathyroids, and thyroid might be abnormal in the rat model of CDH as a result of disturbed neural-crest development. Time-mated pregnant rats were fed either 100 mg 2-4-dichlorophenyl-p-nitrophenyl ether (nitrofen) or vehicle on gestational day 9.5. Diaphragm, lung, heart, and thymic malformations were sought after dissection and the parathyroids and thyroid were histologically investigated in term fetuses. Ten control fetuses had no malformations, whereas 22 of 32 nitrofen fetuses had CDH and 20 had cardiovascular defects like narrow pulmonary outflow tract (n = 7), aberrant right subclavian artery (n = 7), ventricular septal defect (n = 4), atrial septal defect (n = 4), tetralogy of Fallot (n = 2), double-outflow right ventricle (n = 2), right ductus arteriosus (n = 2), and others. The thymus was present but was significantly hypoplastic in all nitrofen fetuses and was ectopic or single-lobed in 28% of them while the parathyroid glands were unilaterally absent or ectopic in 50%. The thyroid was only minimally malformed or ectopic. In conclusion, malformations of structures derived from the pharyngeal arches are likely neural-crest related in rats exposed to nitrofen.
先天性膈疝（CDH）常与其他畸形相关。本研究检验了这样一个假设：在CDH大鼠模型中，由于神经嵴发育紊乱，心脏、大血管、胸腺、甲状旁腺和甲状腺可能会出现异常。在妊娠第9.5天，将按时间交配的怀孕大鼠分别喂食100毫克2-4-二氯苯基-对硝基苯基醚（硝呋烯腙）或赋形剂。在解剖后寻找膈肌、肺、心脏和胸腺畸形，并对足月胎儿的甲状旁腺和甲状腺进行组织学研究。10只对照胎儿无畸形，而32只硝呋烯腙处理的胎儿中有22只患有CDH，20只患有心血管缺陷，如肺动脉流出道狭窄（n = 7）、右锁骨下动脉异常（n = 7）、室间隔缺损（n = 4）、房间隔缺损（n = 4）、法洛四联症（n = 2）、右心室双出口（n = 2）、右动脉导管（n = 2）等。所有硝呋烯腙处理的胎儿均有胸腺，但明显发育不全，其中28%的胸腺异位或为单叶，而甲状旁腺单侧缺如或异位的占50%。甲状腺仅有轻微畸形或异位。总之，在接触硝呋烯腙的大鼠中，源自咽弓的结构畸形可能与神经嵴有关。
