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与杜氏肌营养不良相关的植入前基因诊断

Preimplantation genetic diagnosis associated to Duchenne muscular dystrophy.

作者信息

Bianco Bianca, Christofolini Denise Maria, Conceição Gabriel Seixas, Barbosa Caio Parente

机构信息

Faculdade de Medicina do ABC, Santo André, SP, Brazil.

出版信息

Einstein (Sao Paulo). 2017 Oct-Dec;15(4):489-491. doi: 10.1590/S1679-45082017RC3994. Epub 2017 Sep 21.

Abstract

Duchenne muscular dystrophy is the most common muscle disease found in male children. Currently, there is no effective therapy available for Duchenne muscular dystrophy patients. Therefore, it is essential to make a prenatal diagnosis and provide genetic counseling to reduce the birth of such boys. We report a case of preimplantation genetic diagnosis associated with Duchenne muscular dystrophy. The couple E.P.R., 38-year-old, symptomatic patient heterozygous for a 2 to 47 exon deletion mutation in DMD gene and G.T.S., 39-year-old, sought genetic counseling about preimplantation genetic diagnosis process. They have had a 6-year-old son who died due to Duchenne muscular dystrophy complications. The couple underwent four cycles of intracytoplasmic sperm injection (ICSI) and eight embryos biopsies were analyzed by polymerase chain reaction (PCR) for specific mutation analysis, followed by microarray-based comparative genomic hybridisation (array CGH) for aneuploidy analysis. Preimplantation genetic diagnosis revealed that two embryos had inherited the maternal DMD gene mutation, one embryo had a chromosomal alteration and five embryos were normal. One blastocyst was transferred and resulted in successful pregnancy. The other embryos remain vitrified. We concluded that embryo analysis using associated techniques of PCR and array CGH seems to be safe for embryo selection in cases of X-linked disorders, such as Duchenne muscular dystrophy.

摘要

杜氏肌营养不良症是男性儿童中最常见的肌肉疾病。目前,尚无针对杜氏肌营养不良症患者的有效治疗方法。因此,进行产前诊断并提供遗传咨询以减少此类男孩的出生至关重要。我们报告了一例与杜氏肌营养不良症相关的植入前基因诊断病例。38岁的E.P.R.是一名有症状的患者,其DMD基因存在2至47外显子缺失突变,为杂合子;39岁的G.T.S.寻求关于植入前基因诊断过程的遗传咨询。他们有一个6岁的儿子,因杜氏肌营养不良症并发症去世。这对夫妇接受了四个周期的卵胞浆内单精子注射(ICSI),并通过聚合酶链反应(PCR)对八个胚胎活检样本进行了特定突变分析,随后通过基于微阵列的比较基因组杂交(阵列CGH)进行非整倍体分析。植入前基因诊断显示,两个胚胎继承了母亲的DMD基因突变,一个胚胎存在染色体改变,五个胚胎正常。移植了一个囊胚并成功妊娠。其他胚胎仍处于冷冻状态。我们得出结论,在杜氏肌营养不良症等X连锁疾病的病例中,使用PCR和阵列CGH相关技术进行胚胎分析对于胚胎选择似乎是安全的。

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