Gazi University Faculty of Medicine, Department of Medical Genetics, Besevler, Ankara, Turkey.
Gene. 2013 Jul 25;524(2):355-60. doi: 10.1016/j.gene.2013.04.053. Epub 2013 May 1.
Isolated partial duplication of the long arm of chromosome 11 is very rare. The main features are dysmorphic facial features, pre/postnatal growth retardation, speech delay, mental retardation, hypotonia, microcephaly, and cardiac, vertebral, limb and genital anomalies. In this case, we report a patient with partial trisomy of 11q13.5→qter due to a de novo rearrangement consisting of the whole X chromosome and part of chromosome 11; 46,X,der(X)(Xqter→Xp22.33::11q13.5→11qter). Additional findings were a separated clavicle, lacrimal duct stenosis and prenatally detected renal hypoplasia. SNP array results revealed a duplication between 11q13.5 and 11qter, measuring 58 Mb, from nucleotide 76,601,607 to 134,926,021. As a result, molecular karyotyping could be performed in such cases in order to establish a definite phenotype-genotype correlation using conventional or molecular cytogenetics techniques.
染色体 11 长臂的孤立部分重复非常罕见。主要特征为畸形面容、出生前/后生长迟缓、言语迟缓、智力障碍、肌张力低下、小头畸形,以及心脏、椎体、四肢和生殖器异常。在此情况下,我们报告了 1 例患者存在部分 11q13.5→qter 三体性,由从头发生的重排引起,包括整个 X 染色体和部分 11 号染色体;46,X,der(X)(Xqter→Xp22.33::11q13.5→11qter)。其他发现包括锁骨分离、泪管狭窄和产前检测到的肾发育不良。SNP 微阵列结果显示在 11q13.5 和 11qter 之间存在 58Mb 的重复,从核苷酸 76,601,607 到 134,926,021。因此,可以在这些情况下进行分子核型分析,以便使用常规或分子细胞遗传学技术建立明确的表型-基因型相关性。