THE DEVELOPMENTAL FIELD CONCEPT IN CLINICAL GENETICS
THE DEVELOPMENTAL FIELD CONCEPT IN CLINICAL GENETICS
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DOI：
10.1016/s0022-3476(82)80337-5
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发表时间：
1982-01-01
影响因子：
5.1
通讯作者：
OPITZ, JM
中科院分区：
文献类型：
--
作者：
OPITZ, JM
场是胚胎的那些部分，在这些部分中，与这些部分相适应的复杂结构的发育过程以空间有序、时间同步和表态分层的方式被控制和协调。场发育的扰动可能被纠正，或者可能导致异常或不完全分化的异常。发育场存在的有力证据来自于对由两种或两种以上不同原因引起的相同畸形的观察;这识别了作为单个发育单位响应的胚胎结构组。在一个领域中，畸形发生原因的早期作用可能导致广泛的缺陷（例如，环状前脑无裂畸形），后来作用于较小的缺陷（例如，单上中切牙为常染色体显性无前脑畸形的最轻度表现）。原基之间“长距离”诱导关系的干扰可能模拟畸形综合征的存在（例如，肾和肢体异常作为顶肾发育区的障碍）。发育领域的生物学是复杂的，但可以通过比较解剖学和胚胎学，胚胎发生学，实验胚胎学，发育遗传学和畸形学的知识来理解。它挑战临床医生更深入地了解发展，以便更好地照顾畸形及其家人。
Fields are those parts of the embryo in which the processes of development of complex structure appropriate to those parts are controlled and coordinated in a spatially ordered, temporally synchronized, and epimorphically hierarchical manner. Disturbances of field development may be corrected or may lead to anomalies of abnormal or incomplete differentiation. Powerful evidence for the existence of developmental fields comes from the observation of identical malformations resulting from two or more different causes; this identifies groups of embryonic structures that respond as a single developmental unit. Early action of a dysmorphogenetic cause in a field may lead to an extensive defect (e.g., cyclopic holoprosencephaly), later action to a lesser defect (e.g., single upper central incisor as mildest expression of autosomal-dominant holoprosencephaly). Disturbances of “long-distance” inductive relationships between primordia may simulate the presence of a malformation syndrome (e.g., renal and limb anomalies as disturbance of the acrorenal developmental field). The biology of developmental fields is complex but accessible through knowledge of comparative anatomy and embryology, phylogeny, experimental embryology, developmental genetics, and teratology. It challenges the clinician to a deeper understanding of development in order to give better care to the malformed and their families.