Moessinger A C
Pediatrics. 1983 Dec;72(6):857-63.
Rat fetuses were paralyzed by daily transuterine injections of curare from day 18 of gestation until term (day 21). The following anomalies were noted at the time of delivery: multiple joint contractures, pulmonary hypoplasia, micrognathia, fetal growth retardation, short umbilical cords, and polyhydramnios. Neither sham-operated nor untouched littermate control fetuses had any of these anomalies. The group of anomalies (or deformation sequence) obtained with this animal model is presumed to result from the paralytic effect of curare. This phenotype bears a striking resemblance to the syndrome of ankyloses, facial anomalies, and pulmonary hypoplasia (also known as Pena and Shokeir I), presumably inherited in an autosomal recessive manner. It is suggested that this phenotype is not specific but, rather, represents a deformation sequence which results from fetal immobilization or akinesia. Diagnostic evaluation of patients with this group of anomalies should include the identification of the underlying pathologic process (etiology of the akinesia) to allow for proper classification and genetic counseling.
从妊娠第18天至足月（第21天），每天经子宫给大鼠胎儿注射箭毒使其麻痹。分娩时发现以下异常情况：多处关节挛缩、肺发育不全、小颌畸形、胎儿生长迟缓、脐带短和羊水过多。假手术组和未处理的同窝对照胎儿均未出现这些异常情况。用该动物模型获得的这组异常（或变形序列）被认为是箭毒麻痹作用的结果。这种表型与强直性脊柱炎、面部畸形和肺发育不全综合征（也称为佩纳和绍凯尔I型）极为相似，据推测是以常染色体隐性方式遗传。有人提出，这种表型并非特异性的，而是代表一种由胎儿固定或运动不能导致的变形序列。对患有这组异常的患者进行诊断评估时，应包括确定潜在的病理过程（运动不能的病因），以便进行正确分类和遗传咨询。
