Iwakoshi Mie, Okamoto Nobuhiko, Harada Naoki, Nakamura Tsuyoshi, Yamamori Shunji, Fujita Hiroko, Niikawa Norio, Matsumoto Naomichi
Nishinomiya Municipal Wakaba-en, Nishinomiya, Japan.
Am J Med Genet A. 2004 Apr 30;126A(3):278-83. doi: 10.1002/ajmg.a.20602.
We described three unrelated children with cryptic 9q34.3 rearrangements and similar clinical manifestations: two with 9q34.3 terminal deletions and the other with an unbalanced translocation involving 9q34.3-qter monosomy and 6p25-pter trisomy. Common features among the three we studied and the other six patients with 9q34.3 deletions in the literature include microcephaly, mental retardation (MR), hypotonic, and epileptic seizures. Their facial characteristics included flat face, arched eyebrows, synophrys, hypertelorism, short nose, anteverted nostrils, carp mouth, protruding tongue, micrognathia, and pointed chin. Other frequent abnormalities were cardiac abnormalities, cryptorchidism or hypospadias, and abnormal toes. These findings are characteristic enough to be a clinically recognizable syndrome.
我们描述了三名患有隐匿性9q34.3重排且临床表现相似的非亲缘关系儿童:两名患有9q34.3末端缺失,另一名患有涉及9q34.3 - qter单体和6p25 - pter三体的不平衡易位。我们研究的这三名患者与文献中其他六名9q34.3缺失患者的共同特征包括小头畸形、智力障碍(MR)、肌张力减退和癫痫发作。他们的面部特征包括面部扁平、眉弓高、眉毛相连、眼距增宽、鼻子短、鼻孔前倾、鲤鱼嘴、舌头突出、小颌畸形和尖下巴。其他常见异常包括心脏异常、隐睾或尿道下裂以及脚趾异常。这些发现具有足够的特征性,足以成为一种临床可识别的综合征。