Schinzel A
Soz Praventivmed. 1985;30(1):13-7. doi: 10.1007/BF02075722.
Two to five percent of newborn infants present with congenital structural defects. This incidence contains mostly rare to extremely rare conditions. Aetiologically, these consist of defects secondary to genetic and environmental causes, with a third group of defects which are presumed to be due to an unfavorable combination of genetic and environmental factors. In many individual patients, however, allocation into one of these groups is difficult or even impossible. The following clinical and epidemiologic observations may help in some cases to arrive at a classification; pedigree, pregnancy history (infections, teratogens, bleeding, foetal movements etc.), clinical picture and various complementary investigations. Pathogenetically, we may classify congenital structural defects into primary defects (malformations) and secondary ones, if a primarily correctly formed structure has secondarily been deformed (deformation) or destroyed (disruption). In many cases, these clinical observations will then allow a classification into one of the groups. The importance of the determination of the aetiology and pathogenesis of structural defects for the individual family is in the subsequent implications for determination of the risk of recurrence.
2%至5%的新生儿存在先天性结构缺陷。这种发病率包含的大多是罕见至极罕见的病症。从病因学角度来看,这些病症包括继发于遗传和环境因素的缺陷,还有第三类缺陷,推测是由遗传和环境因素的不利组合导致的。然而,在许多个体患者中,很难甚至不可能将其归入这些类别中的某一类。以下临床和流行病学观察在某些情况下可能有助于进行分类:家族谱系、妊娠史(感染、致畸物、出血、胎动等)、临床表现以及各种辅助检查。从发病机制上讲,如果一个原本正常形成的结构继发变形(畸形)或破坏(中断),我们可以将先天性结构缺陷分为原发性缺陷(畸形)和继发性缺陷。在许多情况下,这些临床观察结果随后将有助于将其归入其中一类。确定结构缺陷的病因和发病机制对个体家庭的重要性在于其对复发风险判定的后续影响。