Pharoah Peter O D
FSID Unit of Perinatal and Paediatric Epidemiology, Department of Public Health, University of Liverpool, Liverpool, United Kingdom.
Twin Res Hum Genet. 2005 Dec;8(6):543-50. doi: 10.1375/183242705774860141.
Congenital anomalies are a major cause of fetal and neonatal death and of childhood morbidity. Chromosomal and other genetic abnormalities, environmental teratogens and some nutritional deficiencies account for some congenital anomalies but the majority are of unknown etiology. The hypothesis is here proposed that a significant proportion of congenital anomalies and cerebral palsy of unknown etiology are attributable to a monozygotic multiple conception with monochorionic placentation and that these anomalies, even in singletons, may be explained by early, unrecognized or unrecorded loss of one conceptus in a monochorionic monozygotic conception. The pathological mechanism is hemodynamic instability with episodes of acute feto-fetal transfusion that produce ischemic organ impairment in either or both twins. The resultant clinical abnormality will depend on range of severity (fetal death, infant death, congenital anomaly, normal infant), site or combination of sites (which organ[s] present[s] with the congenital anomaly) and timing (early, middle or late in gestation as shown by variation in brain pathology that is observed).
先天性异常是导致胎儿和新生儿死亡以及儿童发病的主要原因。染色体及其他遗传异常、环境致畸物和一些营养缺乏可导致部分先天性异常,但大多数先天性异常的病因不明。本文提出一种假说,即相当一部分病因不明的先天性异常和脑瘫可归因于单绒毛膜胎盘的单卵多胎妊娠,并且这些异常即使在单胎妊娠中,也可能是由于单绒毛膜单卵妊娠中一个胚胎早期未被识别或记录的丢失所致。其病理机制是血流动力学不稳定伴急性胎儿 - 胎儿输血发作,这会导致其中一个或两个胎儿出现缺血性器官损伤。由此产生的临床异常将取决于严重程度范围(胎儿死亡、婴儿死亡、先天性异常、正常婴儿)、部位或部位组合(哪些器官出现先天性异常)以及时间(如观察到的脑病理学变化所示,妊娠早期、中期或晚期)。