Boonen Susanne Eriksen, Grønskov Karen, Brøndum-Nielsen Karen
Kennedy Instituttet-Statens Øjenklinik, Glostrup.
Ugeskr Laeger. 2006 Oct 23;168(43):3727-8.
A case story is presented of a child diagnosed by chromosome analysis to be carrier of the fragile X chromosome at a low frequency in cultured lymphocytes. DNA analysis of the FMR1 gene at a later date did not reveal expansion of the FMR1 repeat, thereby refuting the diagnosis of fragile X syndrome. The discrepancy was discovered only when years later other family members came for counselling due to subsequent development of DNA-based analyses. It is recommended that persons and families investigated before DNA methods were used are re-evaluated and re-examined when relevant. Genetic diagnoses need regular revision, and information to families is important.
本文介绍了一个病例。通过染色体分析,该儿童在培养的淋巴细胞中被诊断为脆性X染色体的低频携带者。后来对FMR1基因进行的DNA分析并未发现FMR1重复序列的扩增,从而推翻了脆性X综合征的诊断。这种差异直到数年后其他家庭成员因后续基于DNA的分析进展前来咨询时才被发现。建议对在DNA方法应用之前接受过调查的个人和家庭,在相关情况下进行重新评估和复查。基因诊断需要定期修订,向家庭提供信息非常重要。