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患者“平衡”易位 9;15 与隐匿性 9q34 重复和 15q21q25 缺失的表型-基因型相关性。

Phenotype-genotype correlation of a patient with a "balanced" translocation 9;15 and cryptic 9q34 duplication and 15q21q25 deletion.

机构信息

Department of Pediatrics, University Hospital of Heraklion, Crete, Greece.

出版信息

Am J Med Genet A. 2010 Jun;152A(6):1515-22. doi: 10.1002/ajmg.a.33302.

DOI:10.1002/ajmg.a.33302
PMID:20503328
Abstract

We report on a 2-year-old boy with intellectual disabilities, distinctive facies, hypotonia, cardiac, and renal malformations. During his infancy he had recurrent episodes of apnea, cyanosis, and bradycardia. Chromosomal analysis showed a de novo apparently balanced translocation 46,XY,t(9;15)(q31;q26)dn. The use of array-comparative genomic hybridization (CGH) however, revealed the presence of additional cryptic complex chromosomal rearrangements involving a approximately 5-5.8 Mb distal duplication on chromosome 9 (9q34.1 --> 9q34.3), and deletions on three separate regions of chromosome 15 adding to approximately 8.1-12.2 Mb (15q21.2 --> 15q21.3, 15q22.31 --> 15q23, 15q25.1 --> 15q25.2). During confirmation with fluorescence in situ hybridization (FISH) an inversion was unexpectedly revealed on chromosome 15 (15q21.1 --> 15q21.2). To our knowledge this is the first patient reported whose phenotype is due to partial trisomy 9q, and complex interstitial deletions of 15q, not involving the Prader-Willi/Angelman region and encompassing the critical region 15q21q25. We provide correlation between the clinical findings of our patient and the phenotype of the 9q34 duplication syndrome, the 15q21, and the 15q25 deletion syndromes.

摘要

我们报告了一例 2 岁男孩,其具有智力障碍、特殊面容、肌张力减退、心脏和肾脏畸形。在婴儿期,他反复出现呼吸暂停、发绀和心动过缓。染色体分析显示存在新生的、似乎平衡的易位 46,XY,t(9;15)(q31;q26)dn。然而,使用阵列比较基因组杂交 (CGH) 发现存在额外的隐匿性复杂染色体重排,涉及 9 号染色体远端约 5-5.8Mb 的重复(9q34.1 --> 9q34.3),以及 15 号染色体三个不同区域的缺失,总计约 8.1-12.2Mb(15q21.2 --> 15q21.3、15q22.31 --> 15q23、15q25.1 --> 15q25.2)。在使用荧光原位杂交 (FISH) 进行确认时,出乎意料地发现 15 号染色体上存在倒位(15q21.1 --> 15q21.2)。据我们所知,这是首例报道的表型归因于 9q 部分三体、15q 复杂的中间缺失的患者,不涉及 Prader-Willi/Angelman 区域,并且包含 15q21q25 的关键区域。我们提供了我们患者的临床发现与 9q34 重复综合征、15q21 缺失综合征和 15q25 缺失综合征表型之间的相关性。

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