Dawrant Michael J, Giles Shay, Bannigan John, Puri Prem
Department of Paediatric Surgery, National Children's Hospital, Tallaght, Dublin 24, Ireland.
J Pediatr Surg. 2007 Oct;42(10):1652-8. doi: 10.1016/j.jpedsurg.2007.05.018.
BACKGROUND/PURPOSE: The Adriamycin rat model is an established model for vertebral, anal, cardiac, tracheal, esophageal, renal, and limb (VACTERL) anomalies and gastrointestinal atresias. Mice are the foremost mammal studied by developmental biologists, providing greater availability of molecular probes, antibodies, and transferable knowledge with transgenic studies. Only tracheoesophageal malformations have been previously described in the Adriamycin mouse model. The aim of this study was to carry out a dose-response analysis of the teratogenicity of Adriamycin in the mouse to determine the effect of the dose and timing of exposure in producing tracheoesophageal malformations and show if it causes other VACTERL anomalies.
CBA/Ca mice were accurately time mated (n = 30). Four different doses (0 [saline], 4, 5, and 6 mg/kg) of Adriamycin (EBEWE Pharma Ges.m.b.H. Nfg.KG, A-4866 Unterach, Austria) at 3 different timings of injections were compared. Dams received 2 intraperitoneal injections, 24 hours apart, commencing on day 7, 7.5, or 8. Fetuses were harvested on day 18. Anomalies were examined using a dissecting microscope and serial transverse sections.
Administering Adriamycin at 6 mg/kg on days 7 and 8 had the most teratogenic effect, with 80% of fetuses having 3 or more VACTERL anomalies: anorectal malformation, 100%; tracheoesophageal malformation, 50%; right-sided aortic arch, 58.3%; bladder agenesis/bilateral hydronephrosis, 100%.
This study establishes a mouse model that should provide insights into the cellular and molecular mechanisms underlying VACTERL anomalies.
背景/目的:阿霉素大鼠模型是一种用于研究椎体、肛门、心脏、气管、食管、肾脏和肢体(VACTERL)畸形以及胃肠道闭锁的既定模型。小鼠是发育生物学家研究最多的哺乳动物,在转基因研究中,分子探针、抗体和可转移知识的可用性更高。此前在阿霉素小鼠模型中仅描述过气管食管畸形。本研究的目的是对阿霉素在小鼠中的致畸性进行剂量反应分析,以确定暴露剂量和时间对产生气管食管畸形的影响,并观察其是否会导致其他VACTERL畸形。
对CBA/Ca小鼠进行精确的定时交配(n = 30)。比较了在3个不同注射时间给予的4种不同剂量(0 [生理盐水]、4、5和6 mg/kg)的阿霉素(EBEWE Pharma Ges.m.b.H. Nfg.KG,奥地利下阿赫A-4866)。母鼠从第7、7.5或8天开始,每隔24小时接受2次腹腔注射。在第18天收集胎儿。使用解剖显微镜和连续横切片检查畸形情况。
在第7天和第8天给予6 mg/kg的阿霉素具有最强的致畸作用,80%的胎儿有3种或更多的VACTERL畸形:肛门直肠畸形,100%;气管食管畸形,50%;右侧主动脉弓,58.3%;膀胱缺如/双侧肾积水,100%。
本研究建立了一个小鼠模型,该模型应能为深入了解VACTERL畸形的细胞和分子机制提供帮助。