Passarge E, Bartsch-Sandhoff M, Rehder H
Teratology. 1982 Apr;25(2):221-5. doi: 10.1002/tera.1420250211.
Two live-born children and one fetus were found to be affected by autosomal imbalance consisting of a partial duplication of the long arm of chromosome 5 (5q33 to qter) and partial deficiency of the short arm of chromosome 8 (8p23 to pter), resulting from segregation of a paternal translocation heterozygote t(5;8) (q33;p23). The phenotype of the children was rather similar to that of the fetus. The main features included craniofacial dysmorphia consisting of downward slant of eyelids and corners of the mouth, flat and widened nasal bridge, prominent philtrum, and small mandible. Ventricle and atrium septum defects were associated with overriding aorta and right heart hypertrophy in all three. Statomotoric retardation was evident postnatally in the two children. This observation indicates that structural autosomal imbalance may produce a fetal phenotype that is specific for the karyotype and comparable to that seen in the newborn.
发现两名活产儿和一名胎儿受到常染色体不平衡的影响,这种不平衡包括5号染色体长臂(5q33至qter)的部分重复和8号染色体短臂(8p23至pter)的部分缺失,这是由于父亲的易位杂合子t(5;8) (q33;p23) 的分离所致。这些儿童的表型与胎儿的表型相当相似。主要特征包括颅面畸形,表现为眼睑和嘴角向下倾斜、鼻梁扁平且变宽、人中突出和下颌小。所有三名患者均存在心室和心房间隔缺损,并伴有主动脉骑跨和右心室肥厚。两名儿童出生后明显存在运动发育迟缓。这一观察结果表明,常染色体结构不平衡可能产生一种特定于核型的胎儿表型,且与新生儿所见的表型相当。