Williams C A, Gray B A, Hendrickson J E, Stone J W, Cantú E S
Raymond C. Philips Research and Education Unit, Department of Pediatrics, University of Florida, Gainesville.
Am J Med Genet. 1989 Mar;32(3):339-45. doi: 10.1002/ajmg.1320320313.
Prometaphase chromosome study of 12 persons with an established diagnosis of the Angelman syndrome demonstrated that 5 had a 15q12 deletion appearing similar to that commonly observed in the Prader-Willi syndrome. Phenotype-karyotype correlation did not show any obvious clinical differences between those with and those without the deletion and no clinical overlap between Angelman and Prader-Willi syndrome was apparent. Our survey suggests that 15q12 deletions are frequent in Angelman syndrome but presence of the deletion does not appear to distinguish different clinical phenotypes. Experience with the cytogenetic study of Prader-Willi syndrome predicts that considerable complexity will emerge between the presence of 15 chromosome abnormalities and clinical expression of Angelman syndrome.
对12名已确诊为天使综合征的患者进行的前中期染色体研究表明,其中5人存在15q12缺失,其表现与普拉德-威利综合征中常见的缺失相似。表型-核型相关性分析显示,有缺失和无缺失的患者之间没有明显的临床差异,且天使综合征和普拉德-威利综合征之间没有明显的临床重叠。我们的调查表明,15q12缺失在天使综合征中很常见,但该缺失的存在似乎并不能区分不同的临床表型。对普拉德-威利综合征的细胞遗传学研究经验表明,15号染色体异常的存在与天使综合征的临床表型之间将出现相当大的复杂性。