Nivelon-Chevallier A, Sidaner I, Mugneret F, Turc-Carel C
Centre de Génétique, Hôpital d'Enfants du Bocage, Dijon.
J Genet Hum. 1988 Aug;36(4):331-6.
Among number of women having consulted for repeated miscarriages about sixty have a gonosomic mosaicism involving chromosome X for which abnormal clones (monosomy and/or excess) are always the minority. A retrospective study of the obstetrical follow up these patients have shown that 23% of them will give birth to a child with a chromosomic abnormality (21 trisomy, 13 trisomy, 45,X, 45,X/46,X iso X (q), 48,XXXX, 49 XXXXXY, del 5 p-). The hypothesis of a tendency toward non-disjunction is pushing for an prenatal diagnosis for patients with X mosaicism. However one can question about the real meaning of this abnormality regarding to the fact that the patients referred are not representative of the general population. Should interchromosomic interaction be taken for responsible? Is the risk for having a child chromosomic abnormality, especially X aneuploidy the same than for the overall population. It certainly would be rewarding to look after these different hypothesis in a multicentric collaborative study.
在因反复流产而咨询的众多女性中,约有60人存在涉及X染色体的生殖细胞嵌合体,其中异常克隆(单体型和/或超数)始终占少数。对这些患者的产科随访进行的回顾性研究表明,其中23%的患者将生下患有染色体异常的孩子(21三体、13三体、45,X、45,X/46,X iso X(q)、48,XXXX、49,XXXXXY、del 5 p-)。非整倍体倾向的假说促使对X染色体嵌合体患者进行产前诊断。然而,鉴于转诊患者不能代表一般人群这一事实,人们可能会质疑这种异常的真正意义。染色体间的相互作用是否应承担责任?生下染色体异常孩子的风险,尤其是X染色体非整倍体的风险,与总体人群是否相同?在多中心合作研究中探究这些不同假说肯定会有所收获。