Fára M, Jelínek R, Peterka M, Dostál M, Hrivnáková J
Department of Plastic Surgery, Charles University Medical Faculty of Hygiene, Prague, Czechoslovakia.
Acta Univ Carol Med Monogr. 1988;124:1-143.
In spite of the existing huge number of data on palate development as well as the incidence, experimental induction and clinical treatment of orofacial clefts, no unitary concept has been made available that would make possible their sorting out, further interpretation and extrapolation. The aim of this monograph has been to provide firm grounds for managing the data within categories consistent with the general principles of teratogenesis reformulated and extended upon the theory of morphogenetic systems, and, upon this basis, to evaluate the present chances of preventing the origin of orofacial clefts. Chapter 1 introduces the problem of birth defects that possess some distinct features in common with the recognised prime problems of present medicine, that is neoplastic and cardiovascular diseases. Orofacial clefts represent a substantial component of the human birth-defect spectrum that is a mere remnant of the original volume of teratogenesis estimated as affecting about 35% of human embryos. The merciful process of prenatal extinction of abnormal conceptuses, or terathanasia, reduces this eminent figure by approximately one order of magnitude. Basing upon the prevalence of clefts in embryos and infants we may say that the prenatal extinction of individuals with orofacial clefts lies somewhere between 70-90%. Chapter 2 deals with the history of recognising and formulating the general principles of teratology that go back to Isidore Geoffroy Saint-Hilaire. Estimating the contribution of the great personalities such as Dareste, Schwalbe, and J. G. Wilson, the chapter enumerates and describes the ten principles of teratogenesis as having arisen from the known rules extended and reformulated by the original theory of morphogenetic systems. In their sum, the principles constitute a deductive system defining teratogenesis at several levels of bioorganisation, capable of predicting the large-scale effects of environmental impact on animal and human reproduction. Chapter 3 presents the orofacial clefts in the light of the theory of morphogenetic systems. Palatal morphogenesis is accomplished under the conditions of extraordinary spatial complexity and extends over a relatively long period of development. Several morphogenetic subsystems may be distinguished, namely the morphogenetic subsystem (smgs) of facial outgrowths, the smgs of palatal shelves, the smgs of the glossomandibular complex and, eventually, the smgs of the axial cervical region, acting at different phases of palatal development.(ABSTRACT TRUNCATED AT 400 WORDS)
尽管目前已有大量关于腭部发育以及口面部裂隙的发生率、实验诱导和临床治疗的数据，但尚未形成一个统一的概念，以便对这些数据进行整理、进一步解读和外推。这本专著的目的是为按照形态发生系统理论重新阐述和扩展的致畸学一般原则，在各类别中管理这些数据提供坚实基础，并在此基础上评估目前预防口面部裂隙发生的可能性。第1章介绍了出生缺陷问题，这些问题与当前医学公认的主要问题（即肿瘤和心血管疾病）有一些明显的共同特征。口面部裂隙是人类出生缺陷谱的一个重要组成部分，而人类出生缺陷谱只是最初估计影响约35%人类胚胎的致畸发生率的残余部分。异常胚胎在产前消亡这一仁慈过程，即“致畸安乐死”，使这一显著数字减少了大约一个数量级。根据胚胎和婴儿中裂隙的患病率，我们可以说，口面部裂隙个体的产前消亡率在70%至90%之间。第2章论述了致畸学一般原则的认识和形成历史，可追溯到伊西多尔·若弗鲁瓦·圣伊莱尔。本章评估了达雷斯特、施瓦尔贝和J.G.威尔逊等伟大人物的贡献，列举并描述了致畸学的十条原则，这些原则源于形态发生系统原始理论扩展和重新阐述的已知规则。这些原则总体上构成了一个演绎系统，在生物组织的几个层面上定义致畸学，能够预测环境影响对动物和人类生殖的大规模效应。第3章根据形态发生系统理论阐述口面部裂隙。腭部形态发生是在极其复杂的空间条件下完成的，并且在相对较长的发育时期内进行。可以区分几个形态发生子系统，即面部生长的形态发生子系统（smgs）、腭突的smgs、舌下颌复合体的smgs，以及最终在腭部发育不同阶段起作用的轴颈区域的smgs。（摘要截取自400字）
