Mattei J F, Mattei M G, Giraud F
Hum Genet. 1983;64(4):356-62. doi: 10.1007/BF00292367.
A chromosome 15 anomaly was observed in 12 of 20 patients, 17 of whom were clinically suspected of having Prader-Willi syndrome (PWS). The clinical features of eight cases with 15q11-12 deletion were very similar to those originally described in PWS. On the other hand, the group of normal karyotype patients is heterogeneous, and their features do not strictly correspond to the clinical definition of PWS. However, the hypothesis that PWS is associated with deletion of 15q11-12 can neither explain the apparently balanced translocations of chromosome 15 nor account for the small supernumerary metacentric chromosomes corresponding to an isochromosome 15 for band 15q11 observed in some cases.
在20例患者中,观察到12例存在15号染色体异常,其中17例临床上疑似患有普拉德-威利综合征(PWS)。8例15q11 - 12缺失患者的临床特征与最初描述的PWS非常相似。另一方面,核型正常的患者组具有异质性,其特征并不严格符合PWS的临床定义。然而,PWS与15q11 - 12缺失相关的假说既无法解释15号染色体明显的平衡易位,也无法解释在某些病例中观察到的与15q11等臂染色体15相对应的小的额外中着丝粒染色体。