Urbina Maria Teresa, Benjamin Isaac, Medina Randolfo, Jiménez José, Trías Laura, Lerner Jorge
Unifertes Fertility Unit, Caracas, Venezuela.
JBRA Assist Reprod. 2017 Dec 1;21(4):356-360. doi: 10.5935/1518-0557.20170062.
To discuss the implications of expanded genetic carrier screening for preconception purposes based on our practice.
One hundred and forty-three potential gamete donors aged 20-32 years old (µ=24, 127 females and 16 males), signed informed consent forms and were selected according to the REDLARA guidelines. Blood or saliva samples were examined by one of these genetic carrier screening methods: Genzyme screening for Cystic Fibrosis (CF), Fragile X and Spinal Muscular Atrophy (SMA); Counsyl Universal panel or Recombine Carrier Map.
Genotyping results for all donors were analyzed; 41% (58/143) of donors were identified as carriers for at least one condition. We found a carrier frequency of 1/24 for CF, 1/72 for SMA and 0/120 for Fragile X syndrome. Among the high-impact most prevalent conditions in our study (Carrier Map group) were: 21-Hydroxilase-Deficient Congenital Nonclassical Adrenal Hyperplasia (1/8), Factor V deficiency (1/12), Hemochromatosis: Type 1: HFE Related (1/12), Short Chain Acyl-CoA (1/14) and MTHFR deficiency 1/3 (39%).
The rate of gamete donors identified as carriers of at least one condition was 41%, which supports the offering of expanded carrier screening to our population. Studies in Latin American populations could help customize screening panels. The ART patient population has a unique opportunity to be offered expanded carrier screening and appropriate counseling, to make its best-informed decisions.
根据我们的实践,探讨孕前进行扩展基因携带者筛查的意义。
143名年龄在20 - 32岁的潜在配子捐赠者(平均年龄24岁,女性127名,男性16名)签署了知情同意书,并根据REDLARA指南进行挑选。通过以下基因携带者筛查方法之一对血液或唾液样本进行检测:针对囊性纤维化(CF)、脆性X综合征和脊髓性肌萎缩症(SMA)的健赞筛查;Counsyl通用检测板或Recombine携带者图谱。
分析了所有捐赠者的基因分型结果;41%(58/143)的捐赠者被确定为至少携带一种疾病的基因。我们发现CF的携带者频率为1/24,SMA为1/72,脆性X综合征为0/120。在我们研究中(携带者图谱组)影响较大且较为常见的疾病包括:21 - 羟化酶缺乏先天性非经典肾上腺皮质增生(1/8)、凝血因子V缺乏(1/12)、血色素沉着症:1型:与HFE相关(1/12)、短链酰基辅酶A(1/14)以及亚甲基四氢叶酸还原酶缺乏1/3(39%)。
被确定为至少携带一种疾病基因的配子捐赠者比例为41%,这支持了对我们的人群进行扩展携带者筛查。针对拉丁裔人群的研究有助于定制筛查检测板。辅助生殖技术患者群体有独特的机会接受扩展携带者筛查和适当的咨询,从而做出最明智的决策。