Steinbach P, Djalali M, Hansmann I, Kattner E, Meisel-Stosiek M, Probeck H D, Schmidt A, Wolf M
Hum Genet. 1983;65(2):155-64. doi: 10.1007/BF00286654.
Ten new cases of accessory bisatellited marker chromosomes examined in different laboratories are reported. As a basis for genetic counseling in the context of prenatal diagnosis a cytogenetic categorization of such marker chromosomes is proposed and an estimation of the genetic risk associated with each category is carried out. The results are as follows: There is no increased risk for offspring with abnormal phenotype born to a healthy carrier of an accessory bisatellited marker chromosome with either a single or two closely adjacent C-bands (Category AI or AII). The unbiased sample of cases with de novo accessory bisatellited marker chromosomes of categories AI and AII is too small to allow a satisfactory estimation of the actual risk that, in case of such a prenatal finding, the foetus may not show a normal phenotype as a consequence of the marker chromosome. There is, however, evidence that this risk may be lower than 10%. Accessory bisatellited marker chromosomes showing a discrete pattern of G- and R-bands situated between two distant C-bands (Category AIII) usually indicate a chromosomal imbalance giving rise to an abnormal phenotype. Mosaic carriers of such dicentric marker chromosomes may, however, present a normal phenotype.
本文报告了在不同实验室检测的10例新的双随体标记染色体病例。作为产前诊断中遗传咨询的基础,本文提出了对此类标记染色体的细胞遗传学分类,并对与每一类相关的遗传风险进行了评估。结果如下:携带单个或两个紧密相邻C带的双随体标记染色体的健康携带者所生的具有异常表型的后代,其风险并未增加(AI或AII类)。AI和AII类新发双随体标记染色体病例的无偏样本太小,无法对产前发现此类标记染色体时胎儿可能因标记染色体而不表现正常表型的实际风险进行令人满意的估计。然而,有证据表明这种风险可能低于10%。显示位于两个远距离C带之间的G带和R带离散模式的双随体标记染色体(AIII类)通常表明存在染色体不平衡,从而导致异常表型。然而,这种双着丝粒标记染色体的嵌合体携带者可能表现出正常表型。